What is colic

Colic also called infant colic or baby colic, is the medical term for excessive, frequent crying in a baby who appears to be otherwise healthy and well fed. Colic is very common, affecting about 1 – 2 in 5 babies (10% to 40% of infants worldwide) ¹⁾. However it is still poorly understood.

All newborns cry and get fussy sometimes. During the first 3 months of life, they cry more than at any other time. But when a baby who is healthy cries for more than 3 hours a day, more than 3 days a week, a doctor may say the baby has colic.

Colic doesn’t mean a baby has any health problems. With time, colic goes away on its own.

In most cases, the intense crying occurs in the late afternoon or evening and usually lasts for several hours.

You may also notice that your baby’s face becomes flushed, and they may clench their fists, draw their knees up to their tummy, or arch their back.

If your baby has colic, they may appear to be in distress. However, the crying outbursts are not harmful and your baby will continue to feed and gain weight normally. There is no clear evidence that colic has any long-term effects on a baby’s health.

• Colic is defined as crying for more than 3 hours a day, for more than 3 days a week, for at least 3 weeks ²⁾. But doctors may diagnose a baby as having colic before that point.
• Most of this crying and fussing seems to happen in the late afternoon and evening, although this can change from day to day.
• Colic is not dangerous, although it can be frightening, frustrating and upsetting for parents.
• Colic usually doesn’t point to any health problems and eventually goes away on its own.
• The incidence of infantile colic is equal between sexes, and there is no correlation with type of feeding (breast vs. bottle), gestational age, or socioeconomic status.
• Colic causes considerable stress for parents and for their health care providers ³⁾. Indeed, in the first 3 months of a baby’s life, crying is the No. 1 reason for pediatric visits ⁴⁾. Parents often perceive—incorrectly—that the inconsolable crying is either a sign of serious illness or a result of poor parenting skills ⁵⁾.

Crying is normal in babies. At six to eight weeks, babies normally cry for two to three hours a day. But babies with colic will cry inconsolably for more than three hours at a time on at least three days each week, and this cycle tends to last for more than three weeks.

Doctors aren’t sure what causes colic. It may be due to digestion problems or a sensitivity to something in the baby’s formula or that a nursing mom is eating. Or it might be from a baby trying to get used to the sights and sounds of being out in the world.

Some colicky babies also have gas because they swallow so much air while crying. But it’s not the gas that causes the colic.

Here are some key facts about colic baby:

• Colicky babies have a healthy sucking reflex and a good appetite and are otherwise healthy and growing well. Call your doctor if your baby isn’t feeding well, isn’t gaining weight, or doesn’t have a strong sucking reflex.
• Colicky babies may spit up from time to time just as non-colicky babies do. But if your baby is actually vomiting and/or losing weight, see your doctor. (Vomiting is a forceful throwing up of stomach contents through the mouth, whereas spitting up is an easy flow of stomach contents out of the mouth.) Vomiting repeatedly is not a sign of colic.
• Colicky babies typically have normal stools (poop). If your baby has diarrhea or blood in the stool, see your doctor.

If your baby has colic, there are things you can do to try to avoid possible triggers. There are also things you can try to soothe them and reduce crying.

There’s no treatment to make colic go away. But there are ways you can help:

• Make sure your baby isn’t hungry.
• Make sure your baby has a clean diaper.
• Try burping your baby more often during feedings.
• If you bottle-feed, try other bottles to see if they help your baby swallow less air.
• Ask your doctor if changing formula could help.
• Some nursing moms find that cutting caffeine, dairy, soy, egg, or wheat from their diet helps. Talk to your doctor before doing this and stop only one thing at a time.
• Rock or walk with the baby.
• Sing or talk to your baby.
• Offer your baby a pacifier.
• Take your baby for a ride in a stroller.
• Hold your baby close against your body and take calm, slow breaths.
• Give your baby a warm bath.
• Pat or rub your baby’s back.
• Place your baby across your lap on his or her belly and rub your baby’s back.
• Put your baby in a swing or vibrating seat. The motion may be soothing.
• Put your baby in an infant car seat in the back of the car and go for a ride. Often, the movement of the car is calming.
• Play music — some babies calm down with sound as well as movement.

Some babies need less stimulation. Babies 2 months and younger may do well swaddled, lying on their back in the crib with the lights very dim or dark. Make sure the swaddle isn’t too tight. Stop swaddling when the baby is starting to be able to roll over.

When does colic start?

Colic usually begins within the first few weeks of life – colic usually starts between the 3rd and 6th week after birth and peaks at around six to eight weeks ⁶⁾ and gets better by the time the baby is 3–4 months old. Any baby can have colic.

How long does colic last?

Colic often stops by the time the baby is four months old and by six months at the latest.

If the baby is still crying excessively after that, another health problem may be to blame.

How do I know if it’s colic or normal crying?

Colic is a special pattern of crying. Babies with colic are healthy, and eating and growing well but cry in spells. The spells happen at the same time of day. Most often, the crying starts in the early evening.

During a colic spell, a baby:

• has high-pitched crying or screaming
• is very hard to soothe
• can have a red face or pale skin around the mouth
• may pull in the legs, stiffen the arms, arch the back, or clench fists.

What if a baby won’t stop crying?

Caring for a colicky baby can be hard. If your baby won’t stop crying:

• Call a friend or family member for support or to take care of the baby while you take a break.
• If nothing else works, put the baby on his or her back in a crib without loose blankets or stuffed animals, close the door, and check on the baby in 10 minutes. During that 10 minutes, do something to try to relax and calm down. Try washing your face, eating a snack, deep breathing, or listening to music.

Don’t blame yourself or your baby for the crying — colic is nobody’s fault. Try to relax, and know that your baby will outgrow this phase.

If you ever feel like you might hurt yourself or the baby, put the baby down in the crib and call for help right away. Never shake a baby.

Can colic be prevented or avoided?

You cannot prevent or avoid your baby from being colicky.

Colic complications

Colic does not cause short-term or long-term medical problems for a child.

Colic is stressful for parents. Research has shown an association between colic and the following problems with parent well-being:

• Increased risk of postpartum depression in mothers ⁷⁾
• Early cessation of breast-feeding
• Feelings of guilt, exhaustion, helplessness or anger.

Shaken baby syndrome

The stress of calming a crying baby has sometimes prompted parents to shake or otherwise harm their child ⁸⁾. Shaking a baby can cause serious damage to the brain and death. The risk of these uncontrolled reactions is greater if parents don’t have information about soothing a crying child, education about colic and the support needed for caring for an infant with colic.

What causes colic

Doctors aren’t sure what causes colic, but a number of theories have been suggested — proposed causes include alterations in fecal microflora, intolerance to cow’s milk protein or lactose, gastrointestinal immaturity or inflammation, increased serotonin secretion, poor feeding technique, and maternal smoking or nicotine replacement therapy. Cow’s milk intolerance has been suggested as a possible culprit, but doctors now believe that this is rarely the case. Breastfed babies get colic too; in these cases, dietary changes by the mother may help the colic to ease. Some breastfeeding women find that getting rid of caffeine in their diet helps, while others see improvements when they eliminate dairy, soy, egg, or wheat products.

Some colicky babies also have gas, but it’s not clear if the gas causes colic or if the babies develop gas as a result of swallowing too much air while crying.

Some theories suggest that colic happens when food moves too quickly through a baby’s digestive system or is incompletely digested. Other theories are that colic is due to a baby’s temperament, that some babies just take a little bit longer to get adjusted to the world, or that some have undiagnosed gastroesophageal reflux (GER) or gastroesophageal reflux disease (GERD). Gastroesophageal reflux is a condition where stomach acid moves back out of the stomach and into the oesophagus (food pipe). Other research suggests that colic may be an early form of migraine headache. However, there is currently little solid evidence to support these theories.

Colic occurs equally in boys and girls, and in babies who are breastfed or bottle-fed. Colic is thought to be more common in babies whose mothers are smokers or who smoked while they were pregnant but the evidence is not conclusive.

Possible contributing factors that have been explored include:

• Digestive system that isn’t fully developed
• Pain or discomfort from gas or indigestion
• Imbalance of healthy bacteria in the digestive tract
• Food allergies or intolerances
• Sensitivity to formula or breast milk
• Overfeeding, underfeeding or infrequent burping
• Early form of childhood migraine
• Family stress or anxiety
• Overstimulation
• Early form of childhood migraine headache.
• Emotional reaction to fear, frustration, or excitement.

Risk factors for colic in babies

Risk factors for colic are not well-understood. Research has not shown differences in risk when the following factors were considered:

• Sex of the child
• Preterm and full-term pregnancies
• Formula-fed and breast-fed babies

Infants born to mothers who smoked during pregnancy or after delivery have an increased risk of developing colic.

Colic signs and symptoms

Fussing and crying are normal for infants, especially during the first three months. And the range for what is normal crying is difficult to pin down. In general, colic is defined as crying for three or more hours a day, three or more days a week, for three or more weeks.

Features of colic may include the following:

• Intense crying that may seem more like screaming or an expression of pain
• Crying for no apparent reason (for example, they aren’t hungry or need a diaper change), unlike crying to express hunger or the need for a diaper change
• Crying around the same time(s) each day. Colicky babies often get fussy toward the end of the day. But it can happen at any time.
• Extreme fussiness even after crying has diminished
• Predictable timing, with episodes often occurring in the evening
• Facial discoloring, such as reddening of the face or paler skin around the mouth
• Clenching their fists when crying or curling up their legs.
• Bodily tension, such as pulled up or stiffened legs, stiffened arms, clenched fists, arched back, or tense abdomen
• Crying like they’re in pain.
• Turning bright red when crying.

Sometimes there is relief in symptoms after the infant passes gas or has a bowel movement. Gas is likely the result of swallowed air during prolonged crying.

When your baby cries, they can swallow air. This may give your baby gas. It can make their tummy look swollen or feel tight. They might show some relief in symptoms after passing gas or having a bowel movement (pooping).

Colic in babies diagnosis

There are no tests to diagnose colic. Health care professionals ask about the crying and how the baby is doing. Colic can usually be diagnosed based on the pattern and amount of crying, and the fact that the baby is otherwise thriving – they are gaining weight normally and eating well. If parents are concerned about their baby’s crying, their doctor may examine the baby and check for problems with their digestive system or signs of other medical conditions.

If you think your baby has colic, see your doctor. Your baby’s doctor will do a complete physical exam to identify any possible causes for your baby’s distress.

The exam will include:

• Measuring your baby’s height, weight and head circumference
• Listening to the heart, lungs and abdominal sounds
• Examining the limbs, fingers, toes, eyes, ears and genitals
• Assessing reaction to touch or movement
• Looking for signs of rash, inflammation, or other signs of infection or allergies

Table 1. Conditions to consider in the evaluation of Unexplained Crying in Infants

Findings	Possible cause	Other historical clues	Physical examination findings	Diagnostic testing
Diarrhea, intermittent, explosive	Hirschsprung disease	Bilious emesis, chronic constipation, fever, no meconium passed in first 24 hours of life, poor feeding; may coexist with Trisomy 21; more common in boys	Abdominal distention or tenderness, jaundice	Abdominal radiography (with or without contrast enema), anorectal manometry, rectal suction biopsy
Scrotal or inguinal swelling	Incarcerated hernia	Abdominal pain, bilious emesis	Abdominal bulging or distention, acute abdomen	Ultrasonography
	Testicular torsion	Acute onset of crying and pain	High-riding testicle, scrotal discoloration, tenderness to palpation	Ultrasonography
Tenderness to palpation in long bones, clavicles, or scalp	Child abuse	History of fall or trauma, lethargy, unwillingness to move extremities	Burns, frenulum tears, geographic scars, retinal hemorrhage, suspicious bruises	Computed tomography to detect intracranial hemorrhage, radiography of extremities
Vomiting, recurrent and/or forceful	Gastroesophageal reflux disease	Apnea, arching of the back with feeding, cough, feeding refusal, hematemesis, irritability, poor weight gain, wheezing	Nonspecific	None required in uncomplicated reflux; 24-hour pH monitoring may be used for complicated reflux; endoscopy for persistent symptoms
	Pyloric stenosis	Normal appetite, progressive nonbilious projectile vomiting; more common in boys; presents at two to six weeks of age	Clinical dehydration, palpable pyloric mass or “olive” in right midepigastrium, visualization of gastric peristalsis with feeding	Ultrasonography of pylorus
No clinical signs or symptoms	Anal fissure	Bloody or painful bowel movements	Fissure	None
	Corneal abrasion	Tearing	Conjunctival erythema, scratches near the eye	Fluorescein testing
	Cow’s milk allergy	Bloody stools, constipation, diarrhea, excessive gas, pain with defecation, rash, vomiting	Rash	Fecal occult blood testing, resolution of symptoms after maternal dietary change (in breastfed infants) or formula change
	Hair tourniquet syndrome	Edema of toes, fingers, or penis; hair found curled up near infant	Hair wrapped around finger, toe, or penis	None
	Inadequate bottle feeding	Aggressive feeding	Clinical dehydration, loss of fat from cheeks, weight loss or poor weight gain	Weight increase with increase in formula feedings
	Inadequate breastfeeding	Breasts not emptying with feeding	Poor latch observed; weight loss or poor weight gain	Weight increase with supplemental formula

[Source ⁹⁾]

How to soothe colic baby

There is no ‘best’ way to comfort your baby or reduce the symptoms of colic. Different babies respond to different methods, so you may have to see what works best for you.

There is no single treatment that has been proven to make colic go away. Many parents have tried things like over-the-counter gas relieving medicine, but these medicines haven’t been shown to improve colic. Other people have tried probiotics (things that help to maintain the balance of ‘good’ bacteria in the digestive system). While some studies have shown that certain probiotics may improve colic, others have shown no improvements. Experts currently don’t recommend probiotics for treating colic.

The probiotic Lactobacillus reuteri (strain DSM 17938) reduced colic symptoms in four out of five clinical trials ¹⁰⁾, ¹¹⁾, ¹²⁾, ¹³⁾. No adverse effects were reported. Two recent meta-analyses and one systematic review found that administration of five drops of Lactobacillus reuteri per day significantly decreased colic in infants who are breastfed (average of 61 minutes less crying time per day at 21 days) ¹⁴⁾, ¹⁵⁾, ¹⁶⁾. One trial found a significant increase in crying or fussing in bottle-fed infants who received Lactobacillus reuteri ¹⁷⁾. Based on these results, Lactobacillus reuteri DSM 17938 may be considered as a treatment option for breastfed infants, but cannot be recommended for formula-fed infants.

Experts have also looked at the effects of changing diets or restricting certain foods, but currently there no specific dietary recommendations for treating colic. Switching from breastfeeding to formula does not help colic. Using a special teat on feeding bottles has not been shown to help colic.

Colic usually gets better on its own in a few months. But there are ways to make life easier for both you and your colicky baby.

First, if your baby is not hungry, don’t try to continue the feeding. Instead, try to console your little one — you won’t be “spoiling” the baby with your attention.

You can also:

• Walk with your baby or sit in a rocking chair, trying various positions.
• Keep calm and talk gently to your baby.
• Check your baby doesn’t need a nappy change.
• Holding your baby during a crying episode can sometimes help, as can wrapping them snugly in a blanket or baby sling.
• Develop a regular daily pattern of feeds and sleeps.
• Don’t overstimulate your baby
• Try to soothe your baby before they become too worked up
• Try not to startle your baby, eg with quick movements
• Check formula is being made up correctly, if you’re using it
• Try burping your baby more often during feedings.
• Place your baby across your lap on his or her belly and rub your baby’s back.
• Put your baby in a swing or vibrating seat. The motion may have a soothing effect.
• Put your baby in an infant car seat in the back of the car and go for a ride. The vibration and movement of the car are often calming.
• Play music — some babies respond to sound as well as movement.
• Place your baby in the same room as a running clothes dryer, white noise machine, or vacuum — some infants find the constant low noise soothing.
• Some babies need decreased stimulation. Babies 2 months and younger may do well swaddled in a darkened room.

Caring for a colicky baby can be extremely frustrating, so be sure to take care of yourself, too. Don’t blame yourself or your baby for the constant crying — colic is nobody’s fault. Try to relax, and remember that your baby will eventually outgrow this phase.

In the meantime, if you need a break from your baby’s crying, take one. Friends and relatives are often happy to watch your baby when you need some time to yourself. If no one is immediately available, it’s OK to put the baby down in the crib and take a break before making another attempt at soothing your little one. If at any time you feel like you might hurt yourself or the baby, put the baby down in the crib and call for help immediately. Never shake a baby.

If your baby has a temperature of 100.4 °F (38 °C) or higher, is crying for more than 2 hours at a time, is inconsolable, isn’t feeding well, has diarrhea or persistent vomiting, or is less awake or alert than usual, see your doctor right away. Also see your doctor if you’re unsure whether your baby’s crying is colic or a symptom of another illness.

Trial changes in diet

If soothing or feeding practices aren’t reducing crying or irritability, your doctor may recommend a short-term trial of dietary changes. If your baby has a food allergy, however, there would likely be other signs and symptoms, such as a rash, wheezing, vomiting or diarrhea. Dietary changes may include:

• Formula changes. If you feed your infant formula, your doctor may suggest a one-week trial of an extensive hydrolysate formula (Similac Alimentum, Nutramigen, Pregestimil, others) that has proteins broken down into smaller sizes.
• Maternal diet. If you’re breast-feeding, you may try a diet without common food allergens, such as dairy, eggs, nuts and wheat. You may also try eliminating potentially irritating foods, such as cabbage, onions or caffeinated beverages.

The prevalence of colic is similar between breastfed and bottle-fed infants; therefore, breastfeeding mothers should be encouraged to continue ¹⁸⁾. A randomized controlled trial showed significant reductions in colic symptoms among breastfed infants whose mothers followed a low-allergen diet ¹⁹⁾. Infants whose mothers excluded cow’s milk, eggs, peanuts, tree nuts, wheat, soy, and fish from their diet cried for 137 minutes less per day, compared with 51 minutes less per day in the control group. A systematic review of six studies supported the effectiveness of low-allergen diets in reducing colic ²⁰⁾. These diets may be an option for some breastfeeding mothers, but should be accompanied by dietary counseling to ensure adequate nutrition. Return to a normal diet can be considered after the infant reaches three to six months of age.

Parents of formula-fed infants with colic often consider switching formulas. A systematic review of 13 studies found a statistically significant decrease in crying time among infants who switched to partially, extensively, or completely hydrolyzed formulas (Table 2) ²¹⁾. These studies suggest that a two-week trial of a different formula may be considered for infants with colic. To make the change more palatable, parents can transition to hydrolyzed formula by mixing the new formula with regular formula incrementally over four days, until only hydrolyzed formula is being given. These formulas are expensive, however, and may not be covered by assistance programs (e.g., Women, Infants, and Children). If the new formula is successful in reducing colic symptoms, regular formula may be restarted after three to six months of age.

Four studies that evaluated the use of soy formula for the treatment of colic provided insufficient evidence to make clinical recommendations ²²⁾. The American Academy of Pediatrics recommends against the routine use of soy formula in the management of infantile colic, because soy can be an allergen ²³⁾.

Table 2. Select Hydrolyzed Infant Formulas

Type	Brand	Formula name
Partially hydrolyzed	Enfamil	Gentlease
	Gerber	Good Start Gentle
	Gerber	Good Start Soothe
	Similac	Similac Total Comfort
Extensively hydrolyzed	Enfamil	Nutramigen
	Enfamil	Pregestimil
	Similac	Alimentum
Completely hydrolyzed	Elecare	Elecare
	Enfamil	Nutramigen AA
	Nutricia	Neocate

[Source ²⁴⁾]

Medications

• Simethicone. Although simethicone drops are readily available and often used to treat colic, a systematic review of three randomized controlled trials found that they are no better than placebo ²⁵⁾.
• Dicyclomine. Although a systematic review of three randomized controlled trials found that dicyclomine was significantly better than placebo for the treatment of colic, it is contraindicated in infants younger than six months because of adverse effects such as drowsiness, constipation, diarrhea, and apnea ²⁶⁾.
• Proton Pump Inhibitors. A four-week randomized controlled trial of 30 infants with colic symptoms and gastroesophageal reflux or esophagitis found that omeprazole (Prilosec) was no better than placebo at reducing crying or fussing time ²⁷⁾.

Coping with colic

Looking after a baby with colic can be very difficult for parents, particularly first-time parents.

It’s important to remember that colic:

• Is not the parents’ fault
• Doesn’t mean a baby is unwell or in pain
• Is temporary: it will go away eventually. Colic episodes often improve after age 3 to 4 months.
• Take a break. Take turns with your spouse or partner, or ask a friend to take over for a while. Give yourself an opportunity to get out of the house if possible.
• Use the crib for short breaks. It’s OK to put your baby in the crib for a while during a crying episode if you need to collect yourself or calm your own nerves.
• Express your feelings. It’s normal for parents in this situation to feel helpless, depressed, guilty or angry. Share your feelings with family members, friends and your child’s doctor.
• Don’t judge yourself. Don’t measure your success as a parent by how much your baby cries. Colic isn’t a result of poor parenting, and inconsolable crying isn’t a sign of your baby rejecting you.
• Take care of your health. Eat healthy foods. Make time for exercise, such as a brisk daily walk. If you can, sleep when the baby sleeps — even during the day. Avoid alcohol and other drugs.

It’s important that parents take care of themselves as well. Parents should try to take a break and ask someone else to take over for a little while, even just for an hour or 2.

Remember that caring for a baby with colic is not a one-person job. A doctor or child health nurse can provide support and will have some ideas that may help to soothe an unsettled baby.

It may be helpful to:

• Check with the doctor to make sure the baby is otherwise well
• Make sure the baby is not hungry or needs a nappy change
• Develop a regular daily pattern for feeding, sleeping and playing
• Develop a routine for settling the baby for naps and sleep
• Avoid excessive stimulation before sleep
• Avoid excessive quiet – most babies find a low level of background noise soothing, e.g. gentle music
• Darken the bedroom for daytime naps
• Try soothing techniques such as baby massage, gentle rocking or gentle patting
• Respond to the baby before they become too worked up
• Avoid startling the baby, e.g. with quick movements
• Handle the baby gently and speak quietly to them
• Carry the baby in a front pack or sling when they are unsettled
• If using formula, check that it is being made up correctly
• Ask for and accept offers of help
• Talk to a health professional if you need support or are feeling that you can’t cope.

