Birth asphyxia

Birth asphyxia also known as neonatal asphyxia or perinatal asphyxia is the medical condition resulting from deprivation of oxygen to a newborn infant that lasts long enough during the birth process to cause physical harm, usually to the brain. Birth asphyxia is a lack of blood flow or gas exchange to or from the fetus in the period immediately before, during, or after the birth process ¹⁾. Perinatal asphyxia can result in profound systemic and neurologic complications due decreased blood flow and/or oxygen to a fetus or infant during the peripartum period. When placental (prenatal) or pulmonary (immediate post-natal) gas exchange is compromised or ceases altogether, there is partial lack of oxygen (hypoxia) or complete lack of oxygen (anoxia) to the vital organs. This results in progressive hypoxemia (low level of oxygen in the blood) and hypercapnia (too much carbon dioxide or CO2 in the blood). If the hypoxemia is severe enough, the tissues and vital organs (muscle, liver, heart, and ultimately the brain) will develop an oxygen debt. Anaerobic glycolysis and lactic acidosis will result. Neonatal hypoxic-ischemic encephalopathy refers specifically to the neurologic complication of perinatal asphyxia ²⁾.

The diagnostic criteria for neonatal hypoxic-ischemic encephalopathy are as follows:

• Metabolic acidosis with pH <7.0 (in umbilical cord or infant blood sample)
• Base Deficit -12
• APGAR score = five at 10 minutes with a continued need for resuscitation
• Presence of multiple organ-system failures
• Clinical evidence of encephalopathy: hypotonia, abnormal oculomotor or pupillary movements, weak or absent suck, apnea, hyperpnea, or clinical seizures
• Neurologic findings cannot be attributed to other cause (inborn error of metabolism, a genetic disorder, congenital neurologic disorder, medication effect).

The incidence of perinatal asphyxia is two per 1000 births in developed countries, but the rate is up to 10 times higher in developing countries where there may be limited access to maternal and neonatal care. Of those infants affected, 15-20% die in the neonatal period, and up to 25% of survivors are left with permanent neurologic deficits ³⁾.

Some of the causes of decreased oxygen before or during the birth process may include:

• Inadequate oxygen levels in the mother’s blood due to heart or respiratory problems or lowered respirations caused by anesthesia
• Low blood pressure in the mother
• Inadequate relaxation of the uterus during labor that prevents oxygen circulation to the placenta
• Early separation of the placenta from the uterus, called placental abruption
• Compression of the umbilical cord that decreases blood flow
• Poor placenta function that may occur with high blood pressure or in post-term pregnancies, particularly those past 42 weeks

Factors that may lower oxygen in the baby after birth include:

• Severe anemia, or a low blood cell count, that limits the oxygen-carrying ability of the blood
• Low blood pressure or shock
• Respiratory problems that limit oxygen intake
• Heart or lung disease

Low oxygen levels may decrease a baby’s heart rate, blood pressure and blood flow out of the heart. This may limit the blood flow to organs and tissues, leading to improper cell function or damage. Organs typically affected by lowered oxygen include the brain, heart and blood vessels, gastrointestinal tract, lungs and kidneys.

Birth asphyxia is not an uncommon event, and because of its high morbidity and mortality, the condition is best managed by an interprofessional team.

Causes of birth asphyxia

Perinatal asphyxia can occur due to maternal hemodynamic compromise (amniotic fluid embolus), uterine conditions (uterine rupture), or placenta and umbilical cord (placental abruption, umbilical cord knot or compression) and infection. The asphyxia can occur prior to the birth or can occur immediately following birth in a compromised patient requiring resuscitation ⁴⁾.

The majority of cases of perinatal asphyxia occur intrapartum, although 20% occur antepartum and other cases occur in the early post-natal period. Perinatal asphyxia can occur due to maternal events (hemorrhage, amniotic fluid embolism; hemodynamic collapse), placental events (acute abruption), uterine events (rupture), cord events (tight nuchal cord, cord prolapse/avulsion) and intrapartum infection (maternal fever in labor). A careful obstetrical and peripartum history is essential to determine the cause.

Perinatal asphyxia pathophysiology

There are three stages to brain injury in hypoxic-ischemic encephalopathy. First, there is an immediate primary neuronal injury that occurs due to interruption of oxygen and glucose to the brain. This decreases ATP and results in failure of the ATP-dependent NaK pump. Sodium enters the cell followed by water, causing cell swelling, widespread depolarization, and cell death. Cell death and lysis cause release of glutamate, an excitatory amino acid, which causes an increase in intracellular calcium and further cell death.

Following the immediate injury is a latent period of about six hours, during which reperfusion occurs, and some cells recover.

Late secondary neuronal injury occurs over the next 24-48 hours as reperfusion results in blood flow to and from damaged areas, spreading toxic neurotransmitters and widening the area of brain affected.

Birth asphyxia stages

Perinatal asphyxia can result in systemic effects, including neurologic insult, respiratory distress and pulmonary hypertension, and liver, myocardial, and renal dysfunction. Depending on the severity and timing of the hypoxic insult, a neonate with hypoxic-ischemic encephalopathy due to perinatal asphyxia can demonstrate a variety of neurologic findings. Using the Sarnat staging for encephalopathy can be useful.

• Sarnat Stage 1, the least severe stage, there is generalized sympathetic tone and the neonate may be hyper-alert with prolonged periods of wakefulness, mydriasis and increased deep tendon reflexes.
• Sarnat Stage 2, the neonate may be lethargic or obtunded, with decreased tone, strong distal flexion, and generalized parasympathetic tone with miosis, bradycardia and increased secretions. Seizures are common in Sarnat Stage 2.
• Sarnat Stage 3, the most severe, is characterized by a profoundly decreased level of consciousness, flaccid tone, decreased deep tendon reflexes and very abnormal EEG. Clinical seizures are less common in Sarnat Stage 3 due to the profound injury in the brain preventing the propagation of clinical seizures.

Symptoms of birth asphyxia

Each baby may experience symptoms of birth asphyxia differently. However, the following are the most common symptoms.

Before delivery, symptoms may include:

• Abnormal heart rate or rhythm
• An increased acid level in a baby’s blood

At birth, symptoms may include:

• Bluish or pale skin color
• Low heart rate
• Weak muscle tone and reflexes
• Weak cry
• Gasping or weak breathing
• Meconium — the first stool passed by the baby — in the amniotic fluid, which can block small airways and interfere with breathing

Birth asphyxia diagnosis

The following test are used to diagnose birth asphyxia:

• Severe acid levels — pH less than 7.00 — in the arterial blood of the umbilical cord.
• Apgar score of zero to three for longer than five minutes. The Apgar test is used just after birth to evaluate a newborn’s color, heartbeat, reflexes, muscle tone and respiration.
• Neurological problems, such as seizures, coma and poor muscle tone.
• Respiratory distress, low blood pressure, or other signs of low blood flow to the kidneys or intestines.

Problems with a baby’s circulatory, digestive and respiratory systems may also suggest that a baby has birth asphyxia.

A chest radiograph may determine the need for intubation and/or need for exogenous surfactant therapy. An arterial blood gas is useful in diagnosing respiratory versus metabolic acidosis and degree of hypoxemia. Liver damage can be determined by serum transaminase levels and coagulation factors. Troponin and CK-MB can be useful in determining myocardial insult and creatinine and blood urea nitrogen can ascertain the extent of renal dysfunction. Physiologically stressed infants rapidly deplete glucose stores and can develop profound hypoglycemia. Frequent blood glucose checks during the critical period of resuscitation are recommended ⁵⁾.

Birth asphyxia treatment

Birth asphyxia is a complex condition that can be difficult to predict or prevent. Prompt treatment is important to minimize the damaging effects of decreased oxygen to the baby.

Specific treatment for birth asphyxia is based on:

• The baby’s age, overall health and medical history
• Severity of the baby’s condition
• The baby’s tolerance for specific medications, procedures or therapies
• Expectations for the course of the condition

Treatment may include:

• Giving the mother extra oxygen before delivery
• Emergency delivery or Caesarean section
• Assisted ventilation and medications to support the baby’s breathing and blood pressure
• Extracorporeal membrane oxygenation (ECMO). An extracorporeal membrane oxygenation (ECMO) machine may be used for babies who are experiencing serious heart or lung failure. The machine delivers oxygen to the baby’s brain and body as temporary support. It works by draining the baby’s blood into an artificial lung where oxygen is added and carbon dioxide is removed, then pumping the blood back into the child.