Alternative medicine

Several small studies have shown some benefits or mixed results for alternative treatments. There’s not enough evidence, however, to judge the potential benefit over the risks. Alternative remedies under investigation include:

• Herbal teas
• Herbal remedies, such as fennel oil
• Sugar water
• Gripe water, a mix of water and herbs
• Massage therapy
• Chiropractic manipulation
• Acupuncture

Herbal supplements, including Mentha piperita (peppermint) and various herbal teas (including fennel, chamomile, vervain, lemon balm, and licorice), have decreased crying time in some studies ²⁸⁾. Despite these findings, a systematic review concluded that further research is required before recommending these treatments ²⁹⁾.

“Gripe water,” which consists of dill seed oil, bicarbonate, and hydrogenated glucose, has also been used for the treatment of colic. However, there are no trials demonstrating its effectiveness, and it may cause harm.

Physical therapies for colic include chiropractic and osteopathic manipulation, massage, and acupuncture. A Cochrane review found insufficient evidence to support chiropractic or osteopathic manipulation, because many studies were small, nonblinded, and had a high likelihood of bias ³⁰⁾. Trials of acupuncture and infant massage have had conflicting results, and further studies are needed to determine their benefits and harms ³¹⁾.

Known risks include the following issues:

• Regular use of herbal teas or other liquid preparations may lead to decreased milk intake or a drop in sodium levels in an infant’s blood.
• The lack of product regulation may result in contamination, unlabeled ingredients or inconsistent dosages in herbal remedies.
• Some homeopathic remedies contain low amounts of potentially toxic substances.

Talk to your doctor before using an alternative medicine to treat your infant for colic.

Colic treatment

The treatment of infantile colic is as varied and often unproven as the theoretical causes for colic ³²⁾. First-line treatment should be directed at the distressed parents, educating them about colic, and reassuring them that the condition is self-limiting and benign ³³⁾. Parents can be encouraged to develop coping responses when the child begins crying, minimizing the likelihood that they will become frustrated and injure the infant. In fact, parents who are frustrated by the infant’s crying should place the infant safely in a crib and walk away briefly, to avoid shaking the infant and causing harm. Traditionally parents have resorted to methods such as swaddling, rocking, or driving the infant in a car to control the symptoms of colic. However, clear evidence for the effectiveness of these methods is lacking ³⁴⁾.

Studies examining the effectiveness of simethicone in treating colic have been inconsistent, but most of the studies demonstrate no effect ³⁵⁾. The anticholinergic dicyclomine has shown improvement in crying time, but its potential side effects of respiratory depression, apnea, seizures, pulse rate fluctuations and muscular hypotonia make its use undesirable in young infants; dicyclomine is not approved for use in children under the age of 6 months. Proton pump inhibitors such as omeprazole are no better than placebo at reducing crying time in infants with colic. Thus, pharmacological approaches to the treatment of colic are not recommended ³⁶⁾.

Dietary modifications in the treatment of colic have been explored in some detail. Breastfed infants with colic have significant reductions in crying time if the mother is placed on a hypoallergenic diet, in which dairy foods, eggs, nuts, wheat, soy, and fish are eliminated from the mother’s diet. Bottle-fed infants who are switched from traditional cow’s milk formulas to soy-based formulas show no improvement in colic symptoms; at the same time, soy-based formulas are not recommended for infants less than 6 months of age due to the high concentrations of phytates, aluminum, and phytoestrogens in those formulas. The American Academy of Pediatrics discourages the use of soy-based formulas in the treatment of colic due to their lack of benefit. In contrast, a number of studies confirm the usefulness of extensively hydrolyzed or completely hydrolyzed cow’s milk protein formulas in the reduction of crying times for infants with colic. Unfortunately, these formulas are more expensive and may provide a financial challenge to the parents; if no improvement in symptoms occurs within two weeks, the infant may be switched back to traditional formulas.

Some treatment approaches are directed at correction of the infant’s gut microflora. While several studies demonstrate a reduction in crying time among bottle-fed infants with colic who receive supplements of Lactobacillus reuteri, other studies have contradicted those findings. However, there seems to be more consistent evidence regarding the positive effects of L. reuteri supplementation among infants who are breastfed. Probiotic supplementation may be useful for colicky breastfed infants but less useful for bottle-fed infants.

Reducing stimulation may be effective for some infants with colic, much like its effectiveness in children with migraines. Techniques may include reducing the lighting in the room, turning down loud music and avoiding toys that make noise, keeping siblings and pets in another room, avoiding strong odors or perfumes, and rocking the baby gently may have beneficial effects. Feeding the infant in a darkened room may facilitate infant sleep.

Comparisons have been made between infants with colic who were treated with massage and those who were treated with rocking. While there are reductions in crying time for both treatment groups in one study, the greatest reduction was seen in infants undergoing regular massage by their mothers. However, other studies examining the effects of massage have been inconsistent in demonstrating effectiveness. While the effectiveness of massage may not be concrete, it is at least a safe intervention that may improve parental-infant bonding and can be used in any intervention for colic.

Feeding your baby

If you are breastfeeding your baby, keep track of what you eat and drink. Everything you consume gets passed to your baby and can affect them. Avoid caffeine and chocolate, which act as stimulants. Avoid dairy products and nuts, which your baby may be allergic to. Ask your doctor if any medicines you’re taking could be a problem.

If you are feeding your baby formula, you might try a different brand. Babies can be sensitive to certain proteins in formula. Try feeding your baby less food more often. Avoid feeding your baby too much or too quickly. One bottle feeding should last about 20 minutes. If your baby eats faster, try using a nipple with a smaller hole. This will slow down their feeding. You can try warming the formula to body temperature. Or try feeding your baby in an upright position.

Holding your baby

Babies who have colic respond to different ways of being held or rocked. You can try:

• Holding your baby across your arm or lap while you massage their back.
• Holding your baby upright, if they have gas.
• Holding your baby in the evening.
• Holding your baby while walking.
• Rocking your baby in your arms or using an infant swing.

Comforting your baby

Babies also respond differently to movements and stimuli. Other things that can help soothe your baby include:

• Providing extra skin-to-skin contact.
• Swaddling your baby.
• Singing to your baby.
• Giving your baby a warm (not hot) bath or putting a warm towel on their stomach.
• Massaging your baby (ask your doctor for guidelines).
• Providing white noise, such as a fan, vacuum cleaner, washing machine, hair dryer, or dishwasher.
• Giving your baby a pacifier.
• Going for a walk with your baby in their stroller.
• Going for a drive with your baby in their car seat.
• Giving your baby simethicone drops. This over-the-counter medicine can help relieve gas.

Living with colic

Colic doesn’t cause any short-term or long-term problems for your baby. But colic can be difficult for parents. It can be hard to care for babies who don’t stop crying. You may feel overwhelmed or frustrated. If you are feeling this way, it is important to ask for help. Ask someone close to you to help watch your baby. Never shake or harm your child. Shaking a baby can cause serious brain damage and even death. If you feel like you might shake or harm your baby, get help right away.

The following are things to keep in mind about colic.

• You didn’t cause the colic, so try not to feel guilty.
• Colic will go away. Most babies outgrow it by the time they are 3 to 4 months old.
• Just because your baby has colic doesn’t mean they are unhealthy.
• There are many ways to soothe your baby.
• Giving your baby extra attention, such as holding them for extended periods, won’t spoil them.

Contact your baby’s doctor if:

• Your baby’s crying is mixed with a fever, vomiting, loose or bloody stools, or decreased movement.
• Your baby’s crying or behavior changes all of a sudden.

Colic prognosis

Newborn colic is a benign and self-limiting condition that typically resolves spontaneously by three months of age. However, there are some indications that infants with colic may be at more risk for developing migraines without aura by age 18 ³⁷⁾. There is also evidence linking excessive crying early in life with preschool adaptive problems, attention deficit hyperactivity disorder, and other behavioral problems, as well as being associated with maternal anxiety and depression. Thus, counseling parents regarding colic and helping them to develop coping mechanisms, as well as offering suggestions on the management of colic symptoms, seems prudent.

Vulvovaginitis in children

Vulvovaginitis is inflammation or irritation of the delicate skin of the vagina and vulva (external female genital area) ¹⁾. The vulva is the outside area of the female genitals; the vagina connects the vulva and the uterus. Mild vulvovaginitis is the most common gynecological problem in prepubertal girls, and some children will have vulvovaginitis many times ²⁾. Once puberty has begun, vulvovaginitis usually occurs less often.

The most common symptoms and signs of vulvovaginitis include itchiness (pruritus), soreness, burning, painful urination (dysuria), redness (erythema), whitish discharge (leucorrhea) and swelling (edema) ³⁾. Sometimes children with vulvovaginitis have a slight discharge from the vagina, which will stain the underpants and produce a strong odor.

Parents often first become aware of the problem when their child complains about the redness and soreness of the skin, which is the most common sign of vulvovaginitis.

Most often, vulvovaginitis is caused when the vulvar and vaginal skin become irritated. This irritation can be caused by the use of products that are too strong for the delicate vulvar skin, clothing that rubs, dampness and less commonly, a skin or vaginal infection. The prevalence of non-specific vulvovaginitis ranges between 25% and 75% of cases in prepubertal girls ⁴⁾. Interestingly, a high percentage of non-specific vulvovaginitis cases are recurrent ⁵⁾. Vulvovaginitis is can be caused by either a pathogenic infection (including intestinal parasites, respiratory streptococci and Candida secies) or by a non-specific cause, such as trauma, skin problems such as eczema or congenital disorders ⁶⁾.

Vulvovaginitis can also be caused by sexual abuse. Vulvovaginitis may result from several of these causes at the same time.

In most cases, vulvovaginitis is not a serious problem and it will usually improve with simple steps at home. Usually no medical treatment or tests are needed.

Some hygienic sanitary measures are fundamental in the management of non-specific vulvovaginitis, such as avoiding wet clothing (in contact with the genital area) for long periods and avoiding the use of tight-fitting and synthetic clothing ⁷⁾. While the use of cotton underwear and adequate cleansing of the body with warm water and soap, excluding the genital area, are recommended ⁸⁾.

Vulvovaginitis toddler key points to remember:

• Mild vulvovaginitis is a very common problem in prepubertal girls ⁹⁾.
• It may recur now and then, but will improve as your child gets older.
• In most mild cases, no medical treatment or tests are necessary.
• Avoid the things that make vulvovaginitis worse, such as tight underwear and irritants like soap.

Figure 1. Pediatric vulvovaginitis

Footnote: Vulvovaginitis is an irritation of the skin in the vulvar and vaginal area. It causes soreness, itching, burning and can be seen as red and irritated skin.

Vulvovaginitis toddler causes

While your child is young, the lining of her vagina and vulva can be quite thin and this can lead to it being easily irritated. Moisture or dampness around the vulva can also lead to vulvovaginitis – this is made worse by tight clothing or being overweight. Another cause of vulvovaginitis is irritants, such as soap residue, bubble baths and antiseptics.

Microorganisms, isolated from vaginal smears, were detected in 48.9% of the patients. Escherichia coli was shown in the urine culture of 3 patients with vulvovaginitis (6.70%). In microscopic stool analysis parasites were detected (45.9%). Scientists found some relevant personal hygiene factors, such as wiping back to front (42.9%), cleaning by herself after defecation (89.3%), using toilet paper (60.7%) and wet wipes (21.4%), and bathing standing (14.3%) and sitting (46.4%) among patients ¹⁰⁾. They also showed that the children wore tight clothing (35.7%).

The findings from this study ¹¹⁾ suggest that vulvovaginitis in prepubertal girls is related not only to microorganisms but also poor personal hygiene, the educational status of mothers, and specific irritants.

Factors associated with the risk of vulvovaginitis include a hypoestrogenized vaginal mucosa in a preadolescent girl, lack of development of the labia minora, close proximity of the vagina to the anus, poor hygiene, tight-fitting clothing or the use of shampoos or soaps ¹²⁾. The hypoestrogenic hormonal milieu in a preadolescent girl is a major factor in making her vaginal mucosa susceptible to infection ¹³⁾.

Pinworms or threadworms sometimes cause or worsen vulvovaginitis. Pinworm infection is caused by a small, thin, white roundworm called Enterobius vermicularis ¹⁴⁾. Children with pinworms often scratch a lot at night. If itching is a major symptom, then you may want to treat your child for pinworms (threadworms).

The medications used for the treatment of pinworm are either mebendazole, pyrantel pamoate, or albendazole. Any of these drugs are given in one dose initially, and then another single dose of the same drug two weeks later. Pyrantel pamoate is available without prescription. The medication does not reliably kill pinworm eggs. Therefore, the second dose is to prevent re-infection by adult worms that hatch from any eggs not killed by the first treatment. Health practitioners and parents should weigh the health risks and benefits of these drugs for patients under 2 years of age.

Repeated infections should be treated by the same method as the first infection. In households where more than one member is infected or where repeated, symptomatic infections occur, it is recommended that all household members be treated at the same time. In institutions, mass and simultaneous treatment, repeated in 2 weeks, can be effective.

Skin in the vaginal area changes over time

In young children, before puberty, the skin in the vaginal area is thin and delicate. This delicate skin is easily irritated. When they grow and begin to produce their own sex hormones, pubic hair and thickened skin will develop and protect the vaginal area. Normal vulvar skin should not look reddish or raw.

Activities and clothing

Many normal things children do can irritate the skin around the vagina. For example, playing in sandboxes or on slides and teeter-totters, and wearing wet clothes, bathing suits or tight ballet leotards can irritate the skin.

Sometimes chidren wait too long to go to the bathroom

Children who are playing often forget to go to the bathroom until the last minute. Sometimes they do not wipe themselves properly. When this happens, bacteria from the anus can get into the vagina. Dirty underwear can also bring bacteria into contact with the vagina and the surrounding skin. Most children will not wash their vaginal area at bath time. Children need to be helped with bathing so they clean themselves properly.

Other problems: soaps and the weather

• Soaps, bubble baths and perfumes can irritate a child’s skin. Strong laundry detergents and fabric softeners can also be a source of irritation.
• Dry winter air can be irritating to the skin.
• Heat and humidity can also irritate the skin, especially if combined with wet bathing suits or tight clothing.

Vulvovaginitis toddler prevention

Things your child can do to treat and avoid vulvovaginitis:

• Take sitz baths twice a day when the skin is irritated.
• Do not use soaps in the vulvar region. It is best to clean with warm water on a soft face cloth. No bubble baths.
• Use gentle detergent on the underwear and do not use fabric softener.
• If the skin is very sore, try using cotton balls with mineral oil after a bowel movement instead of toilet paper.
• Do not wear underwear to bed.
• Do not wear tight clothing such as pantyhose, tight pants or leotards.
• Change out of wet bathing suits or any other wet clothing as soon as possible.
• Girls who are menstruating should not use perfumed tampons or pads.
• Do not use topical medications, or topical antibiotic ointments or feminine hygiene sprays.
• Use petroleum jelly or zinc oxide diaper cream on the area. It can be soothing and is harmless.

Vulvovaginitis toddler symptoms

If your child has vulvovaginitis, she may have:

• itching in the vaginal area
• some discharge from the vagina
• redness of the skin between the labia majora (outside lips of the vagina)
• burning or stinging when she passes urine.

The most common symptoms among the patients were vaginal discharge (44.4%), vulvar erythema (37.8%), and vaginal itch (24.4%) ¹⁵⁾.

Vulvovaginitis toddler diagnosis

If your child develops skin irritation in the vaginal area, develops an odor in the area or has discharge from the vagina, she should see her health-care provider. The diagnosis of vulvovaginitis in toddler gir is clinical and is mainly based on an adequate history and physical examination ¹⁶⁾.

Depending on the problem and how severe the symptoms are, your doctor may refer them to a gynecology clinic.

The gynecologist will ask questions about your child’s health and do a physical examination. The doctor will want to make sure that there is no infection or skin problem that needs treatment. Sometimes, a small vaginal swab will be taken to look for infection. If there has been any bleeding, the doctor may need to examine further.

The doctor will also ask questions and examine your child to make sure that they have not been subject to any sexual abuse. Tell your child what the examination is about, using words they can understand. The goal is to let your child feel comfortable with the examination and make sure they do not have a painful or frightening experience.

After the examination, the doctor will tell you what the best treatment is. Whatever treatment is recommended, there will be some things that you can do to help at home.

Vulvovaginitis toddler treatment

The treatment of vulvovaginitis will depend on the cause of the irritation. Usually, the irritation comes from hygiene measures. It may be that your child is not cleaning themselves well enough. It is important to remember that the most common cause of vaginal discharge in children with vulvovaginitis is skin irritation. It is rare to find an infection. If there is an infection, it can be treated with antibiotics or antifungals.

In most mild cases of vulvovaginitis, you can care for your child at home without visiting a doctor. Reassure your child that they don’t need to worry, as vulvovaginitis is a common problem and a normal part of growing up.

You can help your child by starting some simple habits and routines. Because your child’s skin is so delicate, anything you can do to help keep the vaginal area clean, dry and healthy will help. Here are some of the things you can do.

Try avoiding the things that make vulvovaginitis worse:

• Wear loose cotton underwear and avoid tight jeans etc.
• If your child is overweight, seek advice on how to maintain a healthy weight with diet and exercise.
• Don’t use a lot of soap in the bath or shower, and make sure any soap is well rinsed from the vulva. Avoid bubble baths and antibacterial products.

Some parents find vinegar baths helpful: add half a cup of white vinegar to a shallow bath and soak for 10 to 15 minutes. Do this daily for a few days and see if it helps.

Soothing creams (for example, soft paraffin, nappy-rash creams) may help settle the soreness, as well as protect the skin from moisture or any discharge, which can be irritating.

You may have to repeat these simple measures if the problem comes back.

Clean and dry underwear and clothes

Make sure your child changes into clean underwear often, especially if their underwear has gotten dirty. Try to have your child change their underwear more than once a day. White cotton underwear are best. Your child should change out of wet or tight-fitting clothing as soon as possible. Encourage your child to not wear underwear at night.

Do not use pantiliners to contain vaginal discharge as this will actually increase symptoms. It is better to make sure your child changes their underwear often.

Wiping properly after the bathroom

Make sure your child knows how to clean themselves well after using the toilet. This is a particular problem for parents whose children go to daycare or to babysitters, because the parents are not there to help their child. Children should wipe themselves from front to back. Use only white toilet paper. Many children can clean themselves better with alcohol- and perfume-free wet wipes. These wipes are easier on the skin than dry toilet paper. A little packet of wet wipes tucked into your child’s things when they go off to daycare may be helpful.

Bathing

If using wet wipes does not solve the problem, have your child take a shallow sitz bath when they get home to make sure that they are nice and clean. A sitz bath is a small tub that can be placed on the toilet and filled with warm water to soak the vulvar region. You can purchase a sitz bath at most drug stores. Do not use soaps, bubble baths or perfumed products on your child. When they have signs of vulvovaginitis, it is a good idea to have your child bathe two to three times a day. Adding non-allergenic skin softeners to the water will help soften and soothe the vulvar skin. You can consider applying a barrier cream such as petroleum jelly or a zinc oxide after bathing to help soothe the skin.

Encourage your childr not to scratch their bottom because scratching can cause more irritation and infection.

After a bath

After the bath, gently blot or pat dry the child’s vaginal area. Do not use scratchy towels or rub the skin too hard. You may find it helpful to use a hairdryer on a low, cool setting to dry the area gently. It is all right for the child to run around the house wearing skirts or loose shorts without underwear to let the air reach their bottom.

A good daily hygiene routine

Your child needs to learn how to take care of their vaginal area. It is an important part of their body that needs special cleaning, just as their teeth need special care. If creams have been recommended, your child can learn how to put their own cream on. They can learn to wash their hands before and after and use a hand mirror so that they can put the cream exactly where it is needed. In this way, your child will learn that this is their body and they have a responsibility to care for it.

Vulvovaginitis prognosis

Vulvovaginitis often comes back. About half the girls who have vulvovaginitis will have it more than once during their childhood. It usually gets better as girls grow up, and will not cause them any long-term harm.

If the irritation comes back, begin strict hygiene measures again. Your child may need to be seen at the clinic again. Sometimes, if the inflammation comes back it may be caused by something different.

The better your child gets at being clean and dry, the less likely they are to get vulvovaginitis again.

If your child has any pain or bleeding, see your health-care provider.

What is meatal stenosis

Meatal stenosis is a narrowing of the opening of the urethra, the tube through which urine leaves the body. Urinary meatal stenosis can affect both males and females. Meatal stenosis is more common in males. Meatal stenosis is a common complication of circumcision, caused from chronic exposure of the irritating effects of urine on the meatus and rubbing of the meatus on a diaper or clothing.

If the narrowing becomes significant, urine will trouble flowing from the bladder and may cause the bladder to not empty completely. If left untreated, this can lead to urinary tract infections and kidney problems.

In males, meatal stenosis is often caused by swelling and irritation (inflammation) after a newborn is circumcised. This leads to abnormal tissue growth and scarring across the opening of the urethra. In most cases, the problem is not found until the child is toilet trained. If your baby boy has recently been circumcised, try to keep the diaper clean and dry. Avoid exposing the newly circumcised penis to any irritants. They may cause inflammation and narrowing of the opening. A systematic review and meta-analysis by Morris et al. ¹⁾ found that application of petroleum jelly to the glans after circumcision was associated with a reduction in the risk of meatal stenosis (relative risk, 0.024).

Meatal stenosis affects 9-20% of males who are circumcised ²⁾. Children who are not toilet-trained are more prone to develop meatal stenosis after circumcision because of exposure of the meatus to urine in diapers. Most children who are toilet-trained can verbalize their difficulties during micturition to their caregivers. Meatal stenosis has no racial predilection. The condition can occur in circumcised males independent of ethnicity.