Therapeutic hypothermia is the treatment for neonatal hypoxic-ischemic encephalopathy ⁶⁾. Following the immediate primary neuronal injury, during which there is an interruption of oxygen and glucose to the brain, there is a latent period of up to 6 hours before a secondary phase of injury occurs as the injured areas are reperfused, and damaged cells lyse, releasing toxic neurotransmitters. The goal of therapeutic hypothermia is to intervene during the latent period and minimize damage from the secondary neuronal injury. Therapeutic hypothermia, when started within six hours of injury decreases mortality and severe disability from 62% to 48% and increases survival with the normal outcome from 24% to 40% with a number needed to treat of six to seven. Whole body cooling appears to be more effective at decreasing death than selective head cooling, but both modalities are effective at decreasing severe disability and a combined outcome of death and severe disability. Infants with moderate encephalopathy (Sarnat Stage 2) benefit most from therapeutic hypothermia. Importantly, cooling does not appear to decrease death at the cost of more severely neurologic impairment in survivors. Side effects associated with therapeutic hypothermia include peripheral vasoconstriction, diuresis, cardiac dysfunction, arrhythmias, coagulopathy, thrombocytopenia, leukocyte dysfunction, pulmonary hypertension and sclerema (calcium deposits in the skin). Therapeutic hypothermia can be delivered safely with specialized equipment and monitoring in sophisticated medical centers ⁷⁾.

The treatment of respiratory distress, pulmonary hypertension, coagulopathy and myocardial dysfunction is supportive. Infants with respiratory distress and pulmonary hypertension may require intubation, surfactant, oxygen and inhaled nitric oxide. Coagulopathy is treated with the prudent use of blood products to maintain oxygen-carrying capacity and coagulation. Myocardial dysfunction may result in a need for vasopressors. Renal dysfunction may result in oliguria or anuria; therefore, use of crystalloid fluid and blood products should be cautious.

Birth asphyxia prognosis

The long-term prognosis of birth asphyxia infants has been difficult to assess. In the short term, the condition is reported to have a mortality in excess of 30%, with the majority of deaths occurring within the first few days after birth. Those infants who survive are often left with mild to severe neurological deficits, and they also end up dying from aspiration or systemic infections. Long-term survivors have been found to have disabling cerebral palsy, inadequate mental development or low psychomotor scores, seizures, blindness, and severe hearing impairment. The management of these infants, in the long run, is complex and prohibitively expensive ⁸⁾.

Newborn assessment

Routine newborn assessment is a broad term referring to the assessment of the newborn occurring at various points in time within the first 6–8 weeks after birth. Newborn assessment includes the brief initial assessment, the full and detailed newborn assessment within 48 hours of birth and the follow-up assessments at 5–7 days and 6 weeks.

Each newborn baby is carefully checked at birth for signs of problems or complications. Your child’s healthcare provider will do a complete physical exam that includes every body system. Throughout the hospital stay, doctors, nurses, and other healthcare providers continually look at the health of your baby. They are watching for signs of problems or illness. Newborn assessments may include the below.

Routine newborn assessment:

• General appearance
  • Skin color, integrity, perfusion
  • State of alertness
  • Activity and range of spontaneous movement
  • Posture and, muscle tone
• Growth status
  • Chart head circumference, length and weight on centile charts
• Head, face, neck
  • Head shape, size
  • Scalp, fontanelles, sutures
  • Eye size, position structure
  • Nose, position, structure
  • Ear position, structure
  • Mouth, palate, teeth, gums tongue, frenulum
  • Jaw size
• Shoulders, arms, hands
  • Length, proportions, symmetry
  • Structure, number of digits
• Chest
  • Size, shape, symmetry, movement
  • Breast tissue, nipples
  • Heart sounds, rate, pulses. A newborn baby’s normal heart rate ranges from 90 to 140 beats per minute.
  • Breath sounds, respiratory rate.
  • Pulse oximetry
• Abdomen
  • Size, shape, symmetry
  • Palpate liver, spleen, kidneys
  • Umbilicus
• Genitourinary
  • Male – penis, foreskin, testes
  • Female – clitoris, labia, hymen
  • Anal position, patency
  • Passage of urine, stool
• Hips, legs, feet
  • Ortolani and Barlow’s manoeuvers
  • Leg length, proportions, symmetry and digits
• Back
  • Spinal column, skin
  • Symmetry of scapulae, buttocks
• Neurological
  • Behavior, posture
  • Muscle tone, spontaneous movements
  • Cry
  • Reflexes – Moro, Suck, Grasp

Measurements

The hospital staff takes other measurements of each baby. These include:

• Head circumference. The distance around the baby’s head.
• Abdominal circumference. The distance around the belly (abdomen).
• Length. The measurement from top of head to the heel.

The staff also checks these vital signs:

• Temperature. This checks that the baby is able to have a stable body temperature in normal room.
• Pulse. A newborn’s pulse is normally 120 to 160 beats per minute.
• Breathing rate. A newborn’s breathing rate is normally 40 to 60 breaths per minute.

Physical exam

A complete physical exam is an important part of newborn care. The healthcare provider carefully checks each body system for health and normal function. The provider also looks for any signs of illness or birth defects. Physical exam of a newborn often includes:

• General appearance. This looks at physical activity, muscle tone, posture, and level of consciousness.
• Skin. This looks at skin color, texture, nails, and any rashes.
• Head and neck. This looks at the shape of head, the soft spots (fontanelles) on the baby’s skull, and the bones across the upper chest (clavicles).
• Face. This looks at the eyes, ears, nose, and cheeks.
• Mouth. This looks at the roof of the mouth (palate), tongue, and throat.
• Lungs. This looks at the sounds the baby makes when he or she breathes. This also looks at the breathing pattern.
• Heart sounds and pulses in the groin (femoral)
• Abdomen. This looks for any masses or hernias.
• Genitals and anus. This checks that the baby has open passages for urine and stool.
• Arms and legs. This checks the baby’s movement and development.

Apgar scoring

The Apgar score helps find breathing problems and other health issues. It is part of the special attention given to a baby in the first few minutes after birth. The baby is checked at 1 minute and 5 minutes after birth for heart and respiratory rates, muscle tone, reflexes, and color. A baby who needs help with any of these issues is getting constant attention during those first 5 to 10 minutes. In this case, the actual Apgar score is given after the immediate issues have been taken care of.

Each area can have a score of 0, 1, or 2, with 10 points as the maximum. Most babies score 8 or 9, with 1 or 2 points taken off for blue hands and feet because of immature circulation. If a baby has a difficult time during delivery and needs extra help after birth, this will be shown in a lower Apgar score. Apgar scores of 6 or less usually mean a baby needed immediate attention and care.

Table 1. Apgar score

Birth weight

A baby’s birth weight is an important marker of health. Full-term babies are born between 37 and 41 weeks of pregnancy. The average weight for full-term babies is about 7 pounds (3.2 kg). In general, very small babies and very large babies are at greater risk for problems. Babies are weighed every day in the nursery to look at growth, and the baby’s need for fluids and nutrition. Newborn babies may often lose 5% to 7% of their birth weight. This means that a baby weighing 7 pounds 3 ounces at birth might lose as much as 8 ounces in the first few days. Babies will usually gain this weight back within the first 2 weeks after birth. Premature and sick babies may not begin to gain weight right away.

Most hospitals use the metric system for weighing babies. This chart will help you convert grams to pounds.

Converting grams to pounds and ounces:

1 lb. = 453.59237 grams; 1 oz. = 28.349523 grams; 1000 grams = 1 Kg.

Gestational assessment

The healthcare provider will check how mature the baby is. This is an important part of care. This check helps figure out the best care for the baby if the dates of a pregnancy are uncertain. For example, a very small baby may actually be more mature than he or she appears by size, and may need different care than a premature baby needs.

Healthcare providers often use an exam called the Dubowitz/Ballard Examination for Gestational Age. This exam can closely estimate a baby’s gestational age. The exam looks at a baby’s skin and other physical features, plus the baby’s movement and reflexes. The physical maturity part of the exam is done in the first 2 hours of birth. The movement and reflexes part of the exam is done within 24 hours after birth. The provider often uses the information from this exam to help with other maturity estimates.