Surgery on the urethra, chronic catheterization, or other medical instruments in the urethra may also lead to meatal stenosis.

In females, meatal stenosis is present at birth (congenital). Less commonly, meatal stenosis may also affect adult women.

Meatal stenosis risks in female include:

• Having many endoscopic procedures (cystoscopy)
• Severe, long-term atrophic vaginitis.

Meatal stenosis is treated with surgery, called a meatoplasty. An incision is made below the meatus to open it or make it wider. Surgery is performed in the operating room under general anesthesia but may also be done using a local anesthetic in the office.

If done in the operating room, an incision is made in the meatus and dissolvable sutures are placed around the meatus. If done in the office, a small incision is made in the meatus, but does not place any sutures.

If the narrowed opening is not fixed, it can harder to urinate, and cause urinary tract infections, and swollen kidneys.

Meatal stenosis causes

In a child who is circumcised, persistent exposure of the meatus to urine and mechanical trauma from rubbing against a wet diaper results in ammoniac dermatitis, loss of meatal epithelium, and fusion of its ventral edges. This results in a pinpoint orifice at the tip of the head of the penis (glans penis) ³⁾.

Other causes of meatal stenosis include the following:

• Previous hypospadias repair
• Trauma
• Prolonged catheterization
• Balanitisxerotica obliterans (BXO), an unusual condition that causes a whitish discoloration and dry appearance of the head of the penis ⁴⁾

A 10-year retrospective series of boys with balanitis xerotica obliterans at Boston Children’s Hospital included 41 patients with a median age of 10.6 years, of whom 85% were aged 8-13 years ⁵⁾. The disease process was found to involve the prepuce, the glans, and sometimes the urethra. The most common referral diagnoses included phimosis (52%), balanitis (13%), and buried penis (10%). In 46% of the patients, circumcision was curative; 27% (11 patients) had meatal involvement that was treated by meatotomy and meatoplasty, and 22% required extensive plastic procedures of the penis, including buccal mucosal grafts.

Meatal stenosis occurs in as many as 20% of pediatric patients with balanitis xerotica obliterans. Although balanitis xerotica obliterans is difficult to treat, meatotomy typically produces a durable treatment of the meatal stenosis.

Meatal stenosis symptoms

Children with urethral meatal stenosis may have the following symptoms:

• Abnormal strength and direction of urine stream
• Urine sprays instead of single stream
• Trouble starting and keeping the flow of urine going trouble aiming the stream into the toilet
• Prolonged urination a thin stream of urine
• Trouble urinating
• Urinate often
• Bed wetting
• Bleeding (hematuria) at end of urination
• Discomfort with urination, straining or arching of the back when urinating
• Incontinence (day or night)
• Visible narrow opening in boys

Some children may have microscopic blood in the urine or pain with urination.

Meatal stenosis complications

Urinary meatal stenosis complications may include:

• Difficult-to-aim (upward-deflected), high-velocity (long-distance) stream of urine; the misdirection of the urine stream is the most important feature of the history, in that this is what is most likely related to meatal stenosis and is most reliably corrected by meatotomy or meatoplasty
• Abnormal urine stream
• Blood in the urine
• Frequent urination
• Painful urination or pain upon initiation of urination
• Urinary incontinence
• Urinary tract infections
• Damage to bladder or kidney function in severe cases

It is important to note that symptoms other than misdirection of the urinary stream (eg, pain, urinary hesitancy, and frequency) may be signs of underlying voiding dysfunction rather than results of meatal stenosis. Consequently, these symptoms may not improve after correction of the meatal stenosis.

Meatal stenosis diagnosis

In boys, a history and physical exam are enough to make the diagnosis of urinary meatal stenosis.

In girls, a voiding cystourethrogram may be done. The narrowing may also be found during a physical exam, or when a health care provider tries to place a Foley catheter. Litvak et al. ⁶⁾ report that the meatus in children younger than 1 year will accept a lubricated 5-French feeding tube; they also report that in children aged 1-6 years, an 8-French feeding tube should pass without difficulty.

Other tests may include:

• Complete blood count (CBC)
• Kidney and bladder ultrasound
• Urine analysis
• Urine culture

Meatal stenosis treatment

In females, meatal stenosis is most often treated in the provider’s office. This is done using local anesthesia to numb the area. Then the opening of the urethra is widened (dilated) with special instruments.

In boys, a minor outpatient surgery called meatoplasty or meatotomy is the treatment of choice. Meatoplasty is a simple procedure in which the ventrum of the meatus is crushed (for hemostasis) for 60 seconds with a straight clamp (the authors have found the striations of a small nontoothed bowel clamp to be ideal for this application) and then divided with fine-tipped scissors.

Dilation of the meatus may also be appropriate in some cases.

Meatal stenosis surgery

Brown et al. ⁷⁾ reported excellent results following 130 office meatotomies, with only two recurrences of meatal stenosis and one patient with bleeding requiring stitches. They also cited the cost-effectiveness of this treatment and noted good patient tolerance when a caring approach is used to reassure the child before and during the procedure. In this series, parents were encouraged to remain with the children during the operation; their presence seemed to have a calming effect.

In a retrospective study of 55 boys who underwent clinic meatotomy under topical lidocaine and prilocaine (EMLA) anesthesia, Fronczak et al. ⁸⁾ found that although their success rate was lower than that reported in earlier studies and the incidence of pain higher, it was nonetheless possible to complete all of the clinic meatotomies, and more than 90% of patients experienced resolution or improvement of their symptoms. The cost of clinic meatotomy was approximately one tenth that of meatotomy performed under general anesthesia, with no evidence of inferiority evident in the literature.

If the caregivers and the patient are cooperative, meatotomy can be performed in the office of the physician with a topical eutectic mixture of local anesthetics (EMLA cream) applied liberally over the entire glans and secured in place for at least 30 minutes with an occlusive dressing. After being in place for 30 minutes, the dressing is removed, and the penis is prepared and draped into a sterile field.

Throughout the procedure, it is important to reassure the child and tell him what is being done.

One blade of a straight clamp is introduced into the meatus, and the ventrum of the meatus is crushed (~3 mm) by closing the clamp. This provides adequate hemostasis in most cases. The crushed area is divided with a straight fine-tipped scissors, and an antibiotic ointment is applied.

After the operation, it is critical that the caregivers separate the edges of the meatus and apply ointment twice a day for 2 weeks and then once a day for another 2 weeks to prevent one side of the meatotomy from adhering to the other side. Some medical professionals recommend dilation with a lubricated feeding tube or the tip of an ointment tube (antibiotic, steroid, or combination) for a period of up to 8 weeks.

In a survey of office pediatric urologic procedures, which included meatotomy, lysis of labial adhesions, and newborn circumcision, Smith and Smith ⁹⁾ found that 95 of 99 parents stated that they were satisfied with their decision to have these procedures performed in the office, and 95% reported good outcomes (only one patient had recurrent meatal stenosis).

Mild dysuria may be present for 1-2 days after meatotomy. If dysuria results in urinary retention, placing the child in a tub of warm water may stimulate urination.

It is also important to note that spraying of the urine stream may occur for a while after surgical correction as a consequence of edema.

Depending on the age and temperament of the child, it may be preferable to perform meatotomy with the patient under sedation or general anesthesia in the operating room.

Some surgeons prefer meatoplasty, in which stitches are placed to help keep the meatus open, to meatotomy, citing research that suggests a lower recurrence rate after meatoplasty ¹⁰⁾. Meatoplasty typically requires sedation or general anesthesia.

Others have reported excellent results with meatotomy. Ultimately, the choice of approach (meatotomy, meatoplasty, or no surgery) is based on a thorough discussion among the provider, the family members, and the patient (if applicable).

Postoperative Care

After meatotomy, caregivers will typically be instructed to separate the edges of the meatus and apply antibiotic, steroid, or combination ointment twice a day for 2 weeks and then once a day for 2 more weeks. The physician may have other specific instructions, based on their usual care.

Activity

After meatotomy, instruct caregivers to dress the child in loose underwear for 24 hours. Restrict activities, such as contact sports, bicycle rides, and playground activities, for 3-4 days.

Complications

Complications include bleeding during or after meatotomy, infection, and recurrence. All of these complications are rare and typically respond readily to appropriate management.

Mild painful urination may persist for 1-2 days. Placing the child in a tub of warm water may provide relief.

Meatal stenosis prognosis

Meatal stenosis prognosis is excellent after surgery: Meatotomy or meatoplasty is typically curative of the misdirection of the urinary stream. Morbidity is limited to the clinical symptoms and complications of surgical repair, including bleeding, infection, and recurrence. If there are also voiding symptoms (e.g, pain with urination or hesitancy), these may be related to underlying voiding dysfunction, and surgical correction of meatal stenosis may not alleviate them.

Chylothorax

Chylothorax is the accumulation of chyle or lymphatic fluid in the pleural cavity around the lungs due to damage to the thoracic duct ¹⁾. Chylothorax can be seen in many conditions and trauma and cancer are the leading causes. This is a rare condition that may develop as a complication of thoracic and esophageal and thoracic surgery and hematologic malignancies. Chylothorax has no predilection for gender or age. The prevalence of after various cardiothoracic surgeries is 0.2% to 1%. Mortality and morbidity rates are approximately 10%. The diagnosis based on pleural fluid can be made by demonstration of triglycerides of more than 110 mg/dL or presence of chylomicrons. Various surgical and nonsurgical management strategies can be used for the treatment of chylothorax. Initially the treatment is conservative but if the leak does not stop, surgery is required. New surgical techniques like pleurovenous or pleuroperitoneal shunting and thoracic duct embolization have been used with success. Currently, somatostatin analogs are effectively used for its treatment.

Chyle is made primarily of chylomicrons, an aggregate of long-chain triglycerides, cholesterol esters, and phospholipids. It is also rich in lymphocytes, primarily T lymphocytes as the major cellular component with concentrations that range from 400 to 6800 cells. Chyle as similar electrolyte concentration plasma but is rich in immunoglobulins with fat-soluble vitamins. As the normal chyle production is around 2.4 liters per day, a considerable amount of chyle can accumulate in the pleural cavity in a very short period.

Chyle is the milky bodily fluid formed in the lacteal system of the intestine. The small and medium chain triglycerides consumed in the diet are easily broken to free fatty acids by the intestinal enzymes and readily absorbed into the portal circulation. However, the large molecules of complex long-chain triglycerides cannot be broken down by the intestinal lipases. They combine with phospholipids, cholesterol and cholesterol esters to form chylomicrons in the jejunum. These large molecules then get absorbed into the lymphatic system of the small intestine to form the chyle. The lymphatic drainage system of intestine will be joined by the lymphatic drainage from the lower extremities to form the thoracic duct system which ultimately drains in the system circulation. If there is the breach of the integrity of thoracic duct, the milky lipid-rich chyle leaks to the surrounding structures.

Pseudo-chylothorax or chyliform effusion

Effusions with the gross appearance similar to chylothorax, milky white are called as pseudo-chylothorax. These are less common than the classical chylothorax and have been reported being in long-standing exudate pleural effusion of various cause. They contain a high concentration of cholesterol, giving them that characteristic milky or white appearance. In long-standing exudative pleural effusion, the cell undergoes slices releasing the cholesterol from the cell membrane into the fluid which gets trapped in the pleural cavity. However, unlike classical chylothorax, they do not contain chylomicrons or long chain fatty acids. Pseudo- chylothorax is commonly associated with tuberculosis and chronic rheumatoid pleural effusion due to the high cell concentration. Other causes described and literature are yellow nail syndrome and paragonimiasis. The cholesterol concentration in pseudo-chylothorax is typically more than 200 mg; triglycerides level is less than 110 mg/dL and cholesterol to triglycerides ratio is always more than 1. Cholesterol crystals may be visible in dried slides from pleural fluid when viewed under polarized light and appear as rectangle plates with notched edges. The presence of cholesterol crystals is virtually diagnostic of pseudo-chylothorax.

What is the thoracic duct

The thoracic duct is the main lymphatic channel for the return of chyle to the venous system. The thoracic duct drains lymph from both lower limbs, abdomen (except the convex area of the liver), left hemithorax, left upper limb and left face and neck. The thoracic duct is present in all individuals. The thoracic duct most inferior part, located at the union of the lumbar and intestinal trunks, is the cisterna chyli which lies on the bodies of vertebrae L1 and L2 (range T10-L3) (Figure 1). From there, the thoracic duct ascends along the vertebral bodies. In the superior thorax, the thoracic duct turns left and empties into the venous circulation at the junction of the left internal jugular and left subclavian veins. The thoracic duct is often joined by the left jugular, subclavian, and/or bronchomediastinal trunks just before it joins with the venous circulation. Alternatively, any or all of these three lymph trunks can empty separately into the nearby veins. When it is joined by the three trunks, the thoracic duct drains three-quarters of the body: the left side of the head, neck, and thorax; the left upper limb; and the body’s entire lower half (see Figure 2).

After leaving the lymph nodes, the largest collecting lymphatic vessels converge to form lymph trunks. The lymph trunks drain into the largest lymphatic vessels, the lymph ducts (Figures 1 and 2). Whereas some individuals have two lymph ducts, others have just one. Lymphatic ducts empty lymph fluid into the venous system. The two lymphatic ducts of the body are the right lymphatic duct and the thoracic duct. The thoracic duct is the larger of the two and responsible for lymph drainage from the entire body except for the right sides of the head and neck, the right side of the thorax, and the right upper extremity which are primarily drained by the right lymphatic duct (see Figure 2) ²⁾.

Figure 1. Thoracic duct

Where does the thoracic duct drain?

The thoracic duct delivers lymph into junction between left subclavian and left internal jugular veins.

Right lymphatic duct delivers lymph into junction between right subclavian and right internal jugular veins.

Figure 2. Thoracic duct drainage

Chylothorax causes

The cause of chylothorax can be classified into three broad categories, spontaneous (non-traumatic), traumatic, and idiopathic. Historically, non-traumatic chylothorax was the more common cause for chylothorax accounting for two-thirds of all cases. Recently, traumatic chylothorax, particularly postoperative chylothorax, accounts for more than 50% of all cases described in the literature ³⁾.

Non-traumatic chylothorax can be due to the following:

1. Congenital chylothorax can be seen as an isolated condition or in association with other lymphatic abnormalities like lymphangiectasis, lymphangiomatosis, tuberous sclerosis, congenital heart disease, or chromosomal abnormalities such as trisomy 21 or Turner syndrome.
2. Neoplastic chylothorax is the most common cause of non-traumatic chylothorax. Various cancers like lymphoma, chronic lymphoid leukemia, lung cancer, esophageal cancer or metastatic carcinoma have been implicated in chylothorax. Interestingly, there is a relative decrease in the incidence of chylothorax in patients with lymphoma in the recent time. This is likely due to early diagnosis and treatment of lymphoma avoiding the late complication of chylothorax.
3. Infectious chylothorax is mostly seen in developing countries as a complication of tuberculous lymphadenitis. Other infections known to cause chylothorax are aortitis, histoplasmosis, and filariasis.
4. Rare causes of chylothorax reported in the literature are due to cattleman disease, sarcoidosis, Kaposi sarcoma, yellow nail syndrome, Noonan syndrome, Down syndrome, Waldenstrom macroglobulinemia, macroglobulinemia, amyloidosis, venous thrombosis, thoracic radiation, goiter. All these reported diseases will lead to obstruction or destruction of thoracic duct causing chylothorax. Even parental nutrition therapy is implicated in chylothorax. Rapid infusion of total parenteral nutrition containing a high concentration of triglycerides can overwhelm the draining capacity of the thoracic duct leading to leakage of chyle to this surrounding pleural space and cause chylothorax.

Traumatic chylothorax occurs due to disruption of thoracic duct anywhere in its course in the mediastinum. They can occur as a complication of the surgical procedure or can follow blunt or penetrating trauma to the chest.

1. Post-operative chylothorax constitutes the most common cause of chylothorax in the modern medicine. The risk of postoperative chylothorax depends on the type of surgery done. Esophagostomy carries the highest risk of 5% to 10% postoperative chylothorax, followed by lung resection with mediastinal lymph node dissection with 3% to 7% risk. Other surgeries like mediastinal tumor resection, thoracic aneurysm repair, sympathectomy, any surgeries in the lower neck and mediastinum carry a risk of chylothorax. Ingestion of milk before the surgery can lead to better visualization of the thoracic duct in the surgical field by causing whitish discoloration of the thoracic duct. It is proposed on of the preoperative steps to prevent postoperative chylothorax, although this has not been formally studied. Nonsurgical posttraumatic chylothorax is also described after central line placement, pacemaker implantation, embolization of the pulmonary arteriovenous malformation.
2. Blunt trauma to the chest or thoracic spine can disrupt the thoracic duct without any obvious injury to the surrounding structure leading to chylothorax. Chylothorax is also described after blasting injuries. Chylothorax is also described following a trivial injury like coughing and sneezing.
3. Penetrating injuries of the chest like gunshot injury and stab injury can directly damage the thoracic duct leading to chylothorax

Idiopathic causes account for nearly 10% of all cases of chylothorax. Chylothorax is considered to be idiopathic after extensive investigation does not reveal any known cause for it. Most of these idiopathic cases are related to undiagnosed malignancy.

Chylothorax symptoms

The clinical features of chylothorax depend upon the cause. Small chylothorax can be asymptomatic and is detected incidentally. Patient with large chylothorax usually presents with signs and symptoms caused by the mechanical effect of compression on lung expansion. Progressive breathlessness decreases exercise capacity; chest pressure is common presenting complaints. Fever and chest pain are usually absent. The patient can accumulate a large amount of chylothorax without any complaints if the fluid recommendation is gradual and respiratory system gets acclimatized to it. Post-traumatic chylothorax can present up to 10 days after the inciting trauma. In the post-surgical patient’s, the chylothorax me the first detected as a pleural effusion or by persistent drainage from the indwelling chest tubes.

Chylothorax symptoms may include:

• Shortness of breath (dyspnea)
• Fast breathing (tachypnea)
• Symptoms of pleural effusion:
  • Chest pain, usually a sharp pain that is worse with cough or deep breaths
  • Cough
  • Fever and chills
  • Hiccups
  • Rapid breathing
  • Shortness of breath

On physical examination, findings of decreased breath sounds and dullness to percussion may be present depending on the size and location of fluid. Eighty percent of chylothorax cases are unilateral. Due to the location of the thoracic duct, the right side is more common than the left, accounting for two-thirds of the total cases.

Chylothorax diagnosis

Further evaluation of chylothorax depends on the suspected cause and the availability of resources.

• Chest x-ray: Chest radiographs routinely done to evaluate dyspnea, particularly in postoperative and traumatic patients can detect unilateral pleural effusion. Chylothorax appears as homogenous density obligating the costophrenic and cardio phrenic angle. A routine chest x-ray cannot differentiate chylothorax from other causes of pleural effusion.
• Thoracic ultrasound: Ultrasound is being increased used for the evaluation of patients with pleuropulmonary pathology. Like other effusion, it appears as an isodense echoic region without any septation or loculation. However, ultrasound cannot differentiate chylothorax from other causes of pleural effusion.
• Chest CT scan: CT scan is more sensitive than chest x-ray and ultrasound for the diagnosis of chylothorax. Routine CT chest can show cisterna chyli in around 2% of cases. Due to the high amount of fat content, it is seen as a low-attenuation tubular area in the posterior mediastinum. CT scan also may show the cause of chylothorax like mass lesions or obstructive lesion in the posterior mediastinum, thoracic malignancy, or evidence of trauma.
• Magnetic resonance imaging (MRI): MRI of the chest can show cisterna chyli in 100% of the cases and can be used for better evaluation of chylothorax. However, as MRI of the chest is not an optimal investigation for the thoracic pathology, it is rarely used in clinical practice.
• Conventional lymphangiography: Lymphangiography is a technique used to delineate lymphatic system. In the modern medicine, this test is rarely used due to the availability of less invasive alternatives which are equally precise. In this technique dye like methylene blue that stains the lymphatics is injected into the web spaces between the toes. A longitudinal or transverse cutaneous incision at the base of the first metatarsal bone is made to expose a lymphatic vessel with blue staining after dissection of the surrounding tissue. The isolated lymphatic vessel is then cannulated using a 30 gauge needle. After accessing the lymphatic vessel, 10 mL of Lipiodol is slowly injected at the rate of 0.2 mL/min to 0.4 mL/min. Serial fluoroscopic spot images are obtained upwardly every 5 to 10 minutes over the course of the injected Lipiodol. If Lipiodol does not reach the area of interest, normal saline at the same rate can be injected to push Lipiodol further into the area of interest. This can reliably show any leakage in the thoracic duct leading to chylothorax. A further modification of this called nodal lymphangiogram has been developed in the recent time. A targeted lymph node is selected, and ethiolised oil is injected into the cortex of the lymph node at a rate 1 ml per min for total 10 ml. Serial spot radiographs of the pelvis, abdomen, and thorax are then acquired to follow the progression of the dye. This Intranodal lymphoscintigraphy is sensitive, technically easier and also has fewer complications. Lymphogram can be therapeutic in up to 40% of cases. The high-density oil used in the procedure can close in leak during the procedure.
• Nuclear Lymphoscintigraphy: This technique has been used more commonly than the traditional lymphangiography to delineate lymphatic system. In this procedure, Tc99m labeled human diethylenetriaminepentaacetic acid (HAS-DTPA) is injected to the subcutaneous lesions of the dorsum of the foot bilaterally. Sequentially anterior and posterior images of the chest are obtained using a gamma camera to identify the leak. This technique can be combined integrated low-dose CT scan with single-photon emission computed tomography combined to get the more accurate SPECT/CT images. Radio nuclear localization using lymphoscintigraphy has been shown to correlate with traditional lymphangiography and surgical localization of leak.