Physical maturity

The physical maturity part of the Dubowitz/Ballard exam looks at physical features that look different at different stages of a baby’s gestational age. Babies who are physically mature usually have higher scores than premature babies.

Points are given for each area of assessment. A low of -1 or -2 means that the baby is very immature. A score of 4 or 5 means that the baby is very mature (postmature). These are the areas looked at:

• Skin textures. Is the skin sticky, smooth, or peeling?
• Soft, downy hair on the baby’s body (lanugo). This hair is not found on immature babies. It shows up on a mature infant, but goes away for a postmature infant.
• Plantar creases. These are creases on the soles of the feet. They can be absent or range up to covering the entire foot.
• Breast. The provider looks at the thickness and size of breast tissue and the darker ring around each nipple (areola).
• Eyes and ears. The provider checks to see if the eyes are fused or open. He or she also checks the amount of cartilage and stiffness of the ears.
• Genitals, male. The provider checks for the testes and how the scrotum looks. It may be smooth or wrinkled.
• Genitals, female. The provider checks the size of the clitoris and the labia and how they look.

Maturity of nerves and muscles

The healthcare provider does 6 checks of the baby’s nerves and muscles.

A score is given to each area looked at. Typically, the more mature the baby is, the higher the score. These are the areas checked:

• Posture. This looks at how the baby holds his or her arms and legs.
• Square window. This looks at how far the baby’s hands can be flexed toward the wrist.
• Arm recoil. This looks at how much the baby’s arms “spring back” to a flexed position.
• Popliteal angle. This looks at how far the baby’s knees extend.
• Scarf sign. This looks at how far the baby’s elbows can be moved across the baby’s chest.
• Heel to ear. This looks at how near the baby’s feet can be moved to the ears.

When the physical assessment score and the nerves and muscles score are added together, the healthcare provider can estimate the baby’s gestational age. Scores range from very low for immature babies to very high scores for mature and postmature babies.

All of these exams are important ways to learn about your baby’s well-being at birth. By finding any problems, your baby’s doctor can plan the best possible care.

Children anger management

Anger is a normal human emotion. Kids feel angry sometimes and in varying degrees. Sometimes a strong emotion like anger can be a powerful tool for change, it can give you the energy to get something done or to stand up for what you believe in. Feeling angry and managing your anger in positive and healthy ways can also give you the chance to set a good example for your children. For example, you might be feeling angry that the kitchen is a mess. Instead of using that anger in a way that might be destructive, you can use that anger to create change. Maybe you start a dishwasher clearing roster. Or maybe there’s a rule that everyone needs to tidy up after themselves. This can be an excellent opportunity for your child to see how you use an emotion like anger to initiate a healthy discussion. But anger can be negative, especially if it happens a lot or it gets out of control. The issue with anger is learning to recognize it, how to use it in a productive way, and manage it effectively.

Many children have difficulty regulating their emotions. Tantrums, outbursts, whining, defiance, fighting: these are all behaviors you see when kids experience powerful feelings they can’t control. While some kids have learned to act out because it gets them what they want — attention or time playing video games — other kids have trouble staying calm because they are unusually sensitive.

Tantrums are very common in children aged 1-3 years. This is because children’s social and emotional skills are only just starting to develop at this age. Children often don’t have the words to express big emotions. They might be testing out their growing independence. And they’re discovering that the way they behave can influence the way other people behave. So tantrums are one of the ways that young children express and manage feelings, and try to understand or change what’s going on around them.

Older children can have tantrums too. This can be because they haven’t yet learned more appropriate ways to express or manage feelings.

When kids are overwhelmed by strong feelings, the emotional side of their brain isn’t communicating with the rational side, which normally regulates emotions and plans the best way to deal with a situation. Experts call it being “dysregulation”. It’s not effective to try to reason with a child who’s “dysregulated”. To discuss what happened, you need to wait until a child’s rational faculties are back “online.”

For both toddlers and older children, there are things that can make tantrums more likely to happen:

• Temperament – this influences how quickly and strongly children react to things like frustrating events. Children who get upset easily might be more likely to have tantrums.
• Stress, hunger, tiredness and overstimulation – these can make it harder for children to express and manage feelings and behavior.
• Situations that children just can’t cope with – for example, a toddler might have trouble coping if an older child takes a toy away.
• Strong emotions – worry, fear, shame and anger can be overwhelming for children.

The first step is to get a picture of what triggers your particular child’s anger. Mental health professionals call this a “functional assessment,” which means looking at what real-life situations seem to generate anger issues — specifically, at what happens immediately before, during, and after the outbursts that might contribute to their happening again.

Sometimes a close look at the pattern of a child’s anger issues reveals a problem that needs attention: a traumatic experience, abuse or neglect, social anxiety, attention deficit hyperactivity disorder (ADHD), or a learning disorder. When children are prone to meltdowns beyond the age in which they are typical, it’s often a symptom of distress that they are struggling to manage. That effort breaks down at moments that require self-discipline they don’t yet have, like transitioning from something they enjoy to something that’s difficult for them.

A majority of kids who have frequent meltdowns do it in very predictable, circumscribed situations: when it’s homework time, bedtime, time to stop playing. The trigger is usually being asked to do something that’s aversive to them or to stop doing something that is fun for them. Especially for children who have ADHD, something that’s not stimulating and requires them to control their physical activity, like a long car ride or a religious service or visiting an elderly relative, is a common trigger for meltdowns.

When children continue to have regular emotional outbursts, it’s usually a symptom of distress. The first step is understanding what’s triggering your child’s behavior. There are many possible underlying causes, including:

• ADHD: Many children with ADHD, especially those who experience impulsivity and hyperactivity, have trouble controlling their behavior. They may find it very hard to comply with instructions or switch from one activity to another, and that makes them appear defiant and angry. Their inability to focus and complete tasks can also lead to tantrums, arguing, and power struggles. In a recent study conducted by Dr. Amy Roy of Fordham University, more than 75 percent of children who presented with severe temper outbursts also fit the criteria for ADHD. That doesn’t necessarily mean they’ve been diagnosed with ADHD—in fact ADHD may be overlooked in kids who have a history of aggression. What people don’t understand is that a lack of focus, an inability to complete work and tolerate boredom, among other symptoms, can contribute to the escalation toward the explosive outbursts. In fact, ADHD is sometimes overlooked in kids who have a history of severe aggression because there are so many bigger issues.
• Anxiety: Anxiety is another major contributor. Even if kids don’t have a full-blown anxiety disorder, they may still be overreactive to anxiety-provoking situations and melt down when they are stressed. Children who seem angry and defiant often have severe, and unrecognized, anxiety. If your child has anxiety, especially if she’s hiding it, she may have a hard time coping with situations that cause her distress, and she may lash out when the demands at school, for instance, put pressure on her that she can’t handle. In an anxiety-inducing situation, your child’s “fight or flight” instinct may take hold—she may have a tantrum or refuse to do something to avoid the source of acute fear.
• Learning problems: Kids who have undiagnosed learning disabilities or who have suffered trauma or neglect may react this way when confronted with an uncomfortable or painful situation. When your child acts out repeatedly in school or during homework time, it’s possible that he has an undiagnosed learning disorder. Say he has a lot of trouble with math, and math problems make him very frustrated and irritable. Rather than ask for help, he may rip up an assignment or start something with another child to create a diversion from his real issues.
• Trauma or neglect: A lot of acting out in school is the result of trauma, neglect, or chaos at home. Kids who are struggling, not feeling safe at home can act like terrorists at school, with fairly intimidating kinds of behavior. Most at risk, she says, are kids with ADHD who’ve also experienced trauma.
• Depression and irritability: Depression and irritability also occur in a subset of kids who have severe and frequent temper tantrums. A new disorder called disruptive mood dysregulation disorder, describes kids who have severe outbursts with chronic severe irritability in between. Kids who are highly irritable are like water at 90 degrees—always on the cusp of boiling. Parents of these kids are always walking on eggshells because they respond to very subtle things, like the slightest thing not going their way.
• Autism (autism spectrum disorder): Children on the autism spectrum are also often prone to dramatic meltdowns. If your child is on the autism spectrum, he may tend to be rigid—needing consistent routine to feel safe for their emotional comfort and any unexpected change can set him off. He may have sensory issues that cause him to be overwhelmed by stimulation, and short-circuit into a meltdown that continues until he exhausts himself. And he may lack the language and communication skills to express what he wants or needs.
• Sensory processing issues: Some children have trouble processing the sensory information they are getting from the world around them. If your child is oversensitive or undersensitive, to stimulation, things like “scratchy” clothes and too much light or noise can make her uncomfortable, anxious, distracted, or overwhelmed. That can lead to meltdowns for no reason that’s apparent to you or other caregivers. Sensory processing challenges, often seen in autistic children and teens as well as many with ADHD, may cause kids to be overwhelmed by stimulation, and short-circuit in inconsolable meltdowns.