Laboratory testing

Thoracentesis and analysis of the fluid is the diagnostic study of choice for chylothorax: All fluid samples should be sent for white blood cell count, differential, glucose, Lactic dehydrogenase, total protein level, cytology, microbiology smear, and culture. If chylothorax is suspected based on the color of the fluid, then additional tests like Ph, total triglycerides levels, and cholesterol levels should be sent.

• Color: Based on the amount of fat content in chylothorax, the appearance of fluid can (white) milky, serous or serosanguineous. The absence of milky appearance does not exclude a chylothorax. If the fluid is kept undisturbed for some time on centrifugation, the supernatant of fluid is clear. In empyema, the supernatant is not clear.
• Cell counts: Chyle is rick in lymphocytes accounting for 80% of all the cells. The lymphocytes are predominately polyclonal population of T cells.
• Lipid analysis: Measurement of the pleural fluid triglyceride content is key to the diagnosis of suspected chylothorax. Unlike in any other body fluids, chylothorax is very rich in large chain fatty acids absorbed from the small intestine. The pleural fluid triglyceride concentration greater than 110 mg/dL confirms the diagnosis of chylothorax. However, 15% of chylothorax are known to have triglyceride concentration less than 110 mg depending on the time of last meal and the fat content of the diet. If clinical suspicion of chylothorax is high, then lipid electrophoresis of the pleural fluid should be done. Detection of chylomicrons in the pleural fluid by lipoprotein electrophoresis confirms chylothorax. Typically, the total cholesterol level in a chylothorax is less than 200 mg/dl.
• Others compositions: The other fluid composition is similar to plasma. Chylothorax is usually alkaline with pH from ranging from 7.4 to 7.8.

Chylothorax treatment

The appropriate management of chylothorax depends on the cause and includes one or more of many interventions like dietary modification, pleurodesis, and thoracic duct ligation. Recently, the use of somatostatin/octreotide, midodrine and sirolimus to prevent chyle formation has been used. New surgical techniques like pleurovenous or pleuroperitoneal shunting and thoracic duct embolization have been used with success. Most patients benefit from a staged care plan from least invasive options to more invasive techniques ⁴⁾, ⁵⁾.

Dietary therapy

As chylous fluid is formed the long chain fatty acids; decreasing or eliminating these fatty acids from the diet will lead to decreased chyle drainage spontaneously closure of leak. Diet with less than 5 kcal of fat per meal is used in this technique. This can effectively reduce chyle formation, but over long periods, it will lead to fat deficiency and malnutrition. In fact, venous fat hemorrhage can remedy some of the shortcomings of this therapy. Small chain and medium chain fatty acids can be provided in a diet, and long chain fatty acids can be supplemented through intravenously.

Thoracentesis

Intermittent therapeutic thoracentesis or use of an indwelling catheter for home drainage is typically used in the initial management of non-traumatic and nonsurgical traumatic effusions to relieve dyspnea caused by the pleural fluid. This technique can be effectively used if there is a gradual recommendation of her pleural fluid. A chest tube can be used in postsurgical chylothorax. Prolonged drainage of pleural fluid comes with risk of significant malnutrition and loss immunoglobulins putting the patient at risk of infections. Hence continuous pleural fluid drainage should typically be restricted to less than 2 weeks. Surgical intervention is recommended if the pleural fluid drainage exceeds 1.5 L per day.

Pleurodesis

This should be reserved for patients who continue to reaccumulate fluid despite dietary modification and repeated thoracenteses. Pleurodesis can be accomplished by tall installation of a chest tube drainage by video-assisted thoracic surgery with talc insufflation. In some cases, this procedure can remedy up to 80% of chylothorax. Concomitant ligation of the thoracic duct to prevent further chyle formation during surgical pleurodesis is also done with high success.

Thoracic duct ligation

This invasive procedure is done using video-assisted thoracic surgery and can be useful in patients where dietary modification and pleurodesis has failed. Lymphedema is a known complication of thoracic duct ligation but usually, resolves after several months due to collateral lymphatic venous communications.

Thoracic duct embolization and disruption

Percutaneous catheterization and embolization are needle disruption of the thoracic duct and cisterna chyli along with prominent retroperitoneal lymphatic ducts have been used increasingly in both traumatic and nontraumatic chylothorax. Initial pedal lymphangiogram follows fluoroscopic visualization of the large retroperitoneal lymphatics, which has been accessed by transabdominal percutaneous needle cannulation. After cannulation of the cisterna chyli, the catheter is advanced with thoracic duct installation of contrast to localize the leak. The affected thoracic segment is then embolized with coils and surgical clue.

Emerging therapies

Somatostatin and octreotide can decrease the gastric, pancreatic, and biliary secretions and reduce the total flow of gastric lymphatics. Because the reduction chyle formation and flow rates, they can spontaneously remedy the leak in thoracic duct. This technique is reported to be useful in many cases of spontaneous chylothorax, congenital chylothorax, postoperative chylothorax and chylothorax due to malignancy. The optimal dose and duration of treatment is not clear.

Sirolimus used for the effect to treatment of lymphangiomyomatosis is also known to decrease the incidence of chylothorax in these set of patients.

Pleuroperitoneal or pleural venous shunt. Shunting of chylous pleural fluid into the venous system or peritoneal cavity can also resolve chylothorax. Two types of pleuroperitoneal shunts are available. Denver pleuroperitoneal shunt is an active pump which requires manual pumping and Le Veen pleuroperitoneal shunt is a passive pump. The main advantage of the shunt procedure is the recycling of the nutritionally rich chyle to the body. Pleural venous shunting of chylous fluid from the pleural space to the subclavian jugular vein has been performed successfully in patients with yellow nail syndrome and postsurgical chylothorax.

Chylothorax prognosis

The prognosis in chylothorax varies in accordance with the condition’s underlying cause. Overall, the outcome for chylothorax from benign causes is good but in cases of cancer, the outcomes are poor ⁶⁾.

Transient tachypnea of the newborn

Transient tachypnea of the newborn (TTN) is a medical term for a mild self-limited respiratory problem of babies that begins after birth and lasts about three days. Other terms for TTN are “wet lungs” or type 2 respiratory distress syndrome. “Transient” means means temporary or it doesn’t last long, usually, less than 24 hours. “Tachypnea” means to breathe quickly or fast breathing rate.

Some newborns have very fast or labored breathing in the first few hours of life because of a lung condition called transient tachypnea of the newborn (TTN). About 1 to 2 percent of all newborns develop TTN ¹⁾. Although premature babies can have TTN, most babies with this problem are full-term. The incidence of transient tachypnea of the newborn is inversely proportional to gestation age and affects approximately 10% of infants delivered between 33 and 34 weeks, approximately 5% between 35 and 36 weeks, and less than 1% in term infants ²⁾.

Transient tachypnea of the newborn may be more likely to develop in babies delivered by cesarean section because the fluid in the lungs doesn’t get squeezed out as in a vaginal birth.

Babies with transient tachypnea are closely watched in the hospital, and some might need extra oxygen for a few days. Most babies make a full recovery. TTN usually does not have any lasting effects on a child’s growth or development.

TTN usually goes away by the time a baby is 3 days old. Until that happens, doctors can help the baby get enough oxygen and nutrition if he or she needs it. Treatments might include:

• Extra oxygen
• An intravenous (IV) feeding tube
• Antibiotics

Transient tachypnea of the newborn causes

It is thought that slow absorption of the fluid in the fetal lungs causes TTN ³⁾. This extra fluid in their lungs makes taking in oxygen harder and your baby breathes faster to compensate.

Before birth, a developing fetus does not use the lungs to breathe — all oxygen comes from the blood vessels of the placenta. During this time, the baby’s lungs are filled with fluid.

As the baby’s due date nears, the lungs begin to absorb the fluid. Some fluid also may be squeezed out during birth as the baby passes through the birth canal. After delivery, as a baby breathes for the first time, the lungs fill with air and more fluid is pushed out. Any remaining fluid is then coughed out or slowly absorbed through the bloodstream and lymphatic system.

Transient tachypnea of the newborn is more common in:

• Premature babies because their lungs are not fully developed
• Babies born by rapid vaginal deliveries or C-sections without labor. They don’t go through the usual hormonal changes of labor, so don’t have time to absorb much fluid.
• Babies whose mothers have asthma or diabetes.

Risk factors for developing transient tachypnea of the newborn

• Maternal risk factors include delivery before completion of 39 weeks gestation, a cesarean section without labor, gestational diabetes, and maternal asthma ⁴⁾.
• Fetal risk factors include male gender, perinatal asphyxia, prematurity, small for gestational age, and large for gestational age infants ⁵⁾.

Transient tachypnea of the newborn pathophysiology

Fetal Lung

• The fetal pulmonary epithelium secretes alveolar fluid at around 6 weeks of gestation ⁶⁾.
• Chloride ions in the interstitium enter the pulmonary epithelial cell through the active transport of sodium, potassium, and chloride into cells (Na-K-2Cl transporter) which, in turn, are secreted into the alveolus through various chloride channels.
• Sodium follows the chloride ions through para-cellular pathways, and water is transported across the cells via aquaporin ⁷⁾.
• Volume of fetal lung is maintained by the larynx, which acts as a one-way valve, allowing only outflow of fluid.

Neonatal Lung

• Passive movement of sodium through epithelial sodium channels (ENaC) is believed to be the principle mechanism of reabsorption of fetal lung fluid with starling forces and thoracic squeeze playing a minor role in clearance.
• With the onset of labor, maternal epinephrine ⁸⁾ and glucocorticoids activate the epithelial sodium channels on the apical membranes of type II pneumocytes.
• Sodium in the alveolus is transported passively across the ENaC proteins which in turn is actively transported back to the interstitium by the Na+/K+-ATPase pump ⁹⁾.
• An osmotic gradient is created which allows chloride and water to follow and be absorbed into pulmonary circulation and lymphatics.

Transient tachypnea of the newborn symptoms

Transient tachypnea of the newborn presents within the first few minutes to hours after birth.

The following are the most common symptoms of transient tachypnea of the newborn. However, each baby may experience symptoms differently.

TTN symptoms may include:

• very fast, labored breathing of more than 60 breaths/minute
• grunting sounds when the baby breathes out (exhales) ¹⁰⁾
• flaring of the nostrils or head bobbing
• skin pulling in between the ribs or under the ribcage with each breath also known as retractions
• bluish skin around the mouth and nose called cyanosis.

Other occasional exam findings:

• Crackles, diminished or normal breath sounds on auscultation
• Tachycardia
• Cyanosis
• Barrel-shaped chest because of hyperinflation

Transient tachypnea of the newborn complications

Air leaks and pneumothoraces are other rare complications.

Longitudinal studies have shown an association between TTN and subsequent development of asthma ¹¹⁾. Epidemiological studies showed that there is a relationship between TTN and the development of asthma in children ¹²⁾. This means that TTN alone is not only a condition itself but also a risk factor for future asthma in later childhood. Despite this, pinpointing a relationship between them is rather complicated due to cause or risk factors ¹³⁾.

Transient tachypnea of the newborn diagnosis

Doctors usually diagnose transient tachypnea of the newborn in the first few hours after a baby is born. Often, TTN is diagnosed when symptoms suddenly resolve by the third day of life.

Duration of respiratory distress is the principal determinant for diagnosis of TTN. If distress resolves within the first few hours of birth, it can be labeled as “delayed transition.” Six hours is an arbitrary cutoff between “delayed transition” and TTN because by this time baby might develop issues with feeding and might require further interventions ¹⁴⁾. TTN is usually a diagnosis of exclusion and hence any tachypnea lasting over 6 hours requires workup to rule out other causes of respiratory distress.

A doctor will examine the baby and also might order one or all of these tests:

• Chest X-ray. This safe and painless test uses a small amount of radiation to take a picture of the chest. Doctors can see if the lungs have fluid in them. On the chest x-ray, the lungs show a streaked appearance (prominent perihilar vascular markings, edema of interlobar septae or fluid in the fissures) and appear over-inflated ¹⁵⁾. However, it may be difficult to tell whether the problem is TTN or another kind of respiratory problem such as hyaline membrane disease.
• Pulse oximetry. This painless test measures how much oxygen is in the blood. A small piece of tape with an oxygen sensor is placed around a baby’s foot or hand, then connected to a monitor.
• Complete blood count (CBC), blood culture, C-reactive protein (CRP) and lactate. This blood tests check for signs of infection (neonatal sepsis).
• Preductal and postductal saturations: to rule out differential cyanosis
• ABG (arterial blood gas) analysis may show hypoxemia and hypocapnia due to tachypnea; hypercapnia is a sign of fatigue or air leak.

Other workups to consider:

• Ammonia level in the setting of lethargy and metabolic acidosis to rule out inborn errors of metabolism
• Echocardiography to rule out congenital cardiac defects in patients with differential cyanosis or persistent tachypnea for over 4 to 5 days.

Transient tachypnea of the newborn differential diagnosis

• Pneumonia
• Respiratory distress syndrome
• Aspiration syndromes: meconium, blood or amniotic fluid
• Pneumothorax
• Left-to-right cardiac shunt defects with failure
• Persistent pulmonary hypertension
• Central nervous system (CNS) irritation or disease: Subarachnoid hemorrhage, hypoxic-ischemic encephalopathy
• Inborn errors of metabolism
• Congenital malformations: Congenital diaphragmatic hernia, cystic adenomatoid malformations

Transient tachypnea of the newborn treatment

Given TTN is a self-limited condition, supportive care is the mainstay of treatment.

• Rule of 2 hours: Two hours after onset of respiratory distress, if an infant’s condition has not improved or has worsened or if fraction of inspired oxygen (concentration of oxygen that a person inhales or FiO2) required is more than 0.4 or chest x-ray is abnormal, consider transferring infant to a center with a higher level of neonatal care ¹⁶⁾.
• Routine NICU care including continuous cardiopulmonary monitoring, maintenance of neutral thermal environment, securing intravenous (IV) access, blood glucose checks, and observation for sepsis should be provided.

Babies with TTN are watched closely and may go to a neonatal intensive care unit (NICU) or special care nursery. There, doctors check babies’ heart rates, breathing rates, and oxygen levels to make sure breathing slows down and oxygen levels are normal.

Specific treatment for transient tachypnea of your newborn may include:

• Supplemental oxygen given by mask on your baby’s face or by placing your baby under an oxygen hood
• Blood tests to measure blood oxygen levels
• Continuous positive airway pressure (CPAP) – a mechanical breathing machine that pushes a continuous flow of air or oxygen to the airways to help keep tiny air passages in the lungs open

Tube feedings may also be necessary if your baby’s breathing rate is too high, because of the risk of aspiration of the food.

Once TTN goes away, your baby usually recovers quickly and has no increased risk for additional respiratory problems.

Breathing help

Some babies with TTN need extra oxygen. They get this through a small tube under the nose called a nasal cannula .

A baby who gets extra oxygen but still struggles to breathe might need continuous positive airway pressure (CPAP) to keep the lungs from collapsing. With CPAP, a machine pushes a steady stream of pressurized air or oxygen through a nasal cannula or mask. This helps keep the lungs open during breathing.

• Oxygen support may be required if pulse oximetry or ABG suggest hypoxemia.
• An oxygen hood is the preferred initial method; however, nasal cannula, CPAP can also be used.
• Concentration should be adjusted to maintain oxygen saturation in low 90s.
• Endotracheal intubation and requirement of extracorporeal membrane oxygenation (ECMO) support is usually uncommon but should always be considered in patients with declining respiratory status.
• Arterial blood gas (ABG) analysis should be repeated, and pulse oximetry monitoring should be continued until signs of respiratory distress have resolved.

Nutrition

• Neonates’ respiratory status is the usual determinant for the degree of nutritional support required.
• Tachypnea of over 80 breaths per minute with associated increased work of breathing often makes it unsafe for the infant to receive oral feeds.
• Such infants should be kept nil per oral (NPO), and intravenous (IV) fluids should be started at 60 to 80 ml per kg per day.
• If respiratory distress is resolving, diagnosis is certain and respiratory rate is less than 80 breaths per minute; enteral feeds can be started.
• Enteral feeds should always be started slowly with progressive increments in volume of feeds until tachypnea has completely resolved

Good nutrition can be a problem when a baby is breathing so fast that he or she can’t suck, swallow, and breathe at the same time. If so, intravenous (IV) fluids can keep the baby hydrated while preventing blood sugar from dipping too low.

If your baby has TTN and you want to breastfeed, talk to your doctor or nurse about pumping and storing breast milk until your baby is ready to feed. Sometimes babies can get breast milk or formula through a:

• nasogastric (NG) tube: a small tube placed through the baby’s nose that carries food right to the stomach
• orogastric (OG) tube: a small tube placed through the baby’s mouth that carries food right to the stomach

If your baby has one of these tubes, ask the doctor about providing breast milk for your baby.

Symptoms of transient tachypnea usually get better within 24–72 hours. A baby can go home when breathing is normal and he or she has been feeding well for at least 24 hours.

Infectious

Since TTN may be difficult to distinguish from early neonatal sepsis and pneumonia, empiric antibiotic therapy with ampicillin and gentamicin should always be considered.

Medications

• Randomized control trials studying the efficacy of furosemide ¹⁷⁾ or racemic epinephrine ¹⁸⁾ in TTN showed no significant difference in duration of tachypnea or length of hospital stay compared with controls
• Salbutamol (inhaled beta2-agonist) has been shown to decrease the duration of symptoms and hospital stay; however, more evidence-based studies are needed to confirm its efficacy and safety ¹⁹⁾.

Transient tachypnea of the newborn prognosis

Overall prognosis is excellent with most of the symptoms resolving within 48 hours of onset.

In some case reports, malignant TTN has been reported in which affected newborns develop persistent pulmonary hypertension due to a possible elevation of pulmonary vascular resistance due to retained lung fluid ²⁰⁾.

What is meconium aspiration

Meconium aspiration occurs when a baby breathes in amniotic fluid containing meconium (the baby’s first stools). Meconium is passed into the amniotic fluid in about 10 percent of births. It usually occurs in babies born at term (37 to 41 weeks) or post-term (after 42 weeks).

Meconium particles in the amniotic fluid can block small airways and prevent the exchange of oxygen and carbon dioxide after birth. Some babies have immediate respiratory distress and have to be resuscitated at birth. Others develop respiratory distress within a few hours.

Some babies with meconium aspiration need a mechanical ventilator (breathing machine) because of the difficulty breathing. The plugged airways may cause air to be trapped and leak into the tissues in and around the lungs. Infection can also occur causing pneumonia. Although the condition often improves within a few days, severe meconium aspiration, and the respiratory problems it causes, may lead to death in a small number of babies.

What causes meconium aspiration?

Before or during labor, the fetus sometimes passes the meconium stool into the amniotic fluid. It is not clearly understood why this happens. It may be a natural event, but it is also thought to be related to fetal distress in some babies. When the thick meconium mixes into the amniotic fluid, it is swallowed and breathed into the airway of the fetus. As the baby takes the first breaths at delivery, meconium particles enter the airway and can be aspirated (inhaled) deep into the lungs.

Meconium aspiration prevention

To prevent problems that lead to meconium being present, stay healthy during pregnancy and follow your health care provider’s advice.

Your doctor will want to be prepared for meconium being present at birth if:

• Your water broke at home and the fluid was clear or stained with a greenish or brown substance.
• Any testing done during your pregnancy indicates there may be problems present.
• Fetal monitoring shows any signs of fetal distress.

Early identification of meconium aspiration is essential to preventing severe aspiration problems. A technique called amnioinfusion is sometimes used during labor with meconium-stained amniotic fluid. This procedure uses a small tube inserted into the uterus through the vagina. Sterile fluid is then infused through the tube to help dilute the thick meconium.

Meconium aspiration symptoms

Meconium in the amniotic fluid gives the fluid a greenish color. This is called meconium staining. Babies who have been exposed to meconium in the amniotic fluid for a long time may have yellowed skin and nails.

The following are the most common symptoms of meconium aspiration. However, each baby may experience symptoms differently. Symptoms may include:

• Rapid breathing
• Retractions (pulling in of the chest wall)
• Grunting sounds with breathing
• Cyanosis (blue coloring)
• Overdistended chest because of trapped air

The symptoms of meconium aspiration may resemble other conditions or medical problems. Always consult your baby’s doctor for a diagnosis.

Meconium aspiration diagnosis

The presence of meconium in the amniotic fluid is key to the diagnosis. A chest X-ray also helps diagnose meconium aspiration. X-rays are a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

Meconium aspiration treatment

Early detection is key. A fetal monitor can detect if your baby is experiencing stress. If your doctor thinks your baby may have inhaled meconium, a special care team should be present during delivery. If after the baby is born, he’s active and crying, no treatment is necessary.

However, if the baby has trouble breathing, the doctor will quickly need to clear away as much meconium as possible to decrease the amount of meconium the baby inhales. He’ll suction the mouth, nose, and throat. Then he’ll insert a tube called a laryngoscope down the baby’s throat and into the trachea to remove any meconium there.

Specific treatment for meconium aspiration will be determined by your child’s doctor based on the following:

• The amount and thickness of the meconium
• The length of time the baby was exposed
• The degree of respiratory distress

At delivery, treatment may include:

• Suctioning of the upper airways (nose, mouth, and throat)
• Suctioning of the lower airways through an endotracheal tube (ET) placed in the windpipe, although this is not recommended routinely unless it is necessary
• Supplemental oxygen given by face mask or mechanical ventilator

If your baby is not breathing or has a low heart rate, a facemask with oxygen can help inflate the baby’s lungs and help him or her breathe. Your baby may need to be monitored closely in a newborn intensive care unit (NICU). In the NICU, he or she may get:

• Oxygen therapy.
• Antibiotics to treat infection.
• Surfactant (a substance that helps the lungs expand properly).
• A radiant warmer to control his temperature.
• Frequent blood tests to see if he’s getting enough oxygen.

Meconium aspiration long term effects

In most cases of meconium-stained fluid, the outlook is excellent and there are no long-term health effects. While rapid breathing may continue for several days, it’s rare for a baby with meconium aspiration syndrome to experiences any long-term damage. However, babies who experienced meconium aspiration syndrome may be at higher risk for an asthma-like disease called “reactive airway disease.” This can cause wheezing, coughing, and shortness of breath.