Whatever the trigger, most mental health professionals believe that children who have frequent emotional outbursts are lacking certain skills that would help them better handle situations that cause them frustration, anxiety or anger. They include:

• Impulse control
• Problem solving
• Delaying gratification
• Negotiating
• Communicating wishes and needs to adults
• Knowing what’s appropriate or expected in a given situation
• Self-soothing

The first step to managing anger in children is getting your child to recognize they feel anger. Some ways to help teach your child about anger include:

• Playing games: Something like “Guessing Faces” is a useful way to help your child recognize feelings. For example, you can pull a face and you ask your child to tell you how you might be feeling. You can also ask your child to pull faces, for example by asking them, “show me a happy face” or “show me an angry face”. Then ask them to tell a story about why someone might have that feeling. This works by drawing faces too.
• Reading stories about emotions: There are many children’s books around that tell stories about feelings. These stories can be a great way to open up a dialogue about challenging emotions and offer an opportunity to discuss what you can do when you feel these emotions.
• Encouraging discussion about feelings: Letting children know that feelings are normal and it is important and encouraging them to talk about the pleasant and not-so-pleasant feelings they might have can help them deal with difficult emotions.
• Being open about your own feelings: Talking about your feelings and why you’re feeling a particular emotion is a great way to model healthy behavior, in particular showing how to manage difficult emotions. For example, “I am feeling angry because the kitchen is a mess.”

Parents can start by helping children understand how their emotions work. Kids don’t go from calm to sobbing on the floor in an instant. That emotion built over time, like a wave. Kids can learn control by noticing and labeling their feelings earlier, before the wave gets too big to handle.

Coping with anger issues

Dealing with anger issues can be very draining and stressful. You might feel you need to step in to end a tantrum straight away. But if it’s safe, it can help to take a breather while you decide how to respond.

Here are ideas for staying calm and keeping things in perspective:

• Develop a strategy for anger issues. Have a clear plan for how you’ll handle a tantrum in whatever situation you’re in. Concentrate on putting your plan into action when the tantrum happens.
• Accept that you can’t control your child’s emotions or behavior directly. You can only keep your child safe and guide their behavior so anger issues are less likely to happen in the future.
• Accept that it takes time for change to happen. Your child has a lot of growing up to do before anger issues are gone forever. Developing and practising self-regulation skills is a life-long task.
• Beware of thinking that your child is doing it on purpose or is trying to upset you. Children don’t have anger issues deliberately – they’re stuck in a bad habit or just don’t have the skills right now to cope with the situation.
• Keep your sense of humor. But don’t laugh at the tantrum – if you do, it might reward your child with attention. It might also upset your child even more if they think you’re laughing at them.
• If other people give you dirty looks, ignore them. They’ve either never had children or it’s been so long since they had a young child that they’ve forgotten what it’s like.

Don’t judge yourself as a parent based on how many anger issues your child has. Remember that all children have anger issues. Instead, focus on how you respond to the anger issues. And remember that you’re only human and part of parenting is learning as you go.

Child anger management therapy

Medication won’t necessarily fix defiant behavior or aggression; it can reduce the symptoms of ADHD, anxiety, and other disorders and improve the conditions for working on those behaviors. Behavioral approaches that have parents and children working together to rein in problem behavior are key to helping the situation.

Find the triggers

The first step in managing anger is understanding what triggers set off a child’s outbursts. So, for instance, if getting out the door for school is a chronic issue for your child, solutions might include time warnings, laying out clothes and showering the night before, and waking up earlier. Some kids respond well to breaking tasks down into steps, and posting them on the wall.

Consistent parenting

When a child’s defiance and emotional outbursts occur, the parent or caregiver’s response affects the likelihood of the behavior happening again.

If a child’s behavior is out of control or causing major problems, it’s a good idea to try step-by-step parent training programs. These programs like the Parent-Child Interaction Therapy (PCIT) and the Parent Management Training (PMT) train you to positively reinforce behavior you want to encourage in your child, and give consistent consequences for behaviors you want to discourage. Most children respond well to a more structured relationship, with calm, consistent responses from parents that they can count on.

What all the parent training programs have in common is that they teach parents how to use praise, or positive reinforcement, more effectively, to encourage the behaviors they want to encourage. And they teach parents how to deploy consistent consequences when kids don’t comply. The result is that kids learn to modulate their behavior to meet expectations and enjoy much more positive interactions with their parents.

Where the parent training programs differ is in how instruction is delivered, how parents practice the skills they’re learning, and the pace at which they’re expected to master these new skills. The programs involve 10 or more sessions, and they target different age groups of children.

Here are some of the key elements taught in parent training:

• Don’t give in. Resist the temptation to end your child’s tantrum by giving her what she wants when she explodes. To give in only teaches her that tantrums work.
• Remain calm and consistent. You’re in a better place to teach and follow through with better, more consistent consequences when you’re in control of your own emotions. Harsh or angry responses tend to escalate a child’s aggression, be it verbal or physical. By staying calm, you’re also modeling—and teaching—your child the type of behavior you want to see in him.
• Ignore negative behavior and praise positive behavior. Ignore minor misbehavior, since even negative attention like reprimanding or telling the child to stop can reinforce her actions. Instead, lavish labeled praise on behaviors you want to encourage. (Don’t just say “good job,” say “good job calming down.”)
• Use consistent consequences. Your child needs to know what the consequences are for negative behaviors, such as time outs, as well as rewards for positive behaviors, like time on the iPad. And you need to show him you follow through with these consequences every time.
• Wait to talk until the meltdown is over. One thing you don’t want to do is try to reason with a child who is upset. Don’t talk to the kid when she’s not available. You want to encourage a child to practice at negotiation when she’s not blowing up, and you’re not either.
• Build a toolkit for calming down. Both you and your child need to build a toolkit for self-soothing, things you can do to calm down, like slow breathing, to relax, because you can’t be calm and angry at the same time. There are lots of techniques, but the nice thing about breathing is it’s always available to you.

Parent-Child Interaction Therapy

Parent-Child Interaction Therapy (PCIT) works with parents and children together, teaching them skills to interact in a positive, productive way. It is effective for kids between the ages of 2 and 7, and usually requires 14 to 17 weekly sessions.

In Parent-Child Interaction Therapy (PCIT), parents receive live coaching (via a bug in the ear) from a therapist who watches from behind a one-way mirror as they and their child perform a series of tasks, and parents practice specific responses to both desired and undesired behavior.

Parent-Child Interaction Therapy (PCIT) is the most practice-intensive, as parents demonstrate mastery of each skill before going on to the next one. It starts out with positive interactions, then waits till parents reach mastery of these skills before moving on to discipline strategies to improve oppositional behavior.

Parent-Child Interaction Therapy (PCIT) is usually recommended if parents need a lot of one-on-one attention in terms of how they’re interacting with their child and the child is under 7 and especially if it there have been a lot of coercive negative interactions. Sometimes it’s very important for parents to learn how to be with their kid in a positive way.

Parent Management Training

In Parent Management Training (PMT), which is for children ages 3 to 13, parents are usually seen without the child present, although children may be asked to participate in some sessions. Skills to deal more effectively with challenging behaviors are taught and modeled by the therapist and then role-played with parents. After each session, parents are expected to practice the skills at home. Families usually participate in at least 10 sessions.

Parent Management Training (PMT) is great because there’s a new skill that’s taught at every module, so if the family really goes home and implements what was talked about in session, the therapist could teach the family all the important skills that they need to know within four or five sessions. From that point on it’s kind of tinkering, refining and tightening up everything.

Parent Management Training (PMT) is usually recommended if there’s a premium on “more change more quickly.” This can be because the situation seems particularly urgent, such as really severe tantrums or aggressive behaviors.

Since Parent Management Training (PMT) is appropriate for all ages, it’s a good choice when kids are too old for Parent-Child Interaction Therapy (PCIT). But Parent Management Training (PMT) may also be recommended for a 4-year-old if the therapist feels that the parent-child interaction is actually pretty good, but there might be other things going on, such as anxiety, extreme impulsiveness or explosive anger.