• Only about one half of babies with meconium-stained fluid will have breathing problems and only about 5% will have meconium aspiration syndrome.
• Babies may need extra support with breathing and nutrition in some cases. This need will often go away in 2 to 4 days. However, rapid breathing may continue for several days.
• Meconium aspiration syndrome rarely leads to permanent lung damage.

Meconium may be present at birth in the amniotic fluid because there is a serious problem with the blood flow to and from the lungs. This is called persistent pulmonary hypertension of the newborn.

Meconium aspiration syndrome

Meconium aspiration syndrome refers to breathing problems that a newborn baby may have when:

• There are no other causes, and
• The baby has passed meconium (stool) into the amniotic fluid during labor or delivery

Meconium aspiration syndrome may occur if the baby breathes in (aspirates) this fluid into the lungs. Meconium aspiration syndrome can happen before, during, or after labor and delivery.

Meconium aspiration syndrome causes

Meconium is the early feces (stool) passed by a newborn soon after birth, before the baby starts to feed and digest milk or formula.

In some cases, the baby passes meconium while still inside the uterus. This can happen when babies are “under stress” due to a decrease in blood and oxygen supply. This is often due to problems with the placenta or the umbilical cord.

Once the baby passes the meconium into the surrounding amniotic fluid, they may breathe it into the lungs. This may happen:

• While the baby is still in the uterus
• During delivery
• Immediately after birth

The meconium can also block the infant’s airways right after birth. It can cause breathing problems due to swelling (inflammation) in the baby’s lungs after birth.

Risk factors that may cause stress on the baby before birth include:

• “Aging” of the placenta if the pregnancy goes far past the due date (past 40 weeks)
• Decreased oxygen to the infant while in the uterus
• Diabetes in the pregnant mother
• Difficult delivery or long labor
• High blood pressure in the pregnant mother
• Poor intrauterine growth.

Meconium aspiration syndrome symptoms

Most babies who have passed meconium into the amniotic fluid do not breathe it into their lungs during labor and delivery. They are unlikely to have any symptoms or problems.

Babies who do breathe in meconium fluid may have the following:

• Bluish skin color (cyanosis) in the infant
• Working hard to breathe (noisy breathing, grunting, using extra muscles to breathe, breathing rapidly)
• No breathing (lack of respiratory effort, or apnea)
• Limpness at birth

Newborn babies with meconium aspiration syndrome have trouble breathing. They may breathe too quickly, grunt when they breathe, or use extra muscles to catch their breath. Their skin may have a bluish tint due to lack of oxygen, or a greenish tint from meconium staining. Their blood pressure may also be too low.

Meconium aspiration syndrome diagnosis

Before birth, the fetal monitor may show a slow heart rate that is slower than expected. During delivery or at birth, meconium can be seen in the amniotic fluid and on the infant.

The infant may need help with breathing or heartbeat right after birth. They may have a low Apgar score.

The health care team will listen to the infant’s chest with a stethoscope. This may reveal abnormal breath sounds, especially coarse, crackly sounds.

A blood gas analysis will show:

• Low (acidic) blood pH
• Decreased oxygen
• Increased carbon dioxide

A chest x-ray may show patchy or streaky areas in the infant’s lungs.

Meconium aspiration syndrome treatment

A special care team should be present when the baby is born if traces of meconium are found in the amniotic fluid. This happens in more than 10% of normal pregnancies. If the baby is active and crying, no treatment is needed.

If the baby is not active and crying right after delivery, the team will:

• Warm and maintain normal temperature
• Dry and stimulate the baby

This intervention is often all babies need to begin breathing on their own.

If the baby is not breathing or has a low heart rate:

• The team will help the baby breathe using a face mask attached to a bag that delivers an oxygen mixture to inflate the baby’s lungs.
• The infant may be placed in the special care nursery or newborn intensive care unit in order to be watched closely.

Other treatments may include:

• Antibiotics to treat possible infection.
• Breathing machine (ventilator) if baby is unable to breathe on their own or needs a large amount of extra oxygen.
• Oxygen to keep blood levels normal.
• Intravenous (IV) nutrition — nutrition through the veins if breathing problems are keeping baby from being able to feed by mouth.
• Radiant warmer to maintain body temperature.
• Surfactant to help lungs exchange oxygen. This is only used in more severe cases.
• Nitric oxide (also referred to as NO, an inhaled gas) to help blood flow and oxygen exchange in the lungs. This is only used in severe cases.
• ECMO (extracorporeal membrane oxygenation) is a kind of heart/lung bypass. It may be used in very severe cases.

What is vesicoureteral reflux

Vesicoureteral reflux is a condition in which urine abnormally flows backward from the bladder to one or both ureters, thin tubes that connect the kidneys to the bladder and sometimes to the kidneys. Vesicoureteral reflux is most common in infants and young children. Most children don’t have long-term problems from vesicoureteral reflux.

Normally, urine flows down the urinary tract, from the kidneys, through the ureters, into the bladder, where it is stored until urination occurs. The ureters normally have a one-way flap valve to prevent urine from traveling in both directions. During normal urination, the bladder muscle contracts and the sphincter muscle relaxes (this is the control mechanism), allowing the urine to leave the bladder through the urethra. With vesicoureteral reflux, some urine will flow back up—or reflux—through one or both ureters and may reach the kidneys.

Approximately 1% of healthy children have vesicoureteral reflux. About 75% of children with vesicoureteral reflux are girls. It is usually diagnosed around 2 to 3 years of age, but it can be discovered at any time.

Doctors usually rank vesicoureteral reflux as grade 1 through 5. Grade 1 is the mildest form of the condition, and grade 5 is the most serious. If backed-up urine reaches the kidneys, it can lead to infection, scarring, and even long-term kidney damage if left untreated. Fortunately, most kids with vesicoureteral reflux don’t have a severe case of it and outgrow it with no long-term complications.

Treatment depends on the severity of the vesicoureteral reflux. In mild cases, no treatment is necessary. Moderate to severe cases may be treated with antibiotic medicines to prevent infection. In cases where kids have infections and fevers along with the vesicoureteral reflux, surgery may be needed.

Moderate to severe vesicoureteral reflux, if not treated, can lead to serious health problems down the road. But with prompt treatment, long-term complications and damage to the kidneys can be prevented.

Figure 1. Vesicoureteral reflux

Vesicoureteral reflux grading

Doctors grade vesicoureteral reflux according to the degree of reflux. A grading system for reflux was established by the International Reflux Study Committee established in 1981 to aid with clinical management and prognostication ¹⁾. Five grades are defined based on the extent of reflux and degree of dilation of the upper tract on imaging (Figure 2). In the mildest cases, urine backs up only to the ureter (grade I). The most severe cases involve severe kidney swelling (hydronephrosis) and twisting of the ureter (grade V).

Spontaneous resolution of primary reflux is common. This is thought to be multi-factorial, due in part to remodeling of the ureterovesical junction, elongation of the intravesical ureter, and stabilization of bladder voiding dynamics over time. At birth, the likelihood of spontaneous resolution is inversely proportional to the initial grade of reflux; approximately 80% of low-grade (I and II) reflux will resolve spontaneously vs. about 50% of grade III reflux. Few or approximately 20% of high-grade (IV and V) reflux will resolve ²⁾. For older children, resolution depends on both initial grade of reflux, gender, and age at initial diagnosis ³⁾. Other factors that affect reflux resolution include voiding dysfunction, presence of renal scarring, and occurrence of reflux during bladder filling or emptying. In general, reflux is more likely to resolve in younger patients with low-grade reflux and normal renal ultrasound.

Figure 2. Vesicoureteral reflux grading

What causes vesicoureteral reflux?

In many children, vesicoureteral reflux seems to be inherited (primary vesicoureteral reflux). If a mother has been treated for vesicoureteral reflux, as many as 50% of her children may also have it. Vesicoureteral reflux cannot be prevented, but most infections that result from vesicoureteral reflux can be.

Normally, urine flows from the kidneys to the bladder, but defects in one or both ureters can allow it to flow the other way. So can a blockage in the ureters or in the bladder.

Children with primary vesicoureteral reflux are born with a defect in the valve that normally prevents urine from flowing backward from the bladder into the ureters. Primary vesicoureteral reflux is the most common type of vesicoureteral reflux affecting kids. If a child is born with primary vesicoureteral reflux, it means that a ureter didn’t grow long enough while the baby was in the womb. This can affect the valve where the ureter enters the bladder. If the valve doesn’t shut properly, urine can flow back up the ureters to reach the kidney. As your child grows, the ureters lengthen and straighten, which may improve the valve function and eventually resolve the vesicoureteral reflux. Primary vesicoureteral reflux tends to run in families, which indicates that it may be genetic, but the exact cause of the defect is unknown.

If a blockage in the urinary tract obstructs the flow of urine and causes it to go back into the kidneys, it’s called secondary vesicoureteral reflux. Kids with this type of vesicoureteral reflux often have reflux in both ureters. Secondary vesicoureteral reflux can be caused by nerve damage, infection, or pressure on the ureter from another organ, such as an enlarged prostate often causing abnormally high pressure inside the bladder.

Primary vesicoureteral reflux

Primary vesicoureteral reflux will often get better and will go away as a child gets older.

Until vesicoureteral reflux goes away on its own, doctors treat any UTIs that develop with antibiotics, a type of medicine that fights bacteria. Treating UTIs quickly and preventing UTIs from developing will make it less likely your child will have a kidney infection.

Your child’s doctor also may consider the use of a long-term, low-dose antibiotic to prevent UTIs. Researchers have found that daily use of a low-dose antibiotic may help many children with vesicoureteral reflux. Talk with your child’s doctor about using antibiotics. The bacteria that cause these infections can become harder to fight when antibiotics are used long term.

Sometimes doctors will consider surgery for a child who has vesicoureteral reflux with repeat UTIs, particularly if the child has renal scarring or severe reflux that is not improving. Doctors can use surgery to correct your child’s reflux and prevent urine from flowing back to the kidney.

In certain cases, treatment may include the use of bulking injections. Doctors inject a small amount of gel-like liquid into the bladder wall near the opening of the ureter. The gel makes a bulge in the bladder wall, which acts like a valve to the ureter if a child’s valve doesn’t work properly. The doctor provides the treatment using general anesthesia and a child can usually go home the same day.

Secondary vesicoureteral reflux

Doctors treat secondary vesicoureteral reflux after finding the exact cause of the condition. Treatment may include

• surgery to remove a blockage
• antibiotics to prevent or treat UTIs
• surgery to correct an abnormal bladder or ureter
• intermittent urinary catheterization—draining the bladder by inserting a catheter through the urethra to the bladder. You can do this at home if your child’s bladder does not empty properly.

Risk factors for vesicoureteral reflux

Risk factors for vesicoureteral reflux include:

• Bladder and bowel dysfunction. Children with bladder and bowel dysfunction hold their urine and stool and experience recurrent urinary tract infections, which can contribute to vesicoureteral reflux.
• Race. White children appear to have a higher risk of vesicoureteral reflux.
• Sex. Generally, girls have about double the risk of having this condition as boys do. The exception is for vesicoureteral reflux that’s present at birth, which is more common in boys.
• Age. Infants and children up to age 2 are more likely to have vesicoureteral reflux than older children are.
• Family history. Primary vesicoureteral reflux tends to run in families. Children whose parents had the condition are at higher risk of developing it. Siblings of children who have the condition also are at higher risk, so your doctor may recommend screening for siblings of a child with primary vesicoureteral reflux.

Can I prevent vesicoureteral reflux?

You can’t prevent vesicoureteral reflux, but good habits may help keep your child’s urinary tract as healthy as possible. To prevent some bladder infections and bladder control problems, have your child:

• drink enough liquids based on the doctor’s advice.
• follow good bathroom habits, such as urinating regularly and wiping front to back.
• changed as soon as possible after his or her diaper becomes dirty, if he or she is not potty trained.
• get treated for constipation if necessary. Try to prevent your child’s constipation if possible.
• treated for related health problems such as urinary incontinence or fecal incontinence.

Vesicoureteral reflux symptoms

There are no specific signs and symptoms of vesicoureteral reflux, however children who have had a urinary tract infection (UTI) with a fever are more likely to have vesicoureteral reflux. Urinary tract infections (UTIs) and vesicoureteral reflux can lead to kidney damage, so occasionally patients have symptoms of renal problems.

Urinary tract infections commonly occur in people with vesicoureteral reflux. A urinary tract infection (UTI) doesn’t always cause noticeable signs and symptoms, though most people have some.

Urinary tract infection (UTI) signs and symptoms can include:

• A strong, persistent urge to urinate
• A burning sensation when urinating
• Passing frequent, small amounts of urine
• Blood in the urine (hematuria) or cloudy, strong-smelling urine
• Fever
• Pain in your side (flank) or abdomen
• Hesitancy to urinate or holding urine to avoid the burning sensation

Lower tract infection (in the bladder):

• frequent or urgent need to urinate
• a burning sensation while urinating
• blood in the urine, or urine that is cloudy or foul smelling

Upper tract infection (in the ureters or kidneys):

• pain in the side or abdomen
• fever and chills

A UTI may be difficult to diagnose in children, who may have only nonspecific signs and symptoms. Signs and symptoms in infants with a UTI may also include:

• An unexplained fever
• Diarrhea
• Lack of appetite
• Irritability

As your child gets older, untreated vesicoureteral reflux can lead to:

• Bed-wetting
• Constipation or loss of control over bowel movements
• High blood pressure
• Protein in urine
• Kidney failure

Another indication of vesicoureteral reflux, which may be detected before birth by sonogram, is swelling of the kidneys or the urine-collecting structures of one or both kidneys (hydronephrosis) in the fetus, caused by the backup of urine into the kidneys.

Vesicoureteral reflux complications

Kidney damage is the primary concern with vesicoureteral reflux. The more severe the reflux, the more serious the complications are likely to be.

Complications may include:

• Kidney (renal) scarring. Untreated UTIs can lead to scarring, also known as reflux nephropathy, which is permanent damage to kidney tissue. Extensive scarring may lead to high blood pressure and kidney failure.
• High blood pressure (hypertension). Because the kidneys remove waste from the bloodstream, damage to your kidneys and the resultant buildup of wastes can raise your blood pressure.
• Kidney failure. Scarring can cause a loss of function in the filtering part of the kidney. This may lead to kidney failure, which can occur quickly (acute kidney failure) or may develop over time (chronic kidney disease).

Vesicoureteral reflux diagnosis

Vesicoureteral reflux is usually diagnosed after a child has a urinary tract infection (UTI) with a fever, but can be identified before the child is born on prenatal ultrasound. Urinalysis — lab analysis of a urine sample — can reveal whether your child has a urinary tract infection (UTI).

Kidney and bladder ultrasound. Also called sonography, this imaging method uses high-frequency sound waves to produce images of the kidney and bladder. Ultrasound can detect structural abnormalities. This same technology, often used during pregnancy to monitor fetal development, may also reveal swollen kidneys in the baby, an indication of primary vesicoureteral reflux.

Nuclear scan. This test, known as radionuclide cystogram, uses a procedure similar to that used for voiding cystourethrogram (VCUG), except that instead of dye being injected into your child’s bladder through the catheter, this test uses a radioactive tracer (radioisotope). The scanner detects the tracer and shows whether the urinary tract is functioning correctly.

Voiding cystourethrography (VCUG) is the gold standard for diagnosis of vesicoureteral reflux. A voiding cystourethrogram (VCUG) x-ray of the kidneys, ureters, bladder and urethra is necessary to evaluate and diagnose vesicoureteral reflux. A voiding cystourethrogram (VCUG), is a minimally invasive test that uses a special x-ray technology called fluoroscopy to visualize your child’s urinary tract and bladder.

Voiding cystourethrogram (VCUG) test uses X-rays of the bladder when it’s full and when it’s emptying to detect abnormalities. A thin, flexible tube (catheter) is inserted through the urethra and into the bladder while your child lies on his or her back on an X-ray table.

After contrast dye is injected into the bladder through the catheter, your child’s bladder is X-rayed in various positions. Then the catheter is removed so that your child can urinate, and more X-rays are taken of the bladder and urethra during urination to see whether the urinary tract is functioning correctly.

Risks associated with this test include discomfort from the catheter or from having a full bladder and the possibility of a new urinary tract infection.

A voiding cystourethrogram (VCUG) can help:

• diagnose vesicoureteral reflux.
• determine why your child has recurring urinary tract infections
• discover if antibiotic treatment or anti-reflux surgery was effective
• check if there are any abnormalities or blockages of the urethra

Once diagnosed, the vesicoureteral reflux is graded. The grade of vesicoureteral reflux indicates how much urine is flowing backward into the ureters and kidneys and helps the provider to determine which type of care is most appropriate. Vesicoureteral reflux is graded on a scale of 1 to 5, with 5 being the most serious.

Vesicoureteral reflux treatment

Treatment options for vesicoureteral reflux depend on the severity of the condition. Children with mild cases of primary vesicoureteral reflux may eventually outgrow the disorder. In this case, your doctor may recommend a wait-and-see approach. As a child gets older, the ureter gets longer and straighter, and the valve where the ureter enters the bladder is able to shut correctly.

Vesicoureteral reflux that occurs with a UTI needs prompt antibiotic treatment to keep the infection from spreading to the kidneys. Kids who are put on antibiotics should take them for as long as prescribed, even if they start to feel better early on.

Urinary tract infections, which are so common to vesicoureteral reflux, can be painful. But you can take steps to ease your child’s discomfort until antibiotics clear the infection. They include:

• Encourage your child to drink fluids, particularly water. Drinking water dilutes urine and may help flush out bacteria. Avoid juices and soft drinks containing citrus and caffeine until your child’s infection has cleared. They can irritate the bladder and tend to aggravate the frequent or urgent need to urinate.
• Provide a warm blanket or towel. Place a towel or blanket in the dryer for a few minutes to warm it up. Be sure the towel or blanket is just warm, not hot, and then place it over your child’s abdomen. The warmth can help minimize feelings of bladder pressure or pain.

If bladder and bowel dysfunction contributes to your child’s vesicoureteral reflux, encourage healthy toileting habits. Avoiding constipation and emptying the bladder every two hours while awake may help.

For more severe vesicoureteral reflux, treatment options include:

Medications

Urinary tract infections (UTIs) require prompt treatment with antibiotics to keep the infection from moving to the kidneys. To prevent urinary tract infections (UTIs), doctors may also prescribe antibiotics at a lower dose than for treating an infection.

A child being treated with medication needs to be monitored for as long as he or she is taking antibiotics. This includes periodic physical exams and urine tests to detect breakthrough infections — urinary tract infections (UTIs) that occur despite the antibiotic treatment — and occasional radiographic scans of the bladder and kidneys to determine if your child has outgrown vesicoureteral reflux.

Vesicoureteral reflux surgery

Surgery for vesicoureteral reflux repairs the defect in the valve between the bladder and each affected ureter. A defect in the valve keeps it from closing and preventing urine from flowing backward. The most common type of surgery is ureteral reimplantation, in which one or both ureters are extended further into the bladder to correct the backflow of urine from the bladder to the ureters and kidneys. This type of surgery usually requires kids to spend a few days in the hospital while they recover.

Methods of surgical repair include:

• Open surgery. Performed using general anesthesia, this surgery requires an incision in the lower abdomen through which the surgeon repairs the malformation that’s causing the problem. This type of surgery usually requires a few days’ stay in the hospital, during which a catheter is kept in place to drain your child’s bladder. Vesicoureteral reflux may persist in a small number of children, but it generally resolves on its own without need for further intervention.
• Robotic-assisted laparoscopic surgery. Similar to open surgery, this procedure involves repairing the valve between the ureter and the bladder, but it’s performed using small incisions. Advantages include smaller incisions and possibly less bladder spasms than open surgery. But, preliminary findings suggest that robotic-assisted laparoscopic surgery may not have as high of a success rate as open surgery. The procedure was also associated with a longer operating time, but a shorter hospital stay.
• Endoscopic surgery also known as the deflux procedure. In this procedure, the doctor inserts a lighted tube (cystoscope) through the urethra to see inside your child’s bladder, then injects a bulking agent around the opening of the affected ureter to try to strengthen the valve’s ability to close properly. This method is minimally invasive compared with open surgery and presents fewer risks, though it may not be as effective as open reimplantation. Endoscopic surgery also requires general anesthesia, but generally can be performed as outpatient surgery. Most kids can leave the hospital on the same day that they have this procedure.

Estimated success rates of open and endoscopic reflux correction are 98.1% and 83.0%, respectively ⁴⁾. Factors that affect the success of endoscopic injection include pre-operative reflux grade and presence of functional or anatomic bladder abnormalities including voiding dysfunction and duplicated collecting systems. Few studies have evaluated the long-term outcomes of endoscopic injection, and with variable results. In patients treated endoscopically, recurrent febrile UTI occurred in 0–21%, new renal damage in 9–12%, and recurrent reflux in 17–47.6% of treated ureters with at least 1 year follow-up ⁵⁾.

What is precocious puberty

Precocious puberty is when a person’s sexual and physical traits develop and mature earlier than normal. Precocious (meaning prematurely developed) puberty (secondary sex characteristics) begins in girls younger than 7½ or 8 and in boys younger than age 9. In the United States, precocious puberty affects about 1 – 2% of children (roughly 3 million children) ¹⁾. Yet, in most of these children, early puberty is a variation of normal and no medical problem is present. The actual age that defines sexual precocity is therefore dependent on the epidemiological data that one uses to define the average age of pubertal onset. Different populations and different time periods will therefore have differing definitions of precocious puberty. Recently, most likely because of increasing weight in the population, puberty appears to be having an earlier age of onset ²⁾.

Many more girls are affected than boys. One study suggests that African American girls have some early breast development or some early pubic hair more often than white girls or Hispanic girls.

The cause of precocious puberty is not always known. Some cases of precocious puberty are due to conditions that cause changes in the body’s release of hormones. Treatment involves medications that can stop the release of sexual hormones ³⁾.

Signs of Precocious Puberty

In girls, the telltale signs of precocious puberty include any of the following before 7 or 8 years of age:

• breast development
• pubic or underarm hair development
• rapid height growth — a growth “spurt”
• start of menstruation (her period)
• acne
• “mature” body odor

In boys, the signs of precocious puberty before 9 years of age include:

• enlargement of the testicles or penis
• pubic, underarm, or facial hair development
• rapid height growth — a growth “spurt”
• voice deepening
• acne
• “mature” body odor.

How Precocious Puberty Affects Kids

When puberty ends, growth in height stops. Because their skeletons mature and bone growth stops at an earlier age than normal, kids with precocious puberty usually don’t reach their full adult height potential. Their early growth spurt may make them initially tall when compared with their peers, but they may stop growing too soon and end up at a shorter height than they would have otherwise.

Going through puberty early also can be difficult for kids emotionally and socially. For example, girls with precocious puberty may be confused or embarrassed about physical changes such as getting their periods or having enlarged breasts well before any of their peers. But the hardest part may be the teasing that kids with the condition — especially girls — may experience.

Even emotions and behavior may change in kids with precocious puberty. Girls can become moody and irritable. Boys can become more aggressive and also develop a sex drive inappropriate for their age.

Children affected by precocious puberty may experience problems such as:

• Failure to reach their full height because their growth halts too soon
• Psychological and social problems, such as anxiety over being “different” from their peers. However, many children do not experience major psychological or social problems, particularly when the onset of puberty is only slightly early.

Who is at risk of early puberty?

There is a greater chance of being affected by precocious puberty if a child is ⁴⁾:

• Female
• African American
• Obese

Normal puberty

Normal puberty typically begins between ages 8 and 13 for girls (the average age is about 10) and ages 9 and 14 for boys (the average age is about 11) ⁵⁾. The start of puberty depends on various factors such as family history, nutrition and gender. Normally, the hypothalamus initiates puberty by releasing a hormone called gonadotropin-releasing hormone (GnRH) that stimulates the anterior pituitary gland to release gonadotropins – follicle-stimulating hormone (FSH) and luteinizing hormone (LH), the hormones which control growth and function of the sex organs (see Figure 1). When gonadotropins follicle-stimulating hormone (FSH) and luteinizing hormone (LH) are released, synthesis and secretion of sex steroids (such as estrogen, progesterone or testosterone) occur in the testicles (testosterone) or ovaries (estrogen & progesterone), leading to development of secondary sexual characteristics. If this occurs prematurely, a child starts to develop secondary sexual characteristics and proceeds to sexual maturity at an unexpectedly early age. Since the maturing of one’s bones is usually accelerated by this condition, early fusion of the growth plates occurs, resulting in shortening of adult stature. However, during childhood, children with precocious puberty are often taller than their peers.

Many kids who show some of the early signs of puberty have what’s known as “partial” precocious puberty. Some girls, usually beginning between the ages of 6 months and 3 years, may show breast development that later disappears or may last without other physical changes of puberty.

Similarly, some girls and boys may have early growth of pubic and/or underarm hair that isn’t related to other changes in sexual development.

Kids with “partial” precocious puberty may need to see their doctor to rule out “true” precocious puberty or other health problems. But most need no treatment and usually will show the other expected signs of puberty at the usual age.

Many children who go through puberty early or late have other family members who went through puberty early or late. Just as there are differences in the age at which puberty starts, there are also differences in the rate at which a child goes through puberty.

Near the end of puberty, growth in height stops. Because the bones of children with precocious puberty mature and stop growing earlier than normal, these children can be shorter than expected as adults. Precocious puberty can also cause emotional and social problems for children who are ahead of their peers in terms of sexual maturity.

Figure 1. Normal puberty

Precocious puberty complications

Possible complications of precocious puberty include:

• Short height. Children with precocious puberty may grow quickly at first and be tall, compared with their peers. But, because their bones mature more quickly than normal, they often stop growing earlier than usual. This can cause them to be shorter than average as adults. Early treatment of precocious puberty, especially when it occurs in very young children, can help them grow taller than they would without treatment.
• Social and emotional problems. Girls and boys who begin puberty long before their peers may be extremely self-conscious about the changes occurring in their bodies. This may affect self-esteem and increase the risk of depression or substance abuse.

Early onset puberty causes

In approximately 90% of girls who experience precocious puberty, no underlying cause can be identified—although heredity and being overweight may contribute in some cases. When a cause cannot be identified, the condition is called idiopathic precocious puberty. In boys with precocious puberty, approximately 50% of cases are idiopathic. In the remaining 10% of girls and 50% of boys with precocious puberty, an underlying cause can be identified.

Sometimes the cause is an abnormality involving the brain. In other children, the signs of puberty occur because of a problem such as a tumor or genetic abnormality in the ovaries, testes, or adrenal glands, causing overproduction of sex hormones.

Precocious puberty can be divided into two categories, depending on where in the body the abnormality occurs—central precocious puberty and peripheral precocious puberty.

The onset of puberty is normally triggered by the hypothalamus (the area of the brain that helps control pituitary gland function). It signals the pituitary gland (a pea-sized gland near the base of the brain) to release hormones that stimulate the ovaries (in girls) or testicles (in boys) to make sex hormones.

Sometimes, precocious puberty stems from a structural problem in the brain (such as a tumor), brain injury due to head trauma, an infection (such as meningitis), or a problem in the ovaries or thyroid gland that triggers the onset of puberty ahead of schedule — but this usually isn’t the case.

For the majority of girls, there’s no medical problem at fault — they simply start puberty too early for no known reason.

In boys, the condition is less common and more likely to be related to another medical problem. And for about 5% of boys, precocious puberty is inherited. (Less than 1% of girls with precocious puberty have inherited the condition.) Early puberty can be passed to a son by his father or to the son from his maternal grandfather through his mother (who will not be affected by the disorder).

Central Precocious Puberty

Central precocious puberty occurs when the hypothalamus releases GnRH (gonadotropin-releasing hormone) which stimulates the pituitary gland to begin puberty at an early age. In most girls with central precocious puberty, there is no underlying medical problem. In boys, the condition is less common and is more likely to have a link to a medical problem. Such problems include a tumor, brain trauma (such as a blow to the head, brain surgery, or radiation treatment to the head), or inflammation (such as meningitis).

Central precocious puberty is the most common form of precocious puberty and affects many more girls than boys.

There’s usually no identifiable cause for this type of precocious puberty.

In central precocious puberty, the puberty process starts too soon. The pattern and timing of the steps in the process are otherwise normal. For the majority of children with this condition, there’s no underlying medical problem and no identifiable reason for the early puberty.

In rare cases, the following may cause central precocious puberty:

• A tumor in the brain or spinal cord (central nervous system)
• Prior radiation to the brain or spinal cord (central nervous system)
• A defect in the brain present at birth, such as excess fluid buildup (hydrocephalus) or a noncancerous tumor (hamartoma)
• Prior infection of the brain
• Other brain abnormalities
• Injury to the brain or spinal cord

Often, however, there is no identifiable abnormality in the brain; this is called idiopathic central precocious puberty.

Peripheral Precocious Puberty

The less common peripheral precocious puberty occurs without the involvement of the gonadotropin hormone in your brain (GnRH) that normally triggers the start of puberty. In peripheral precocious puberty, the abnormality is not in the brain but in the testicles, ovaries, or adrenal glands, causing overproduction of sex hormones, like testosterone and estrogens.

Peripheral precocious puberty may be caused by ⁶⁾:

• Tumors of the ovary, testis, or adrenal gland
• In boys, tumors that secrete a hormone called hCG, or human chorionic gonadotropin
• Certain rare genetic syndromes, such as McCune-Albright syndrome (a rare genetic disease that affects bones and skin color and causes hormonal problems) or familial male precocious puberty
• Severe hypothyroidism, in which the thyroid gland secretes abnormally low levels of hormones
• Disorders of the adrenal gland, such as congenital adrenal hyperplasia — a group of genetic disorders involving abnormal hormone production by the adrenal glands
• Exposure of the child to medicines or creams that contain estrogens or androgens

In girls, peripheral precocious puberty may also be associated with:

• Ovarian cysts
• Ovarian tumors

In boys, peripheral precocious puberty may also be caused by:

• A tumor in the cells that make sperm (germ cells) or in the cells that make testosterone (Leydig cells)
• Gene mutation — a rare disorder called gonadotropin-independent familial sexual precocity, which is caused by a defect in a gene, can result in the early production of testosterone in boys, usually between ages 1 and 4.

Risk factors for precocious puberty

Factors that increase a child’s risk of precocious puberty include:

• Being a girl. Girls are much more likely to develop precocious puberty.
• Being African-American. Precocious puberty appears to affect African-Americans more often than children of other races.
• Being obese. Children who are significantly overweight have a higher risk of developing precocious puberty.
• Being exposed to sex hormones. Coming in contact with an estrogen or testosterone cream or ointment, or other substances that contain these hormones (such as an adult’s medication or dietary supplements), can increase your child’s risk of developing precocious puberty.
• Having other medical conditions. Precocious puberty may be a complication of McCune-Albright syndrome or congenital adrenal hyperplasia — conditions that involve abnormal production of the male hormones (androgens). In rare cases, precocious puberty may also be associated with hypothyroidism.
• Having received radiation therapy of the central nervous system. Radiation treatment for tumors, leukemia or other conditions can increase the risk of precocious puberty.

Precocious puberty signs and symptoms

The symptoms of precocious puberty are similar to the signs of normal puberty but they manifest earlier—before the age of 8 in girls and before age 9 in boys.

Normal Puberty

In Girls

The signs of puberty include:

• Growth of pubic and other body hair
• Growth spurt
• Breast development
• Onset of menstruation (after puberty is well advanced)
• Acne

In Boys

The signs of puberty include ⁷⁾:

• Growth of pubic hair, other body hair, and facial hair
• Enlargement of penis and testicular enlargement (>3 ml)
• Muscle growth
• Growth spurt
• Acne
• Deepening of the voice

Precocious puberty diagnosis

To identify whether a child is entering puberty, a pediatrician (a physician specializing in the treatment of children) will carefully examine the following:

• In girls, the growth of pubic hair and breasts
• In boys, the increase in size of the testicles and penis and the growth of pubic hair

The pediatrician will compare what he or she finds against the Tanner scale, a 5-point scale that gauges the extent of puberty development in children ⁸⁾.

After giving a child a complete physical examination and analyzing his or her medical history, a health care provider may perform tests to diagnose precocious puberty, including ⁹⁾:

• A blood test to check the level of hormones, such as the gonadotropins (luteinizing hormone [LH] and follicle-stimulating hormone [FSH]), estradiol, testosterone, dehydroepiandrosterone sulfate (DHEAS), and thyroid hormones
• A gonadotropin-releasing hormone agonist (GnRHa) stimulation test, which can tell whether a child’s precocious puberty is gonadotropin-dependent or gonadotropin-independent
• Measuring blood 17-hydroxyprogesterone to test for congenital adrenal hyperplasia
• Thyroid testing. The doctor may also test your child’s thyroid if he or she shows any signs of slow thyroid function (hypothyroidism), such as fatigue, sluggishness, increased sensitivity to cold, constipation, a drop in school performance or pale, dry skin.
• A “bone age” X­-ray to determine if bones are growing at a normal rate

The health care provider may also use imaging techniques to rule out a tumor or other organ abnormality as a cause.

These imaging methods may include ¹⁰⁾:

• Ultrasound (sonography) to examine the gonads. An ultrasound painlessly creates an image on a computer screen of blood vessels and tissues, allowing a health care provider to monitor organs and blood flow in real time
• An MRI (magnetic resonance imaging) scan of the brain and pituitary gland using an instrument that produces detailed images of organs and bodily structures.

Precocious puberty treatment

If your doctor thinks that your child has precocious puberty, he or she may refer you to a pediatric endocrinologist (a doctor who specializes in growth and hormonal disorders in children) for further evaluation and treatment.

Once precocious puberty is diagnosed, the goal of treating it is to stop or even reverse sexual development and stop the rapid growth and bone maturation that can eventually result in adult short stature.

There are a number of reasons to treat precocious puberty ¹¹⁾.

Treatment for precocious puberty can help stop puberty until the child is closer to the normal time for sexual development. One reason to consider treating precocious puberty is that rapid growth and bone maturation, caused by precocious puberty, can prevent a child from reaching his or her full height potential. Children grow rapidly in height during puberty and reach their final adult height after puberty. Children who go through puberty too early may not reach their full adult height potential because their growth stops too soon.

Another reason to consider treating precocious puberty is that a young child may not be psychologically ready for the physical and hormonal changes that occur in puberty.

However, not all children with precocious puberty require treatment, particularly if the onset of puberty is only slightly early. The goal of treatment is to prevent the production of sex hormones to prevent the early halt of growth, short stature in adulthood, emotional effects, social problems, and problems with libido (especially in boys).

If precocious puberty is caused by a specific medical problem, treating the underlying problem can often stop the progression of precocious puberty. In addition, precocious puberty can often be stopped by medical treatment to block the hormones that cause puberty.

Depending upon the cause, there are two possible approaches to treatment:

• treating the underlying cause or disease, such as a tumor
• lowering the high levels of sex hormones with medicine to stop sexual development from progressing

Sometimes, treatment of a related health problem can stop the precocious puberty. But in most cases, no other disease is triggering the condition, so treatment usually involves hormone therapy to stop sexual development.

The currently approved hormone treatment is with drugs called gonadotropin releasing hormone (GnRH) analogues — synthetic hormones that block the body’s production of the sex hormones that cause the early puberty — usually includes a monthly injection of a medication, such as leuprolide (Lupron Depot), which delays further development. Some newer formulations can be given at longer intervals. In 2017, Triptodur was approved by the US Food and Drug Administration (FDA) for pediatric patients ages 2 years and older with central precocious puberty. Triptodur is a gonadotropin releasing hormone (GnRH) analog that is given via intramuscular injection of 22.5 mg once every 24 weeks or twice yearly ¹²⁾.

Dramatic results are usually seen within a year of starting treatment with an Gn-RH analogue, which is generally safe and usually causes no side effects in kids.

In girls, breast size may decrease — or at least there will be no further development. In boys, the penis and testicles may shrink back to the size expected for their age. Growth in height will also slow down to a rate expected for kids before puberty. A child’s behavior usually becomes more age-appropriate, too.

The child continues to receive this medication until he or she reaches the normal age of puberty. On average, 16 months after he or she stops receiving the medication, the process of puberty begins again.

Treating an underlying medical condition

If another medical condition is causing your child’s precocious puberty, treatment of that condition is necessary to stop the progress of puberty. For example, if a child has a tumor that’s producing hormones and causing precocious puberty, puberty usually will stop when the tumor is surgically removed.

Caring for Your Child

Give your child a simple, truthful explanation about what’s happening. Explain that these changes are normal for older kids and teens, but that his or her body has started developing a little too early. Keep your child informed about treatment and what can be expected along the way.

Also be sure to watch for signs that teasing or other difficulties associated with precocious puberty may be affecting your child’s emotional development.

Common warning signs to discuss with your doctor include:

• poor grades
• problems at school
• loss of interest in daily activities
• depression

How you cope with the issue can also determine how well your child will cope. The goal is to prevent kids from dwelling on sexual development or developing a poor self-image or low self-esteem. To create a supportive environment, try not to focus your comments on appearance. Instead, offer praise for achievements in school or sports and support your child’s participation in other activities.

The important thing to remember is that kids with precocious puberty can be treated. Doctors can help them preserve their adult height potential as well as limit the emotional and social difficulties they may face from maturing early.

What is nephrotic syndrome

Nephrotic syndrome is not a specific disease but the name given to the set of problems that causes the kidneys to leak large amounts of protein into the urine. Nephrotic syndrome can lead to a range of problems, including swelling of body tissues and a greater chance of catching infections.

Nephrotic syndrome includes the following:

• Albuminuria—large amounts of protein in the urine. In nephrotic syndrome, damaged glomeruli allow 3 grams or more of protein to leak into the urine when measured over a 24-hour period, which is more than 20 times the amount that healthy glomeruli allow.
• Hyperlipidemia—higher than normal fat and cholesterol levels in the blood
• Edema, or swelling, usually in the legs, feet, or ankles and less often in the hands or face
• Hypoalbuminia—low levels of albumin in the blood

Albumin is a protein that acts like a sponge, drawing extra fluid from the body into the bloodstream where it remains until removed by the kidneys. When albumin leaks into the urine, the blood loses its capacity to absorb extra fluid from the body, causing edema.

Nephrotic syndrome results from a problem with the kidneys’ filters, called glomeruli (see Figures 3 and 4). Glomeruli are tiny blood vessels in the kidneys that remove wastes and excess fluids from the blood and send them to the bladder as urine.

One of the main jobs of your kidneys is to filter your blood. This allows you to pass your body’s waste products, extra fluid and salts through your urine. The glomeruli are the parts of the kidney where this happens

With healthy kidneys, proteins in your blood don’t usually leak into the urine during the filtering process. But if the glomeruli become damaged, proteins such as albumin can leak into your urine along with the waste products. If too many proteins leak out, that is known as nephrotic syndrome.

Although nephrotic syndrome can affect people of any age, it’s usually first diagnosed in children aged between 2 and 5 years old. It affects more boys than girls.

Kidneys and kidney function

The kidneys are two bean-shaped organs, each about the size of a fist. They are located just below the rib cage, one on each side of the spine (Figures 1 to 4). Every day, your kidneys filter about 120 to 150 quarts of blood to produce about 1 to 2 quarts of urine, composed of wastes and extra fluid. Children produce less urine than adults and the amount produced depends on their age. The urine flows from the kidneys to the bladder through tubes called ureters. The bladder stores urine. When the bladder empties, urine flows out of the body through a tube called the urethra, located at the bottom of the bladder.

Kidneys work at the microscopic level. The kidney is not one large filter. Each kidney is made up of about a million filtering units called nephrons. Each nephron filters a small amount of blood. The nephron includes a filter, called the glomerulus, and a tubule. The nephrons work through a two-step process. The glomerulus lets fluid and waste products pass through it; however, it prevents blood cells and large molecules, mostly proteins, from passing. The filtered fluid then passes through the tubule, which sends needed minerals back to the bloodstream and removes wastes.

Figure 1. Kidney location

Figure 2. Kidney anatomy

Figure 3. Kidney structure

Figure 4. Microcirculation of the kidney

Nephrotic syndrome in children

Childhood nephrotic syndrome is not a disease in itself; rather, it is a group of symptoms that:

• indicate kidney damage—particularly damage to the glomeruli, the tiny units within the kidney where blood is filtered
• result in the release of too much protein from the body into the urine

When the kidneys are damaged, the protein albumin, normally found in the blood, will leak into the urine. Proteins are large, complex molecules that perform a number of important functions in the body.

The two types of childhood nephrotic syndrome are:

1. Primary—the most common type of childhood nephrotic syndrome, which begins in the kidneys and affects only the kidneys
2. Secondary—the syndrome is caused by other diseases

Around 1 in every 50,000 children are diagnosed with nephrotic syndrome each year. Childhood nephrotic syndrome tends to be more common in families with a history of allergies or those of an Asian background, although it’s unclear why.

A health care provider may refer a child with nephrotic syndrome to a nephrologist—a doctor who specializes in treating kidney disease. A child should see a pediatric nephrologist, who has special training to take care of kidney problems in children, if possible. However, in many parts of the country, pediatric nephrologists are in short supply, so the child may need to travel. If traveling is not possible, some nephrologists who treat adults can also treat children.

The symptoms of childhood nephrotic syndrome can usually be controlled with steroid medication. Most children with nephrotic syndrome respond well to steroids and aren’t at risk of kidney failure.

However, a small number of children have congenital (inherited) nephrotic syndrome and usually do less well. They may eventually have kidney failure and need a kidney transplant.

Most children with nephrotic syndrome have times when their symptoms are under control (remission), followed by times when symptoms return (relapses).

In most cases, relapses become less frequent as they get older and often stop by their late teens.

Some of the main symptoms associated with nephrotic syndrome in children include:

• swelling – the low level of protein in the blood reduces the flow of water from body tissues back into the blood vessels, leading to swelling (oedema). Swelling is usually first noticed around the eyes, then around the lower legs and rest of the body.
• infections – antibodies are a specialised group of proteins in the blood that help to fight infection. When these are lost, children are much more likely to get infections.
• urine changes – occasionally, the high levels of protein being passed into the urine can cause it to become frothy. Some children with nephrotic syndrome may also pass less urine than usual during relapses.
• blood clots – important proteins that help to prevent the blood clotting can be passed out in the urine of children with nephrotic syndrome. This can increase their risk of potentially serious blood clots. During a relapse, the blood also becomes more concentrated, which can lead to clotting.

Nephrotic syndrome in children causes

While idiopathic, or unknown, diseases are the most common cause of primary childhood nephrotic syndrome, researchers have linked certain diseases and some specific genetic changes that damage the kidneys with primary childhood nephrotic syndrome.

The cause of secondary childhood nephrotic syndrome is an underlying disease or infection. Called a primary illness, it’s this underlying disease or infection that causes changes in the kidney function that can result in secondary childhood nephrotic syndrome.

Congenital diseases—diseases that are present at birth—can also cause childhood nephrotic syndrome.

Primary Childhood Nephrotic Syndrome

The following diseases are different types of idiopathic childhood nephrotic syndrome:

• Minimal change disease means that their kidneys appear normal or nearly normal if a tissue sample is studied under a standard microscope. Most children with nephrotic syndrome have “minimal change disease”. Minimal change disease is the most common cause of idiopathic childhood nephrotic syndrome ¹⁾. Minimal change disease involves damage to the glomeruli that can be seen only with an electron microscope. This type of microscope shows tiny details better than any other microscope. The cause of minimal change disease is unknown.
• Focal segmental glomerulosclerosis is scarring in scattered regions of the kidney:
  • “Focal” means that only some of the glomeruli become scarred.
  • “Segmental” means damage affects only part of an individual glomerulus.
• Membranoproliferative glomerulonephritis is a group of disorders involving deposits of antibodies that build up in the glomeruli, causing thickening and damage. Antibodies are proteins made by the immune system to protect the body from foreign substances such as bacteria or viruses.