However, it’s important to note that Parent Management Training (PMT), which establishes a token economy with point charts used to reinforce positive behaviors, can be tougher for younger kids to grasp, especially if they’re cognitively delayed. It’s sometimes hard for them to understand that they’re earning points that they can then spend on things. But if a kid is able to make the connection that they’re earning these stars for behavior and that these stars get them a reward, then it’s appropriate.

Positive Parenting Program (Triple P)

Positive Parenting Program (Triple P) focus is on equipping parents with information and skills to increase confidence and self-sufficiency in managing child behavior. It can be utilized with a wide age range of children from toddlerhood through adolescence.

There are different levels of intervention depending on how severe the case is, with more of a whole blanket system of improving parenting on many different levels.

First, for parenting basics, there’s a four-session treatment that can be given in doctor’s offices that include social workers or other mental health professionals for integrated care.

But for families with more severe behavior problems, there’s a 12-session parent-and-child-focused treatment. It incorporates the best of Parent Management Training (PMT) and Parent-Child Interaction Therapy (PCIT) by having some sessions in which the clinician is meeting one-on-one with parents and talking about skills and strategies, and some sessions where kids are included and the therapist can do live coaching.

The Incredible Years Program

The Incredible Years program offers small-group-based training for parents of kids from infants through age 12. The Incredible Years programs are broken into four age groups (baby, toddler, preschool and school age) and they range from 12 to 20 weeks.

There is also specialized training designed for high-risk socioeconomically disadvantaged families, and for families with children diagnosed with ADHD and oppositional defiance disorder (ODD).

The Incredible Years is in between Parent Management Training (PMT), where they’re giving you everything up front, and Parent-Child Interaction Therapy (PCIT), where skills are added only after earlier ones have been mastered.

There’s a different topic for parents each session. The Incredible Years program starts with a focus on improving parent-child relationships and positive attachment before moving on to consistent routines, rules, and limit-setting. Finally it covers child management strategies such as ignoring, redirection, logical and natural consequences, time to calm down and problem-solving.

For children from four to eight years old, Incredible Years offers children’s groups that focus on helping them acquire emotion regulation strategies and social skills.

Research shows that the kids’ group works well at improving pro-social behavior and decreasing disruptive behaviors, but when you add the parenting part it really boosts the effectiveness of it.

One of the great things for parents is that they learn not only from therapists but from each other. Parents hear what others tried. It also normalizes what they’re seeing in their kids.

Behavioral and Emotional Skills Training

Designed to introduce effective behavior management techniques in a single session, Behavioral and Emotional Skills Training (BEST) is a full-day, interactive training for parents and caregivers to help manage problem behaviors in the home. Attendees of Behavioral and Emotional Skills Training (BEST) workshops learn the basics of behavior management and how to apply those skills consistently and effectively to increase their confidence in managing challenging behaviors. They also develop a behaviorally-minded network of other caregivers for continued support following training completion.

How to handle toddler temper tantrums when they happen

Sometimes tantrums happen, no matter what you do to avoid them. Here are some ideas for handling tantrums when they happen:

• Stay calm (or pretend to!). Take a moment for yourself if you need to. If you get angry, it’ll make the situation harder for both you and your child. When you speak, keep your voice calm and level, and act deliberately and slowly.
• Acknowledge your child’s strong feelings. For example, ‘It’s very upsetting when your ice-cream falls out of the cone, isn’t it?’ This can help prevent behavior getting more out of control and gives your child a chance to reset emotions.
• Wait out the tantrum. Stay close so your child knows you’re there. But don’t try to reason with your child or distract them. It’s too late once a tantrum has started.
• Take charge when you need to. If the tantrum happens because your child wants something, don’t give your child what they want. If your child doesn’t want to do something, use your judgment. For example, if your child doesn’t want to get out of the bath, pulling out the plug might be safer than lifting out your child.
• Be consistent and calm in your approach. If you sometimes give your child what they want when they have tantrums and you sometimes don’t, the problem could get worse.

Learning to manage anger

While it’s great to think of using emotion, like anger, in a healthy way, what this means is that you need to first manage those sometimes intense feelings. For kids, this can be especially challenging, as they often don’t always have the language required to express themselves.

The first thing you have to do to manage anger issues is to understand them. That is not always as easy as it sounds, since anger and meltdowns are generated by a lot of different things: fear, frustration, sensory overload, to name a few. And since a tantrum isn’t a very clear way to communicate (even though it may be a powerful way to get attention), parents are often in the dark about what’s driving the behavior.

Some ways you can help, as parents and carers, is to teach them to manage their emotions. For example:

• Lead by example: Showing how you manage and express your emotions will help to teach your child how they can do the same. For example, if you’re stuck in traffic, you might say, “let’s put on some music to help me calm down and feel better.”
• Remain calm: When your child is angry, it will be more difficult to diffuse the situation if you meet his or her anger with more anger. If anything, this teaches them that it is ok to yell and raise your voice when you feel angry. Instead, try and stay calm, ask clarifying questions and offer solutions.
• Breathing exercises: Ask your child to put one hand on their chest and one on their belly. Ask them to take a deep breathe, all the way into their belly and to let the air out, slowly. This can quickly take the charge out of a situation and helps to get them into their body.
• Physical activity: Sometimes a muscle relaxation exercise can be the perfect antidote to a stressful situation. But other times, getting your child to spend some of that excess energy in a non-destructive way can be extremely useful. For example, suggest you both go for a walk around the block. Or encourage them to jump on the trampoline. These kinds of physical activities can help to calm them down.
• Other things that might help:
  • Listening to music
  • Reading a story
  • Drawing or coloring in

Teaching children new ways to deal with anger isn’t easy and usually takes time, a lot of repetition and a lot of healthy modelling. So stick with it, maybe pick one thing to work on during the month. Be patient and if things still aren’t improving get in touch with the Growing Early Minds team to see how we can help.

Accepting emotions

If you can show you accept your child’s emotions and even empathize with them, for example, “I can understand why you might be angry that it’s time to stop playing video games and start your homework,” it can help teach them that emotions are OK and it’s safe to feel them, without necessarily acting on them. Accepting their emotions, while remaining calm, can open up a dialogue and presents an opportunity for expressing that emotion without being destructive.

Delving under the anger

When you stay calm and open up a dialogue with your child, you begin to make it safe for them to be vulnerable. This usually helps you to get under the anger, which is often something like sadness or disappointment. This also helps children grow more awareness around their feelings and develop emotional intelligence.

How to make toddler anger issues less likely

There are things you can do to make tantrums less likely to happen:

• Reduce stress. Tired, hungry and overstimulated children are more likely to experience tantrums.
• Tune in to your child’s feelings. If you’re aware of your child’s feelings, you might be able to sense when big feelings are on the way. You can talk about what’s going on and help your child manage difficult feelings. You might also be able to distract your child.
• Identify tantrum triggers. For example, your child might have tantrums when you’re shopping. You might be able to plan for this situation or change the environment to avoid tantrums. For example, it might help to go shopping after your child has had a nap and a snack.
• Talk about emotions with your child. When your child struggles with a strong feeling, encourage your child to name the feeling and what caused it. For example, ‘Did you throw your toy because you were cross that it wasn’t working? What else could you have done?’

Macrodactyly

Macrodactyly is a rare congenital condition in which a baby is born with abnormally large fingers or toes due to an overgrowth of the underlying bones and soft tissue ¹⁾. The abnormality develops in one or more toes and involves thickening of both soft tissue and bone of the affected digit. (Figures 1 and 2). Macrodactyly is further characterized by increased size of all elements – tendons, nerves, vessels, subcutaneous fat, nails, skin and phalanges – all but the metatarsals. The increase of nerve size is
noticeable and the digit appears to be infiltrated with fatty tissue ²⁾. In macrodactyly toe, accumulation of fat appears to be the most striking feature ³⁾ and histopathological findings suggest that excessive proliferation of fatty tissue is the basis of cause for this abnormality whereas macrodactyly of the hand involves hypertrophied and tortuous digital nerves ⁴⁾.

Macrodactyly occurs more often in hands than the feet. One or more fingers or toes may be involved. Macrodactyly can occur on one side only, or may affect both hands or both feet. Often macrodactyly is equated with its most common cause, macrodystrophia lipomatosa ⁵⁾. Rarely macrodactyly can be seen as a form of Proteus syndrome ⁶⁾.