Secondary Childhood Nephrotic Syndrome

Some common diseases that can cause secondary childhood nephrotic syndrome include

• Diabetes, a condition that occurs when the body cannot use glucose—a type of sugar—normally
• Henoch-Schönlein purpura, a disease that causes small blood vessels in the body to become inflamed and leak
• Hepatitis, inflammation of the liver caused by a virus
• Human immunodeficiency virus (HIV), a virus that alters the immune system
• Lupus, an autoimmune disease that occurs when the body attacks its own immune system
• Malaria, a disease of the blood that is spread by mosquitos
• Streptococcal infection, an infection that results when the bacteria that causes strep throat or a skin infection is left untreated
• Sickle cell anaemia
• In very rare cases, certain types of cancer – such as leukemia, multiple myeloma or lymphoma.

Other causes of secondary childhood nephrotic syndrome can include certain medications, such as aspirin, ibuprofen, or other nonsteroidal anti-inflammatory drugs (NSAIDs), and exposure to chemicals, such as mercury and lithium.

Congenital nephrotic syndrome

Congenital nephrotic syndrome is a kidney condition that begins in infancy and typically leads to irreversible kidney failure (end-stage renal disease) by early childhood. Children with congenital nephrotic syndrome begin to have symptoms of the condition between birth and 3 months of life ²⁾. Children with congenital nephrotic syndrome typically develop end-stage renal disease between ages 2 and 8, although with treatment, some may not have kidney failure until adolescence or early adulthood.

Congenital nephrotic syndrome affects 1 to 3 per 100,000 children worldwide. In Finland, where this condition is particularly common, congenital nephrotic syndrome is estimated to affect 1 in 10,000 children.

The features of congenital nephrotic syndrome are caused by failure of the kidneys to filter waste products from the blood and remove them in urine. Signs and symptoms of this condition are excessive protein in the urine (proteinuria), increased cholesterol in the blood (hypercholesterolemia), an abnormal buildup of fluid in the abdominal cavity (ascites), and swelling (edema). Affected individuals may also have blood in the urine (hematuria), which can lead to a reduced number of red blood cells (anemia) in the body, abnormal blood clotting, or reduced amounts of certain white blood cells. Low white blood cell counts can lead to a weakened immune system and frequent infections in people with congenital nephrotic syndrome.

Researchers have found that medications are not effective in treating congenital nephrotic syndrome, and that most children will need a kidney transplant by the time they are 2 or 3 years old. A kidney transplant is surgery to place a healthy kidney from someone who has just died or a living donor, most often a family member, into a person’s body to take over the job of the failing kidney.

To keep the child healthy until the transplant, the health care provider may recommend the following:

• albumin injections to make up for the albumin lost in urine
• diuretics to help remove extra fluid that causes swelling
• antibiotics to treat the first signs of infection
• growth hormones to promote growth and help bones mature
• removal of one or both kidneys to decrease the loss of albumin in the urine
• dialysis to artificially filter wastes from the blood if the kidneys fail

If your child has congenital nephrotic syndrome, they’ll need frequent albumin infusions to help them grow and develop normally. This often requires a stay in hospital.

Sometimes parents can be trained to administer the treatment at home. Your child will be regularly reviewed at a clinic, where their blood pressure, growth, weight, kidney function and bone health will be monitored.

It can be difficult for parents to decide which option is best, so you should talk to your doctor about the pros and cons of hospital-based and home-based treatment.

Congenital nephrotic syndrome Genetic Changes

Mutations in the NPHS1 or NPHS2 gene cause most cases of congenital nephrotic syndrome. These genes provide instructions for making proteins that are found in the kidneys. Specifically, the proteins produced from the NPHS1 and NPHS2 genes are found in cells called podocytes, which are located in specialized kidney structures, called glomeruli, that filter the blood. The proteins are found at the podocyte cell surface in the area between two podocytes called the slit diaphragm. The slit diaphragm is known as a filtration barrier because it captures proteins from blood so that they remain in the body while allowing other molecules like sugars and salts to be excreted in urine. The proteins produced from the NPHS1 and NPHS2 genes also help relay cell signals.

Mutations in the NPHS1 or NPHS2 gene result in a decrease or absence of functional protein, which impairs the formation of normal slit diaphragms. Without a functional slit diaphragm, more molecules pass through the kidneys abnormally and get excreted in urine, including proteins and blood cells. The filtering ability of the kidneys worsens from birth, eventually leading to end-stage renal disease.

NPHS1 gene mutations cause all cases of congenital nephrotic syndrome of the Finnish type. This form of the condition is found in people of Finnish ancestry. NPHS1 gene mutations can cause congenital nephrotic syndrome in non-Finnish individuals, but they are a less common cause than NPHS2 gene mutations, which appear to be the most frequent cause of all cases.

Mutations in other genes cause a small number of cases of congenital nephrotic syndrome. Fifteen to 20 percent of individuals with congenital nephrotic syndrome do not have an identified mutation in one of the genes associated with this condition. In these cases, the cause of the condition may be environmental, including infections such as congenital syphilis or toxoplasmosis, or it may be caused by mutations in unidentified genes.

Congenital nephrotic syndrome is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

Figure 5. Congenital nephrotic syndrome autosomal recessive inheritance pattern

Diagnosing nephrotic syndrome in children

Nephrotic syndrome can usually be diagnosed after dipping a dipstick into a urine sample. If there are large amounts of protein in a person’s urine, there will be a color change on the stick.

A blood test showing a low level of a protein called albumin will confirm the diagnosis.

In some cases, when the initial treatment doesn’t work, your child may need a kidney biopsy. This is when a very small sample of kidney tissue is removed using a needle so it can be studied under a microscope.

Pediatric nephrotic syndrome treatment

The main treatment for nephrotic syndrome is steroids, but additional treatments may also be used if a child develops significant side effects.

Most children have relapses until their late teens and need to take steroids when these occur.

Your child may be referred to a childhood kidney specialist (pediatric nephrologist) for tests and specialist treatment.

Steroids

Children diagnosed with nephrotic syndrome for the first time are normally prescribed at least a 4-week course of the steroid medicine prednisolone, followed by a smaller dose every other day for 4 more weeks.

This stops protein leaking from your child’s kidneys into their urine.

When prednisolone is prescribed for short periods, there’s usually no serious or long-lasting side effects, although some children may experience:

• increased appetite
• weight gain
• red cheeks
• mood changes

Most children respond well to treatment with prednisolone, with the protein often disappearing from their urine and the swelling going down within a few weeks. This period is known as remission.

Other medications may be used alongside or in place of steroids if your child’s remission can’t be maintained with steroids or they experience significant side effects.

Additional medications that may be used include:

• levamisole
• cyclophosphamide
• ciclosporin
• tacrolimus
• mycophenolate
• rituximab

Diuretics

Diuretics, or “water tablets”, may also be given to help reduce the fluid build-up. They work by increasing the amount of urine produced.

Penicillin

Penicillin is an antibiotic, and may be prescribed during relapses to reduce the chances of an infection.

Nephrotic syndrome diet

You may be advised to reduce the amount of salt in your child’s diet to prevent further water retention and edema. This means avoiding processed foods and not adding salt to what you eat.

Vaccinations

Children with nephrotic syndrome are advised to have the pneumococcal vaccine. Some children may also be recommended the varicella (chickenpox) vaccination between relapses.

Live vaccines, such as MMR (measles, mumps and rubella vaccine), chickenpox and BCG (Bacillus Calmette-Guérin vaccine or tuberculosis vaccine), shouldn’t be given while your child is taking medication to control their symptoms.

Albumin infusions

Most of the protein lost in nephrotic syndrome is a type called albumin. If your child’s symptoms are severe, they may be admitted to hospital to receive albumin infusions.

Albumin is slowly added to the blood over a few hours through a thin plastic tube called a cannula, which is inserted into one of the veins in their arm.

Caring for your child at home

If your child has been diagnosed with nephrotic syndrome, you’ll need to monitor their condition on a daily basis to check for signs of relapses.

You’ll need to use a dipstick to test your child’s urine for protein the first time they urinate each day.

The results of a dipstick test are recorded as either:

• negative – 0mg of proteinuria per decilitre of urine (mg/dL)
• trace – 15-30mg/dL
• 1+ – 30-100mg/dL
• 2+ – 100-300mg/dL
• 3+ – 300/1,000mg/dL
• 4+ – over 1,000mg/dL

The result for each day needs to be written down in a diary for your doctor or specialist nurse to review during your outpatient appointments.

You should also note down the dose of any medication they’re taking and any other comments, such as whether your child is feeling unwell.

If the dipstick shows 3+ or more of protein in the urine for 3 days in a row, this means your child is having a relapse.

If this happens, you either need to follow the advice given about starting steroids or contact your doctor.

You should seek immediate medical advice if:

• your child has come into contact with someone who has chickenpox or measles and your doctor has told you that your child is not immune to these illnesses
• your child is unwell or has a fever
• your child has diarrhea and is vomiting.

Childhood nephrotic syndrome diet

Children who have nephrotic syndrome may need to make changes to their diet, such as:

• limiting the amount of sodium, often from salt, they take in each day
• reducing the amount of liquids they drink each day
• eating a diet low in saturated fat and cholesterol to help control elevated cholesterol levels

Parents or caretakers should talk with the child’s health care provider before making any changes to the child’s diet.

Dialysis and kidney transplants

In some cases, your doctor may recommend surgery to remove one or both of your child’s kidneys.

This will stop proteins being lost in your child’s urine and reduce their risk of potentially serious problems, such as blood clots.

This means they’ll be dependent on dialysis, where a machine replicates kidney function, from an early age until they can receive a kidney transplant.

A person only needs one kidney to survive, so a living person can donate a kidney. Ideally, this should be a close relative.

Nephrotic vs Nephritic syndrome

Acute nephritic syndrome is a group of disorders that cause inflammation of the internal kidney structures (specifically, the glomeruli) or glomerulonephritis.

Acute nephritic syndrome is often caused by an immune response triggered by an infection or other disease.

Common causes of acute nephritic syndrome in children and adolescents include:

• Hemolytic uremic syndrome
• Henoch-Schönlein purpura
• IgA nephropathy
• Post-streptococcal glomerulonephritis

Common causes of acute nephritic syndrome in adults include:

• Abdominal abscesses
• Goodpasture syndrome
• Hepatitis B or C
• Infective endocarditis
• Membranoproliferative GN I
• Membranoproliferative GN II
• Rapidly progressive (crescentic) glomerulonephritis
• SLE or lupus nephritis
• Vasculitis
• Viral diseases such as mononucleosis, measles, mumps

The inflammation affects the function of the glomerulus. This is the part of the kidney that filters blood to make urine and remove waste. As a result, blood and protein appear in the urine, and excess fluid builds up in the body.

Swelling of the body occurs when the blood loses a protein called albumin. Albumin keeps fluid in the blood vessels. When it is lost, fluid collects in the body tissues.

Blood loss from the damaged kidney structures leads to blood in the urine.

Risk Factors for Acute Nephritic Syndrome (Acute Glomerulonephritis, Acute Nephritis Syndrome)

Infections with Group A Streptococcal bacteria

In warm climates, the disease most commonly follows infected skin lesions and occurs more often in the summer. In cold climates the disease occurs more frequently because of streptococcal throat infection during winter months. The risk of developing acute nephritic syndrome depends on the type of streptococcal bacteria.

Other causes

There are a variety of other causes of acute nephritic syndrome. The following is a list of such conditions:

Primary renal diseases:

• Immunoglobulin A nephropathy;
• Membranoproliferative glomerulonephritis;
• Idiopathic rapidly progressive crescenteric glomerulonephritis.

Secondary renal diseases:

• Subacute bacterial endocarditis;
• Infected ventriculoperitoneal shunt;
• Glomerulonephritis with visceral abscess;
• Glomerulonephritis with bacterial, viral or parasitic infections.

Multi-system disease:

• Systemic lupus erythematosus (SLE);
• Wegener’s granulomatosis;
• Goodpasture’s syndrome;
• Microscopic polyarteritis;
• Mixed cryoglobulinaemia;
• Henoch-Schonlein purpura;
• Haemolytic uraemic syndrome.

Allergy:

Acute allergic tubulointerstitial nephritis.

Acute Nephritic Syndrome (Acute Glomerulonephritis, Acute Nephritis Syndrome) prognosis/outlook

The outlook depends on the disease that is causing the nephritis. When the condition improves, symptoms of fluid retention (such as swelling and cough) and high blood pressure may go away in 1 or 2 weeks. Urine tests may take months to return to normal.

Children tend to do better than adults and usually recover completely. Only rarely do they develop complications or progress to chronic glomerulonephritis and chronic kidney disease.

Adults do not recover as well or as quickly as children. Although it is unusual for the disease to return, in some adults, the disease does return and they will develop end-stage kidney disease and may need dialysis or a kidney transplant. Some forms of glomerulonephritis such as, rapidly progressive crescentic glomerulonephritis move to end stage renal failure if early treatment is not administered (especially when due to anti-glomerular basement membrane antibody disease and systemic vasculitis.)

The microscopic appearance of the kidney defines the prognosis of the condition. Clinical findings of no urine production, heavy amounts of protein in the urine and persistent elevated blood pressure are associated with poor prognosis. Recovery from other post-infectious cases is expected except when associated with the complication of abscess formation where only 50% recover renal function. Recovery from causes other than streptococcal infection is less predictable.

Acute nephritic syndrome symptoms

Common symptoms of nephritic syndrome are:

• Blood in the urine (urine appears dark, tea-colored, or cloudy)
• Decreased urine output (little or no urine may be produced)
• Swelling of the face, eye socket, legs, arms, hands, feet, abdomen, or other areas
• High blood pressure

Other symptoms that may occur include:

• Blurred vision, usually from burst blood vessels in the retina of the eye
• Cough containing mucus or pink, frothy material from fluid buildup in the lungs
• Shortness of breath, from fluid buildup in the lungs
• General ill feeling (malaise), drowsiness, confusion, aches and pains, headache

Symptoms of acute kidney failure or chronic kidney disease may develop.

Acute Nephritic Syndrome (Acute Glomerulonephritis, Acute Nephritis Syndrome) diagnosis

Blood tests and urine analysis will be required to obtain a diagnosis of acute nephritic syndrome. This may require the collection of urine over a 24 hour period to assess the amount of protein lost over this time period. The function of the kidneys will also be tested using a simple blood test to detect signs of renal failure and treat these accordingly.

During an examination, your health care provider may find the following signs:

• High blood pressure
• Abnormal heart and lung sounds
• Signs of excess fluid (edema) such as swelling in the legs, arms, face, and belly
• Enlarged liver
• Enlarged veins in the neck

Tests that may be done include:

• Blood electrolytes
• Blood urea nitrogen (BUN)
• Creatinine – blood
• Creatinine clearance
• Potassium test
• Protein in the urine
• Urinalysis
• Urine appearance and color

A kidney biopsy will show inflammation of the glomeruli, which may indicate the cause of the condition.

Tests to find the cause of acute nephritic syndrome may include:

• ANA titer (lupus)
• Antiglomerular basement membrane antibody
• Antineutrophil cytoplasmic antibody for vasculitis (ANCA)
• Blood culture
• Culture of the throat or skin
• Serum complement (C3 and C4)

Acute nephritic syndrome treatment

The goal of treatment is to reduce inflammation in the kidney and control high blood pressure. You may need to stay in a hospital to be diagnosed and treated.

Your provider may recommend:

• Bed rest until you feel better with treatment
• A diet that limits salt, fluids, and potassium
• Medicines to control high blood pressure, reduce inflammation, or to remove fluid from your body
• Kidney dialysis, if needed

The principles of managing acute nephritic syndrome include:

• Control and prevention of elevated blood pressure: The presence of hypertension can increase the rate of deterioration of renal function in this condition. This may involve the use of anti-hypertension medications, and restriction of fluid and salt from the diet.
• Anti-inflammatory and immunosupressant therapy: To control the inflammatory process of some causes of nephritic syndrome including vasculitis causes.
• Supportive therapy for renal failure: Blood tests will be required regularly to ensure that renal failure is diagnosed and controlled early. Dialysis may be required if the renal failure cannot be controlled by conservative means.
• Antibiotic therapy: For those with proven post-streptococcal nephritic syndrome.

Nephrotic syndrome causes

Nephrotic syndrome can be caused by diseases that affect the kidneys directly, such as focal segmental glomerulosclerosis (FSGS) or membranous nephropathy, as well as underlying diseases that affect the whole body.

Diseases that affect only the kidneys are called primary causes of nephrotic syndrome. The glomeruli are usually the targets of these diseases for reasons that are not fully understood. In focal segmental glomerulosclerosis—the most common primary cause of nephrotic syndrome—scar tissue forms in parts of the glomeruli. In membranous nephropathy, immune molecules form harmful deposits on the glomeruli.

Nephrotic syndrome can also be caused by systemic diseases, which are diseases that affect many parts of the body, such as diabetes or lupus. Systemic diseases that affect the kidneys are called secondary causes of nephrotic syndrome. More than 50 percent of nephrotic syndrome cases in adults have secondary causes, with diabetes being the most common ³⁾.

Kidney diseases that commonly cause nephrotic syndrome include:

• Minimal change disease – this causes a small change in the filters of the kidneys. This is the most common cause of nephrotic syndrome in children. Minimal change disease results in abnormal kidney function, but when the kidney tissue is examined under a microscope, it appears normal or nearly normal. The cause of the abnormal function typically can’t be determined.
• Focal segmental glomerulosclerosis – this causes scar tissue to build up in the kidneys’ filters. Characterized by scattered scarring of some of the glomeruli, this condition may result from another disease or a genetic defect or occur for no known reason.
• Membranous glomerulopathy, also called glomerulous nephropathy – this causes thickening of the lining of the filters. This kidney disorder is the result of thickening membranes within the glomeruli. The exact cause of the thickening isn’t known, but it’s sometimes associated with other medical conditions, such as hepatitis B, malaria, lupus and cancer.
• Nephritis, which is inflammation of the kidneys.
• Diabetic kidney disease. Diabetes can lead to kidney damage (diabetic nephropathy) that affects the glomeruli.
• Systemic lupus erythematosus. This chronic inflammatory disease can lead to serious kidney damage.
• Amyloidosis. This disorder occurs when substances called amyloid proteins accumulate in your organs. Amyloid buildup often affects the kidneys, damaging their filtering system.
• Blood clot in a kidney vein. Renal vein thrombosis, which occurs when a blood clot blocks a vein connected to the kidney, can cause nephrotic syndrome.

Risk factors for nephrotic syndrome

Factors that can increase your risk of nephrotic syndrome include:

• Medical conditions that can damage your kidneys. Certain diseases and conditions increase your risk of developing nephrotic syndrome, such as diabetes, lupus, amyloidosis and other kidney diseases.
• Certain medications. Examples of medications that can cause nephrotic syndrome include nonsteroidal anti-inflammatory drugs and drugs used to fight infections.
• Certain infections. Examples of infections that increase the risk of nephrotic syndrome include HIV, hepatitis B, hepatitis C and malaria.

Nephrotic syndrome signs and symptoms

One of the most common symptoms of nephrotic syndrome is fluid retention, which causes puffy eyes, a swollen abdomen and swollen ankles and feet.

Other symptoms can include:

• frothy urine
• extreme tiredness
• infections
• anemia (lack of red blood cells)
• hyperlipidemia
• hypoalbumina.

Nephrotic syndrome can lead to serious illness such as blood clots and kidney failure.

People with nephrotic syndrome may also experience:

• weight gain due to excess fluid retention
• fatigue
• loss of appetite

Nephrotic syndrome complications

The loss of different proteins from the body can lead to a variety of complications in people with nephrotic syndrome. Blood clots can form when proteins that normally prevent them are lost through the urine. Blood clots can block the flow of blood and oxygen through a blood vessel. Loss of immunoglobulins—immune system proteins that help fight disease and infection—leads to an increased risk of infections. These infections include pneumonia, a lung infection; cellulitis, a skin infection; peritonitis, an abdominal infection; and meningitis, a brain and spine infection. Medications given to treat nephrotic syndrome can also increase the risk of these infections.

Other complications of nephrotic syndrome include:

• Hypothyroidism—a condition in which the thyroid gland does not produce enough thyroid hormone to meet the body’s needs
• Anemia—a condition in which red blood cells are fewer or smaller than normal, which means less oxygen is carried to the body’s cells
• Coronary artery disease, also called coronary heart disease—heart disease caused by narrowing of the arteries that supply blood to the heart
• High blood cholesterol and elevated blood triglycerides. When the level of the protein albumin in your blood falls, your liver makes more albumin. At the same time, your liver releases more cholesterol and triglycerides.
• High blood pressure, also called hypertension—a condition in which blood flows through the blood vessels with a force greater than normal
• Acute kidney failure. If your kidneys lose their ability to filter blood due to damage to the glomeruli, waste products may build up quickly in your blood. If this happens, you may need emergency dialysis — an artificial means of removing extra fluids and waste from your blood — typically with an artificial kidney machine (dialyzer).
• Chronic kidney disease. Nephrotic syndrome may cause your kidneys to gradually lose their function over time. If kidney function falls low enough, you may require dialysis or a kidney transplant.
• Infections. People with nephrotic syndrome have an increased risk of infections.

Nephrotic syndrome diagnosis

Nephrotic syndrome can usually be diagnosed after dipping a dipstick into a urine sample. If there are large amounts of protein in a person’s urine, there will be a color change on the stick.

To confirm whether you have nephrotic syndrome, your doctor is likely to:

• assess your symptoms
• take your medical history
• examine you
• ask you to do a series of urine tests, usually over a 24-hour period
• take a blood sample for testing.