Macrodactyly can be divided into two types – static and progressive macrodactyly.

1. Static macrodactyly is present at birth and the digit appears to have increased in size and grows at the same pace as unaffected fingers or toes.
2. Progressive macrodactyly is characterized by disproportionate growth – the affected fingers or toes grow at a much faster rate than unaffected digits ⁷⁾. The affected fingers or toes can become enormous and severely impact a child’s ability to perform normal activities.

In some cases, a child’s macrodactyly-affected limb may appear to have a static growth pattern during infancy, but then can rapidly progress when the child is about 2 years old. In these cases, the affected fingers or toes grow very quickly, and often involve abnormally growth in the adjacent palm or forefoot. Macrodactyly foot tends to be of the progressive type. Most reported cases indicate a slight male preponderance ⁸⁾ and the condition can occur unilaterally, bilaterally, symmetrically, or asymmetrically ⁹⁾.

Children with macrodactyly may also have other musculoskeletal differences. One study showed that macrodactyly may be associated with syndactyly (two or more digits are fused together or webbed or conjoined fingers or toes) of the adjacent digit in as much as 10% of cases reported ¹⁰⁾.

A number of conditions may manifest with asymmetric limb hypertrophy, including:

• Neural fibrolipoma (some term any macrodactyly with a neural fibrolipoma as macrodystrophia lipomatosa) ¹¹⁾
• Angiomatosis
• Neurofibromatosis type 1

Though macrodactyly is a benign condition, it does cause deformities, appear cosmetically different, and may affect your child’s normal hand or foot function.

Treatment for macrodactyly is on a case by case basis but generally includes surgery and the major aim is the reconstruction of a pain-free functioning foot ¹²⁾. The growth of affected digit in children may be retarded by destroying, stapling, or wiring the epiphyseal plate ¹³⁾. The accepted treatment for foot macrodactyly is reduction of the fibro-fatty bulk via dissection and ablation combined with removal of as much fat as possible ¹⁴⁾. De-fatting occurs in a two step procedure where the first stepinvolves only the convex side of the digit and reducing its thickness by 10% to 20% while taking care to preserve its vascularity. The second procedure involves de-fatting the remaining side and performing a phalangectomy – shortening of the bone and resectioning and suturing of the excess skin. However there are new one-step procedures which greatly reduce compromising vascularity. Amputationis also an option, though it is reserved as a last option in most cases ¹⁵⁾. Complications with surgery include delayed wound healing and inadequate initial de-fatting could cause subsequent re-growth of the fibro-fatty tissue and may require a second and more proximal amputation ¹⁶⁾.

Figure 1. Macrodactyly finger

Figure 2. Macrodactyly toe

Macrodactyly causes

The cause of macrodactyly remains unknown. Researchers believe the condition is not inherited, but may instead be linked to disruptions in prenatal development. Streeter ¹⁷⁾ hypothesizes that the condition might be a “consequence of germ plasm abnormality or pathology; by accident during cell cleavage or organogenisis, certain areas become supersensitive to growth stimulus.” During fetal development, some disturbance of growth limiting factor occurs in areas affected and because of this lack of inhibitor the part affected continues to increase in size and accounts for progressive overgrowth in later years. Barsky ¹⁸⁾ also states that the “condition might be caused by interaction of genetic and environmental factors and may be the result of interaction of two or more extrinsic agents each incapable of producing teratological effects by themselves.”

Enlargements may also be due to hemangiomas where soft tissues, skin and x-rays do not show any increased size of the digits affected ¹⁹⁾. Some propose that the development of neuorfibromatomas in the periosteal nerves due to bony destruction and regeneration may be the cause of rapid localized overgrowth ²⁰⁾.

Macrodactyly can occur in conjunction with other conditions and syndromes, including neurofibromatosis, lipofibromatous hamartoma, vascular conditions, and tuberous sclerosis complex.

The fatty tissues in foot macrodactyly found in children resemble adult subcutaneous fat – dark lobules fixed by many fine vessels which traverse the tissue – rather than the fat of the normal child’s age ²¹⁾. Most of the abnormal bulk is due to excessive fibrofatty tissues and is abundant on the sides and plantar aspects of the toe. The fibro-fatty tissue extends from the toe in the forefoot and causes the bulk to expand laterally. The skin is markedly thickened and the digits involved have a soft and rubbery consistency ²²⁾. The bone age, as denoted by epiphyseal centers in the phalanges of affected digits, is increased when compared to unaffected bone ²³⁾. There is also an increased length and breadth of phalanges ²⁴⁾ with proliferation of fibroblastic tissue between the cortex and periosteum and accounted for cortical thickening and gigantism of phalanges in affected digits ²⁵⁾.

As previously stated, macrodactyly may be related to several neurogenic disorders. Abnormalities of peripheral nerves are uncommon in the pedal form though there are nerves more prominent with much proliferation of epineural and perineural tissues ²⁶⁾.

Macrodactyly signs and symptoms

The main symptom of macrodactyly is the appearance of one or more abnormally large fingers or toes. The larger digits may grow at the same rate as other non-affected fingers/toes, or have much faster growth. Macrodactyly may also result in reduced function owing to the secondary degenerative joint disorders and may cause compressions of adjacent nerves and vessels as well as disfigurement ²⁷⁾.

Other symptoms of macrodactyly may include:

• Abnormal growth in the affected hand or foot
• Stiffness
• Difficulty moving, grasping, or using the affected hand/foot
• Swelling
• Pain (though this is rare).

Macrodactyly diagnosis

Most children are born with obvious signs of macrodactyly. Occasionally, this condition presents a bit later, when the child is a year or two old. Diagnosing macrodactyly typically begins with a physical examination of your child’s affected hand or foot. A referral to the genetics team is sometimes necessary.

In most cases, clinicians will recommend an X-ray to confirm the diagnosis. X-rays produce images of bones and help doctors identify the underlying structure of the hand or foot.

In addition to a physical exam and X-rays, your child may also undergo:

• Magnetic resonance imaging (MRI), which uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs, soft tissues, muscles, ligaments and other structures within the body. This test can help determine which underlying tissue is enlarged. Your child is exposed to no radiation during an MRI.
• Range of motion tests to determine if the condition is affecting movement and dexterity
  Nerve assessment tests to determine if the condition has damaged or compressed any nerves

Accurate diagnosis helps your doctor determine the best course of treatment for your child.

Macrodactyly treatment

Treatment for macrodactyly will depend on the severity of your child’s condition, as well as the age and development of your child. Your child’s physician will discuss specific treatment options with you, and treatment depends on whether the condition occurs in the hand or the foot. Most of the time, however, surgery is required to correct macrodactyly. Your child’s doctor will discuss whether staged surgical treatment could benefit your child.

Your doctor’s goal will be to give your child optimal function of the affected area.

A hand can still be quite functional with a finger or two larger than the rest. Even a slightly enlarged toe may make wearing shoes impossible.

In mild cases, treatment for macrodactyly may involve observation or, in the case of an enlarged foot, shoe modification alone.

Nonsurgical interventions

If the growth of your child’s affected fingers or toes is static — meaning it is not getting worse — and is not interfering with their mobility, clinicians may recommend ongoing observation to ensure the condition doesn’t worsen.

Macrodactyly surgery

Because macrodactyly often produces an obvious deformity and can progressively worsen, many families seek more active treatment. For the bulk of children with macrodactyly, this includes surgery. Surgery for macrodactyly is complex because it involves multiple layers of tissue. It may take several surgical procedures to achieve the goals of halting the abnormal growth and creating a close-to-normal looking hand or foot.

Size-altering surgery will involve extensive observation and planning, because doctors will want to plot the rate of growth of your child’s normal digits versus the enlarged digits.

Surgery can involve debulking extra skin and subcutaneous tissue to narrow the width of the affected digits.

Epiphysiodesis could also be required when the hand bones reach adult length. In this procedure, the growth plates of individual hand bones are closed in order to prevent continued increase in length. In the most severe progressive cases, partial hand amputation may be required if all other means to control the growth of the finger(s) have been exhausted.