A more precise measurement is usually needed to confirm the diagnosis. Either a single urine sample or a 24-hour collection of urine can be sent to a lab for analysis. With the single urine sample, the lab measures both albumin and creatinine, a waste product of normal muscle breakdown. The comparison of the measurements is called a urine albumin-to-creatinine ratio. A urine sample containing more than 30 milligrams of albumin for each gram of creatinine may signal a problem. With a 24-hour collection of urine, the lab measures only the amount of albumin present. The single urine sample is easier to collect than the 24-hour sample and is usually sufficient to confirm diagnosis, though the 24-hour collection may be used in some cases.

Once nephrotic syndrome is diagnosed, blood tests are usually needed to check for systemic diseases that may be causing the nephrotic syndrome and to find out how well the kidneys are working overall. A blood test involves drawing blood at a health care provider’s office or commercial facility and sending the sample to a lab for analysis.

Though blood tests can point toward systemic diseases, a kidney biopsy is usually needed to diagnose the specific underlying disease causing the nephrotic syndrome and to determine the best treatment. A kidney biopsy is a procedure that involves taking a piece of kidney tissue for examination with a microscope. Kidney biopsies are performed by a health care provider in a hospital with light sedation and local anesthetic. A biopsy is often not needed for a person with diabetes because the person’s medical history and lab tests may be enough to diagnose the problem as being a result of diabetes.

Nephrotic syndrome treatment

Treating nephrotic syndrome includes addressing the underlying cause as well as taking steps to reduce high blood pressure, edema, high cholesterol, and the risks of infection. Treatment usually includes medications and changes in diet.

Nephrotic syndrome diet

It will be important to follow the right diet. You might see a dietitian who is likely to recommend a diet with:

• a reasonable amount of protein, but not too much
• not much fat
• very little salt.

You might also be advised to restrict how much fluid you drink.

Eating, diet, and nutrition have not been shown to play a role in causing or preventing nephrotic syndrome in adults. For people who have developed nephrotic syndrome, limiting intake of dietary sodium, often from salt, and fluid may be recommended to help reduce edema. A diet low in saturated fat and cholesterol may also be recommended to help control hyperlipidemia.

Medications

Medications that lower blood pressure can also significantly slow the progression of kidney disease causing nephrotic syndrome. Two types of blood pressure lowering medications, angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers, have proven effective in slowing the progression of kidney disease by reducing the pressure inside the glomeruli and thereby reducing albuminuria. Many people require two or more medications to control their blood pressure. In addition to an angiotensin-converting enzyme (ACE) inhibitor or an angiotensin receptor blocker, a diuretic—a medication that aids the kidneys in removing fluid from the blood—can also be useful in helping to reduce blood pressure as well as edema. Beta blockers, calcium channel blockers, and other blood pressure medications may also be needed.

Statin medications may be given to lower cholesterol.

People with nephrotic syndrome should receive the pneumococcal vaccine, which helps protect against a bacterium that commonly causes infection, and yearly flu shots.

Blood thinning medications are usually only given to people with nephrotic syndrome who develop a blood clot; these medications are not used as a preventive measure.

Medicines to manage the symptoms, such as:

• fluid tablets (diuretics) – to reduce fluid retention
• blood pressure medicine – to reduce pressure in your kidneys’ filters, so that less protein is filtered out
• pneumococcal vaccine – if you are at high risk of infection
• anti-clotting medicine – to help thin your blood if there is a risk of blood clots forming
• steroid tablets – which seems to be helpful for minimal change disease
• cholesterol-reducing medications. Medications called statins can help lower cholesterol levels. However, it’s currently unclear whether or not cholesterol-lowering medications can specifically improve the outcomes of people with nephrotic syndrome, such as avoiding heart attacks or decreasing the risk of early death. Statins include atorvastatin (Lipitor), fluvastatin (Lescol), lovastatin (Altoprev), pravastatin (Pravachol), rosuvastatin (Crestor) and simvastatin (Zocor).

Nephrotic syndrome may go away once the underlying cause has been treated.

If you suspect you have nephrotic syndrome symptoms, contact your doctor.

Lifestyle and home remedies

Changes to your diet may help you cope with nephrotic syndrome. Your doctor may refer you to a dietitian to discuss how what you eat can help you cope with the complications of nephrotic syndrome. A dietitian may recommend that you:

• Choose lean sources of protein
• Reduce the amount of fat and cholesterol in your diet to help control your blood cholesterol levels
• Eat a low-salt diet to help control the swelling (edema) you experience
• Limit foods that increase blood sugar levels when taking medications that can lead to weight gain, such as steroids

Some people with nephrotic syndrome may also be deficient in the mineral zinc. A recent study showed treatment with zinc supplements in children under 18 improved nephrotic syndrome. But always check with your doctor before giving your child a supplement or taking one yourself to avoid any potential adverse interactions.

What is hypospadias

Hypospadias is a birth defect (congenital condition) in which a baby boy’s urethra is located on the under side of his penis rather than at the tip ¹⁾; you may have concerns about what this means for everything from toilet training to his future life as an adult. In boys with hypospadias, the urethra forms abnormally during weeks 8–14 of pregnancy. The abnormal opening can form anywhere from just below the end of the penis to the scrotum.

Hypospadias is common and doesn’t cause difficulty in caring for your infant. The urethra is the tube through which urine drains from your bladder and exits your body.

Hypospadias is fairly common birth defect affecting about 1 in 200 to 1 in 300 male newborns ²⁾. Hypospadias is often readily corrected through outpatient surgery. Hypospadias also occurs in girls, but it’s extremely rare (affecting an estimated one in 500,000 babies) and a vastly different condition. If your daughter is born with hypospadias, your child’s specialist will be your best source of information and support.

In hypospadias, the urethral opening can be located at any point along the underside of the penis (also called the “ventral aspect”) (see Figure 1). Where the opening falls will determine how severe the condition is, and how your child’s medical team will approach repairing it.

There are different degrees of hypospadias; some can be minor and some more severe.

• Subcoronal hypospadias – anterior or distal (near the tip of the penis): The opening of the urethra is located somewhere near the head of the penis. This is the mildest form of hypospadias, occurring in about 50 percent of cases.
• Midshaft hypospadias – middle (midway up the penis): The opening of the urethra is located along the shaft of the penis. Considered moderate hypospadias, this accounts for about 30 percent of cases.
• Penoscrotal hypospadias – posterior or proximal (at the scrotum or perineum): The opening of the urethra is located where the penis and scrotum meet. This is the most severe kind of hypospadias, and occurs in 20 percent of cases.

Some parents may confuse hypospadias with epispadias, in which the urethra opens along the top of the penis, but these are two separate and distinct conditions with very different treatments.

Boys with hypospadias can sometimes have a curved penis. They could have problems with abnormal spraying of urine and might have to sit to urinate. In some boys with hypospadias, the testicle has not fully descended into the scrotum (cryptorchidism).

While some children with very mild forms of this condition may not require surgery, if your son has hypospadias you should seek an evaluation from a pediatric urologic surgeon.

Surgery usually restores the normal appearance of your child’s penis. The outlook for infants who undergo this operation is extremely good: In most instances, they make a full recovery and have a normal-looking, fully functional penis within about six months. With successful treatment of hypospadias, most males can have normal urination and reproduction.

If left untreated, more severe forms of hypospadias can interfere with sexual intercourse when your child is an adult.

Figure 1. Hypospadias types

Figure 2. Normal penis with normal urethral opening

Epispadias

Epispadias is a congenital condition in which the urethra most often opens on the top or side of the penis instead of the tip. Moreover, the urethra may also be open along the whole length of the penis. This birth defect is most commonly observed as a part of the exstrophy epispadias complex, which is a spectrum of failures of midline fusion affecting the pelvis, abdominal wall and urinary bladder. Characteristics of epispadias include a very short and wide penis often classified as complete epispadias when the entire urethra is exposed from the abdominal wall to the tip of the penis. There is usually an upward bending of the penis (chordee) associated with epispadias.

Epispadias can also occur in females, where it is manifested as a split clitoris and displacement of the urethral opening.

Epispadias is always part of the exstrophy epispadias complex, but can also occur as an isolated birth defect in a child with an otherwise normal bladder, pelvis and abdominal wall. In isolated epispadias, the bladder is closed and covered by the lower abdominal wall muscles and skin. In some children with epispadias, there may be bony- pelvis abnormalities similar to, but not as severe as, that seen with bladder exstrophy.

Isolated male epispadias in the absence of bladder-exstrophy-epispadias complex is a rare malformation, with an estimated incidence of around 1 per 117,000 live male births and only one of every 484,000 female births ³⁾. Like hypospadias, epispadias covers a wide spectrum in which the urethral opening can be located anywhere from the distal penile shaft to the pubic area. Unlike in hypospadias, the severity of the condition is related not only to the urethral opening but also to the degree of incontinence associated with the urethral opening position, as the bladder neck might be involved in more proximal variants of isolated male epispadias ⁴⁾.

Bladder exstrophy is a rare, complex birth defect involving the urinary, reproductive and intestinal tracts, as well as the musculoskeletal system. During a baby’s development in the womb, the abdominal wall and underlying organs sometimes do not fuse properly, and the infant is born with the bladder inside out and exposed on the outside of the body.

Bladder exstrophy usually involves several systems within the body, including the urinary tract, reproductive tract (external genitalia) and pelvic skeletal muscles and bones. In rare cases, intestinal tracts are involved.

Bladder exstrophy can result in weakened abdominal muscles and a shorter than average urethra and vagina or penis. It can cause a variety of complications and problems, including incontinence, catheterization, reflux, infertility and the need for repeated reconstructive surgery. It may be associated with urinary incontinence and back up of urine from the bladder to the kidneys, called vesicoureteral reflux.

What causes exstrophy of the bladder and epispadias?

The cause of bladder exstrophy is unknown. Some studies show a clustering of the condition in families, suggesting that there’s an inherited factor. Popular theories suggest overdevelopment of a normal structure known as the cloacal membrane. Overdevelopment or prolonged presence of the cloacal membrane may prevent appropriate tissue development, ingrowth and the joining together of the supportive lower abdominal wall structure. However, the chance for parents to have another child with exstrophy of the bladder is less than one percent.

Risk factors for exstrophy-epispadias complex include Caucasian race, young maternal age, and maternal multiparity ⁵⁾. Furthermore, exstrophy-epispadias complex is increased among children conceived with assisted-reproductive technologies such as in vitro fertilization ⁶⁾.

Patients who have more than a mild case of epispadias will need surgery.

Leakage of urine (incontinence) can often be repaired at the same time. Seventy-five percent of male epispadias patients are incontinent, but 80% are continent postoperatively ⁷⁾. Those who are still incontinent may require later bladder neck reconstruction ⁸⁾. Although there is a paucity of literature on isolated female epispadias, reports suggest 87% to 100% continence after repair ⁹⁾.

How is bladder exstrophy treated?

Treatment for bladder exstrophy begins at birth. Your care team should be readily available to help you in the immediate post-natal period. The primary goal when caring for a child with bladder exstrophy is to:

• preserve normal kidney function
• develop adequate bladder function and promote urinary continence
• provide acceptable appearance and function of the external genitalia
• ensure that your child has a typical and normal childhood

There are two main approaches to the repair of bladder exstrophy:

Complete primary repair of exstrophy

Complete primary repair of exstrophy is a procedure in which both the bladder is closed and the epispadias is repaired (entire urethra is closed into a tube) at the same time. Children who undergo complete primary repair of exstrophy will likely require additional surgery during later years to manage urinary incontinence and vesicoureteral reflux. This procedure is usually performed at approximately six-to-eight weeks of life. Delaying complete primary repair of exstrophy beyond the first, 2-to-3 days of life may have several advantages for the child. The delay presents an opportunity for normal bonding between the baby and parents before the initial reconstructive surgery and lengthy recovery period that follows.

The time between birth and initial repair allows for growth, development and additional maturation of some organs and systems prior to the complex surgery and makes the anesthesia and surgery safer.

Modern staged repair of exstrophy

Modern staged repair of exstrophy involves three surgeries for reconstruction of the bladder:

• The initial repair: The bladder and abdominal wall are closed, the belly button is reconstructed and an osteotomy is sometimes performed (the pelvic bones are reformed to aid in the repair). The initial repair is usually performed in the first 2-to-3 days of life.
• The second stage: In boys, the proximal part of the urethra close to the bladder is closed along with the bladder. In girls, the urethra is usually closed along its entire length, extending all the way from the bladder to the surface of the skin between the labia as it normally should be. The second stage repair occurs around 6-to-12 months of age.
• The third stage: This stage involves bladder neck reconstruction with bilateral ureteral reimplantation. This stage is performed when the bladder has grown sufficiently to hold an appropriate volume of urine. This procedure is usually performed between the ages of 6-to-10 years of age.

Possible complications of surgery

Some people with this condition may continue to have urinary incontinence, even after surgery.

Ureter and kidney damage and infertility may occur.

What is the long-term outlook for bladder exstrophy?

After initial treatment for bladder exstrophy, lifelong follow-up care is necessary. Care is ongoing and can involve a range of surgical procedures and tests. Every child is different, and our doctors and nurses will work with you to develop the best plan for your baby. In many cases, additional surgery is required during childhood, adolescence and young adulthood. To ensure that your child’s kidneys and bladder are functioning properly, follow-up testing, such as a renal ultrasound, may be necessary.

Hypospadias causes

Hypospadias is present at birth (congenital). As the penis develops in a male fetus, certain hormones stimulate the formation of the urethra and foreskin. Hypospadias results when a malfunction occurs in the action of these hormones, causing the urethra to develop abnormally. As the fetus develops, the tissue on the underside of the penis that forms the urethra doesn’t completely close, shortening the passageway. In many cases, the foreskin—the fold of skin covering the penis tip, or glans—also doesn’t develop properly, resulting in extra foreskin on the top side of the penis and none on the underside.

In most cases, the exact cause of hypospadias is unknown. Sometimes, hypospadias is genetic, but environment also may play a role. Hypospadias is slightly more common in boys whose father or brother also had the condition.

Risk factors for hypospadias

Although the cause of hypospadias is usually unknown, these factors may be associated with the condition:

• Family history. This condition is more common in infants with a family history of hypospadias.
• Genetics. Certain gene variations may play a role in disruption of the hormones that stimulate formation of the male genitals.
• Maternal age over 35 and weight. Some research suggests that there may be an increased risk of hypospadias in infant males born to women older than 35 years and who were considered obese had a higher risk of having a baby with hypospadias ¹⁰⁾.
• Fertility treatments: Women who used assisted reproductive technology to help with pregnancy had a higher risk of having a baby with hypospadias ¹¹⁾.
• Exposure to certain substances during pregnancy. There is some speculation about an association between hypospadias and a mother’s exposure to certain hormones ¹²⁾ or certain compounds such as pesticides or industrial chemicals, but further studies are needed to confirm this.

Hypospadias symptoms

In hypospadias, the opening of the urethra is located on the underside of the penis instead of at the tip. In most cases, the opening of the urethra is within the head of the penis. Less often, the opening is at the middle or the base of the penis. Rarely, the opening is in or beneath the scrotum.

Signs and symptoms of hypospadias may include:

• Opening of the urethra at a location other than the tip of the penis
• Downward curve of the penis (chordee)
• Hooded appearance of the penis because only the top half of the penis is covered by foreskin
• Abnormal spraying during urination or a downward urinary spray (in older children with more severe hypospadias, this may mean he has to sit down to urinate)
• An abnormal appearance of the tip of the penis (the glans)
• In some cases, boys born with hypospadias may also have undescended testicles and/or inguinal hernias (that is, hernias of the groin).

Hypospadias won’t cause your son physical pain or block his urination (though if it goes untreated it can make it difficult for him to direct his urine spray).

Hypospadias complications

If hypospadias is not treated, it can result in:

• Abnormal appearance of the penis
• Problems learning to use a toilet
• Abnormal curvature of the penis with erection
• Problems with impaired ejaculation

Hypospadias diagnosis

Your child’s pediatrician can diagnose hypospadias based on a physical exam. He or she will likely refer you to a surgeon who specializes in genital and urinary conditions (pediatric urologist) for further evaluation. Medical centers with specialty teams can help you evaluate options and can provide expert treatment.

When the opening of the urethra is abnormal and the testicles cannot be felt on exam, the genitals may be difficult to identify as clearly male or female (ambiguous genitalia). In this case, further evaluation with a multidisciplinary team is recommended.

Hypospadias treatment

If your son has a very mild case, he may not require surgery because his condition will not have a large impact on his life. However, sometimes parents of boys born with minor abnormalities still opt for surgery for cosmetic reasons, like straightening the penis and removing excess foreskin. However, treatment usually involves surgery to reposition the urethral opening and, if necessary, straighten the shaft of the penis. Surgery is usually done between the ages of 6 and 12 months.

If the penis looks abnormal, circumcision should not be done. If hypospadias is found during circumcision, the procedure should be completed. In either case, referral to a pediatric urologist is recommended.

Are there any medical alternatives to surgery?

No, surgery remains the best and only way to resolve your son’s urinary difficulties; to straighten and repair his penis so it will look more normal; and to help ensure that he will have full sexual function as an adult.

Hypospadias surgery

Most forms of hypospadias can be corrected in a single surgery that’s done on an outpatient basis. Some forms of hypospadias will require more than one surgery to correct the defect.

When the urethral opening is near the base of the penis, the surgeon may need to use tissue grafts from the foreskin or from the inside of the mouth to reconstruct the urinary channel in the proper position, correcting the hypospadias.

After surgery

Depending on the extent of surgery, your child may either go home the same day or stay in the hospital overnight. Your child’s surgeon/nurse will talk with you how to care for him at home and will provide detailed instruction sheets (how to manage his dressing, what activities should be avoided, etc.).

Also, your son’s surgeon will likely have inserted a soft drainage catheter, or stent, into the new urethra during the procedure. The stent will remain in your child’s urethra (you will be able to see the tip) to hold it open while it heals and to allow urine to drain from the bladder.

What to Expect at Home

Your child may feel sleepy when first at home. He may not feel like eating or drinking. He may also feel sick to his stomach or throw up the same day he had surgery.

Your child’s penis will be swollen and bruised. This will get better after a few weeks. Full healing will take up to 6 months.

Your child may need a urinary catheter for 5 to 14 days after the surgery.

• The catheter may be held in place with small stitches. The health care provider will remove the stitches when your child does not need the catheter anymore.
• The catheter will drain into your child’s diaper or a bag taped to his leg. Some urine may leak around the catheter when he urinates. There may also be a spot or two of blood. This is normal.

If your child has a catheter, he may have bladder spasms. These may hurt, but they are not harmful. If a catheter has not been put in, urinating be uncomfortable the first day or 2 after surgery.

Your child’s provider may write a prescription for some medicines:

• Oral antibiotics to prevent infection.
• Antibiotic ointment, to be applied to the penis several times a day
• Antispasmodic medication to relax the bladder and stop bladder spasms. These may cause your child’s mouth to feel dry.
• Prescription pain medicine, if needed. You can also give your child acetaminophen (Tylenol) for pain and soreness in the first few days after surgery

Taking Care of Your Child

Your child may eat a normal diet. Make sure he drinks plenty of fluids. Fluids help keep the urine clean.

A dressing with a clear plastic covering will be wrapped around the penis.

• If stool gets on the outside of the dressing, clean it gently with soapy water. Be sure to wipe away from the penis. DO NOT scrub.
• Give your child sponge baths until the dressing is off. When you do start bathing your son, use only warm water. DO NOT scrub. Gently pat him dry afterwards.

Some oozing from the penis is normal. You may see some spotting on the dressings, diaper, or underpants. If your child is still in diapers, ask your provider about how to use two diapers instead of one.

DO NOT use powders or ointments anywhere in the area before asking your child’s provider if it is ok.

Your child’s provider will probably ask you to take off the dressing after 2 or 3 days and leave it off. You may do this during a bath. Be very careful not to pull on the urine catheter. You will need to change the dressing before this if:

• The dressing rolls down and is tight around the penis.
• No urine has passed through the catheter for 4 hours.
• Stool gets underneath the dressing (not just on top of it).

Infants may do most of their normal activities except for swimming or playing in a sandbox. It is fine to take your baby for walks in the stroller.

Older boys should avoid contact sports, riding bicycles, straddling any toys, or wrestling for 3 weeks. It is a good idea to keep your child home from preschool or daycare the first week after his surgery.

When to Call the Doctor

Call the health care provider if your child has:

• Persistent low-grade fever or fever over 101°F (38.3°C) in the week after surgery.
• Increased swelling, pain, drainage, or bleeding from the wound.
• Trouble urinating.
• A lot of urine leakage around the catheter. This means the tube is blocked.

Also call if:

• Your child has thrown up more than 3 times and cannot keep fluid down.
• The stitches holding the catheter come out.
• The diaper is dry when it is time to change it.
• You have any concerns about your child’s condition.

Results of surgery

In most cases, surgery is highly successful. Most of the time the penis looks normal after surgery, and boys have normal urination and reproduction.

Occasionally, a hole (fistula) develops along the underside of the penis where the new urinary channel was created and results in urine leakage. Rarely, there is a problem with wound healing or scarring. These complications may require an additional surgery for repair.

Follow-up care

Your child’s doctor will remove the stent during a follow-up appointment 7 to 10 days after the surgery.

Your child will need a couple of visits to the surgeon after surgery. You typically can expect to see the full results of successful surgery within six months.

After that, regular follow-up with your child’s pediatric urologist is recommended after toilet training and at puberty to check for healing and possible complications.

When should the operation be scheduled?

This depends on what kind of hypospadias your child has, and whether he was diagnosed at birth (most boys are). Typically, if he requires surgery, we recommend that it be scheduled when he is between 4 and 6 months old.

Will this affect when and whether my son will be circumcised?

Babies who have hypospadias that requires surgery shouldn’t be circumcised, because the foreskin may be needed for tissue grafts during the operation.

How soon after surgery will my son’s penis looks like other little boys’?

It varies from patient to patient, but typically you’ll be able to see the full results of your son’s surgery after six months.