Your child’s doctor may recommend some combination of the following surgical procedures:

• Soft tissue debulking: To help correct width, this procedure involves the surgical removal of the thickened layers of skin and fat and the replacement of skin with skin grafts harvested from healthy skin in a nearby area. This procedure is usually performed in several stages around three months apart. It’s more often used to treat the milder forms of macrodactyly or as a part of the treatment of the more progressive forms.
• Shortening procedures: To help correct length, shortening procedures usually involve either surgical removal of one of the phalanges of the finger or toe, or removal of a metacarpal (hand bone) or metatarsal (foot bone).
• Ray resection: Surgical removal of the entire digit or digits is sometimes necessary, particularly when the condition is progressive. It is also an option if there is excessive widening of the forefoot, where the digital shortening and debulking procedures may not be effective.

After surgery, your child’s affected hand or foot will be immobilized with a cast or splint.

Surgery can dramatically improve the long-term outcomes for your child with macrodactyly, but it can also be a stressful experience for you and your child.

Follow-up care

Follow-up care for macrodactyly will depend on the treatment needed. If your child received nonsurgical treatment, they should be monitored regularly to ensure the condition does not begin to significantly affect their daily activities.

If your child needs surgery, they will be examined a few times in the first 2 to 3 months after surgery, and then every 6 to 12 months to monitor function and continued growth.

Additional operations will be performed as needed. Your child’s doctor will give you specific information about a recovery program for your child and how soon they can return to daily activities.

Macrodactyly prognosis

The long-term outlook for a child treated for macrodactyly varies from child to child, depending on how severe his problem is. You and your child can expect an overall improvement in appearance and function. In most cases, surgery can control hand growth, improve hand function, and help children better manage their daily activities. However, it’s rare that a child’s affected digits look and move perfectly.

ABO incompatibility

ABO incompatibility is the most common cause of hemolytic disease of the newborn (HDN) which occurs in 15 to 25% of pregnancies ¹⁾. ABO incompatibility happens when a mother’s blood type is O becomes pregnant with a fetus with a different blood type (type A, B, or AB) ²⁾. The mother’s immune system may react and make antibodies against her baby’s red blood cells.

Blood is made up of red blood cells, white blood cells and platelets in a liquid called plasma. Your blood group is identified by antibodies and antigens in the blood.

Antibodies are proteins found in plasma. They’re part of your body’s natural defences. They recognize foreign substances, such as germs, and alert your immune system, which destroys them.

Antigens are protein molecules found on the surface of red blood cells.

The type is O mother’s serum contains naturally occurring anti-A and anti-B, which tend to be of the immunoglobulin G (IgG) class and can therefore cross the placenta and hemolyse fetal red blood cells (breakdown of red blood cells). Hemolysis associated with ABO incompatibility exclusively occurs in type-O mothers with fetuses who have type A or type B blood, although it has rarely been documented in type-A mothers with type-B infants with a high titer of anti-B immunoglobulin G (IgG).

In mothers with blood type A or type B, naturally occurring antibodies are of the immunoglobulin M (IgM) class and do not cross the placenta, whereas 1% of type-O mothers have a high titer of the antibodies of immunoglobulin G (IgG) class against both A and B. They cross the placenta and cause hemolysis in fetus.

The direct antiglobulin test (direct Coombs test) determines whether your red blood cells (RBCs) circulating in the bloodstream are covered with antibodies. The antibodies that are attached to the surface of the red blood cells are responsible for their destruction.

Hemolysis due to anti‐A is more common (1 in 150 births)than hemolysis due to anti-B. Affected newborns will usually be direct anti‐globulin test (DAT) positive, but, in contrast with the clinical picture with anti‐Rh antibodies, both anti‐A and anti‐B hemolytic disease of the newborn usually result predominantly in hyperbilirubinemia without significant neonatal anemia. This is mainly because of the relatively few group A or B antigenic sites on neonatal red blood cells, allowing the antibody‐coated cells to remain in the circulation for a longer period than in Rh D disease ³⁾. As a reflection of this, the blood film in ABO haemolytic disease characteristically shows very large numbers of spherocytes, with little or no increase in nucleated red blood cells, whereas in Rh D hemolytic disease of the newborn there are few spherocytes and large numbers of circulating nucleated red blood cells.

However, hemolysis due to anti-B IgG can be severe and can lead to exchange transfusion. Because A and B antigens are widely expressed in various tissues besides red blood cells, only a small portion of antibodies crossing the placenta are available to bind to fetal red blood cells. Recent analysis of IgG subclass in ABO incompatible direct Coombs positive neonates showed IgG2 was predominent antibody which is poorly transferred across placenta and less efficient in causing hemolysis while IgG1 was noted in 22% of neonates and as a group had similar rate of hemolysis and severity of hyperbilirubinemia ⁴⁾.

In addition, fetal RBCs appear to have less surface expression of A or B antigen, resulting in few reactive sites; hence the low incidence of significant hemolysis in affected neonates. This results in hyperbilirubinemia as a predominant manifestation of incompatibility (rather than anemia), and peripheral blood film frequently reveals a large number of spherocytes and few erythroblasts, unlike what is seen in Rh incompatibility (erythroblastosis fetalis), in which blood film reveals a large number of nucleated RBCs and few spherocytes ⁵⁾.

Hemolytic disease of the newborn due to ABO incompatibility is usually less severe than Rh incompatibility (Rhesus disease). One reason is that fetal red blood cells express less of the ABO blood group antigens compared with adult levels. In addition, in contrast to the Rh antigens, the ABO blood group antigens are expressed by a variety of fetal (and adult) tissues, reducing the chances of anti-A and anti-B binding their target antigens on the fetal red blood cells.

ABO incompatibility consequences and treatment are similar to Rhesus disease or Rh incompatibility.

Infants with hemolytic disease of the newborn may be treated with:

• Feeding often and receiving extra fluids.
• Light therapy (phototherapy) using special blue lights to convert bilirubin into a form which is easier for the baby’s body to get rid of.
• Antibodies (intravenous immunoglobulin, or IVIG) to help protect the baby’s red cells from being destroyed.
• Medicines to raise blood pressure if it drops too low.
• In severe cases, an exchange transfusion may need to be performed. This involves removing a large amount of the baby’s blood, and thus the extra bilirubin and antibodies. Fresh donor blood is infused.
• Simple transfusion (without exchange). This may need to be repeated after the baby goes home from the hospital.

Management of ABO hemolytic disease of the newborn is usually successful with phototherapy alone provided by modern equipment. However, close monitoring of the affected neonate is essential, and exchange transfusion is occasionally required. This is particularly the case in ABO hemolytic disease of the newborn due to anti‐B IgG where racial differences in disease severity exist, severe cases being prevalent in mothers and neonates of black African origin. In such cases, severe anemia as well as hyperbilirubinemia ⁶⁾ can occur and rarely antenatal hydrops fetalis has been described ⁷⁾.

Blood groups

Blood types are based on the markers (specific carbohydrates or proteins) or antigens on the surface of red blood cells. Two major antigens or surface identifiers on human red blood cells are the A and B antigens. Another important surface antigen is called Rh (Rhesus). Blood typing detects the presence or absence of these antigens to determine a person’s ABO blood group and Rh type.

There are 4 main blood groups (types of blood) – A, B, AB and O. Your blood group is determined by the genes you inherit from your parents.

Each group can be either RhD positive or RhD negative, which means in total there are 8 blood groups.

The ABO system is regarded as the most important blood-group system in transfusion medicine because of severe hemolytic transfusion reactions and, to a lesser degree, hemolytic disease of the newborn.

ABO grouping is a test performed to determine an individual’s blood type. It is based on the premise that individuals have antigens on their red blood cells (RBCs) that correspond to the four main blood groups: A, B, O, and AB. Antibodies (isohemagglutinins) in an individual’s plasma are directed against blood group antigens that their own red blood cells lack (see Table 1, below). These antibodies (isohemagglutinins) form early in life. ABO antigens are expressed on red blood cells, platelets, and endothelial cells and are present in body fluids.

ABO testing is performed to prevent an adverse transfusion reaction that could be caused by ABO incompatibility between the blood of a patient (recipient) and that of a donor.

The ABO system

There are 4 main blood groups defined by the ABO system:

• blood group A – has A antigens on the red blood cells with anti-B antibodies in the plasma
• blood group B – has B antigens with anti-A antibodies in the plasma
• blood group O – has no antigens, but both anti-A and anti-B antibodies in the plasma
• blood group AB – has both A and B antigens, but no antibodies

Table 1. ABO Genotyping

Table 2. ABO Phenotype frequencies among different ethnic groups

Blood group O is the most common blood group. Almost half of the US population (48%) has blood group O.

Receiving blood from the wrong ABO group can be life threatening. For example, if someone with group B blood is given group A blood, their anti-A antibodies will attack the group A cells.

This is why group A blood must never be given to someone who has group B blood and vice versa.

As group O red blood cells do not have any A or B antigens, it can safely be given to any other group.

The Rh system

Red blood cells sometimes have another antigen, a protein known as the RhD antigen. If this is present, your blood group is RhD positive. If it’s absent, your blood group is RhD negative.

This means you can be 1 of 8 blood groups:

• A RhD positive (A+)
• A RhD negative (A-)
• B RhD positive (B+)
• B RhD negative (B-)
• O RhD positive (O+)
• O RhD negative (O-)
• AB RhD positive (AB+)
• AB RhD negative (AB-)

About 85% of the US population is RhD positive (36% of the population has O+, the most common type).

In most cases, O RhD negative blood (O-) can safely be given to anyone. It’s often used in medical emergencies when the blood type is not immediately known.

It’s safe for most recipients because it does not have any A, B or RhD antigens on the surface of the cells, and is compatible with every other ABO and RhD blood group.

ABO incompatibility signs and symptoms

ABO incompatibility can destroy the newborn baby’s blood cells very quickly, which can cause symptoms such as:

• Edema (swelling under the surface of the skin)
• Newborn jaundice which occurs sooner and is more severe than normal

Signs of ABO incompatibility include:

• Anemia or low blood count
• Enlarged liver or spleen
• Hydrops (fluid throughout the body’s tissues, including in the spaces containing the lungs, heart, and abdominal organs), which can lead to heart failure or respiratory failure from too much fluid.

ABO incompatibility complications

Acute bilirubin encephalopathy from the buildup of bilirubin in an infant’s brain may manifest as hypotonia or poor suck reflex, which then progresses to irritability and hypertonia with retrocollis and opisthotonos. Long term consequences of chronic bilirubin encephalopathy may lead to cerebral palsy, auditory dysfunction, paralysis of upward gaze, and permanent intellectual dysfunction ⁹⁾. Thus, early recognition and treatment are imperative to prevent the detrimental progression of hemolytic disease of the newborn.

ABO incompatibility diagnosis

Hemolytic disease of the fetus and newborn should be considered in the differential diagnosis of newborns with jaundice/hyperbilirubinemia and certainly in the case of neonatal anemia. Diagnosis of hemolytic disease of the newborn can be made by identifying the presence of maternal red blood cell antibodies (agglutination in an indirect antibody test) and/or a positive direct antibody test (DAT) in the infant’s serum ¹⁰⁾. If a pregnant woman is identified to have alloimmunization, the first step in further evaluation is to determine the paternal red blood cell antigen status. If positive, the next step is to identify the fetal blood type, typically done through amniocentesis ¹¹⁾.

Which tests are done depends on the type of blood group incompatibility and the severity of symptoms, but may include:

• Complete blood count and immature red blood cell (reticulocyte) count
• Bilirubin level
• Blood typing

According to the American Academy of Pediatrics, “if a mother has not had prenatal blood grouping or is Rh-negative, a direct antibody test (Coombs’ test), blood type, and an Rh (D) type on the infant’s (cord) blood are strongly recommended” ¹²⁾.

ABO incompatibility treatment

If hemolytic disease of the fetus and newborn is identified or suspected in utero, a consult to maternal-fetal medicine should be placed as early as possible in the pregnancy. Affected pregnancies can be managed by monitoring antibody titers, and fetal middle cerebral artery velocities, intrauterine transfusions, and possibly early delivery as infants with severe anemia may not tolerate term labor well ¹³⁾.

Hemolytic disease of the newborn is managed by treating hyperbilirubinemia with phototherapy and exchange transfusions if needed. Routine universal screening with transcutaneous bilirubin often occurs at 24 hours of life, but screening should be conducted as soon as hyperbilirubinemia is suspected. An elevated transcutaneous bilirubin should always be verified with a serum total bilirubin. The hour-specific Bhutani nomogram is then used to risk stratify the amount of bilirubin in the infant’s blood ¹⁴⁾. This nomogram provides a recommended threshold for starting phototherapy versus early transfusions depending on the infant’s risk level.

Phototherapy was introduced in the 1970s and has become the mainstay of hyperbilirubinemia management in newborns. Photo isomerization causes the transformation of bilirubin into a water-soluble isomer that can then be excreted by the kidneys and stool without the need for processing in the liver. The main determinants of phototherapy efficacy are the wavelength of light used, the intensity of that light, the total light dose (time exposed and surface area exposed), and the threshold at which phototherapy is initiated. The American Academy of Pediatrics recommends the use of intensive phototherapy in hemolytic disease of the newborn. Optimal light used for phototherapy has a wavelength of 460-490 nm. The light should be at a close distance (about 20cm above the infant), and double phototherapy has proven to be more efficacious than single. There is limited data on the efficacy of continuous versus intermittent phototherapy for infants >2000g ¹⁵⁾. During the use of phototherapy, mothers should be encouraged to breastfeed their infants at timely intervals despite needing to remove them from phototherapy to do so.

Phototherapy implementation guidelines were addressed in clinical practice guidelines published by the American Academy of Pediatrics ¹⁶⁾. The recommendations are as follows:

• The guidelines are based on total serum bilirubin levels and the direct fraction should not be subtracted from the total unless it is more than 50% of the total serum bilirubin level.
• Intensive phototherapy should be started for babies with hemolytic disease. This implies the use of irradiance in the 430-490 nm band of more than 30 µW/cm²/nm delivered to as much of the infant’s surface area as possible. This can be accomplished using special blue fluorescent tubes that are labeled F20T12/BB or TL52/20W and positioning them 10-15 cm above the infant. When fluorescent tubes are used, they should be brought as close to the infant as possible to increase irradiance. However, when halogen spotlights are used, the distance above the infant should be as per the manufacturer’s instructions because spotlights can cause burns. Phototherapy lights emit minimal ultraviolet (UV) radiation that does not cause erythema and is completely absorbed by the acrylic Plexiglas covering of the tubes.
• Irradiance should be measured using radiometers recommended by the manufacturers of phototherapy systems at multiple sites on the infant’s body surface illuminated by the phototherapy lamp and the measurements averaged.
• The infant should be in the bassinet, and the sides should be lined with white cloth or aluminum foil to expose more surface area. The exposed surface area is increased by the use of 1-2 fiberoptic pads that should be placed under the infant or by the use of BiliBed or Bili-Bassinet, which provides phototherapy from above and below. The diaper should be removed if bilirubin is approaching exchange levels.
• The serum bilirubin declines by 0.5-1 mg/dL in the first 4-8 hours on intensive phototherapy and should be measured in 2-3 hours to document the effectiveness.
• If the serum bilirubin level continues to rise despite intensive phototherapy or is within 2-3 mg/dL of exchange level, administer intravenous immunoglobulin (IVIG) at 0.5-1 g/kg over 2 hours and repeat every 12 hours if needed.
• High-dose IVIG 1 g/kg given early in high-risk neonates with rapid rise of bilirubin level (>0.5 mg/kg/h) and worsening anemia (hemoglobin [Hb] < 2 g/dL) despite intensive phototherapy, is be able to eliminate the need for exchange transfusion and to reduce duration of phototherapy. The number needed to treat is 6 ¹⁷⁾.

Phototherapy is indicated in the term infant with hemolytic disease of the newborn immediately after birth due to Rh disease and due to ABO incompatibility as follows ¹⁸⁾:

• Unborn (cord blood): Total serum bilirubin level of more than 3.5 mg/dL
• Age less than 12 hours: Total serum bilirubin level of more than 10 mg/dL
• Age less than 18 hours: Total serum bilirubin level of more than 12 mg/dL
• Age less than 24 hours: Total serum bilirubin level of more than 14 mg/dL
• Age 2-3 days: Total serum bilirubin level of more than 15 mg/dL
• Immediately after birth in all preterms who weigh less than 2500 g

An exchange transfusion may be needed for severely anemic newborns, which involves replacing infant red blood cells with antigen-negative red blood cells, thereby preventing further hemolysis. 5mL/kg aliquots are removed and replaced over several minutes for a total of 25-50mL/kg exchange of red blood cells. The process is time consuming and labor intensive but remains the ultimate treatment to prevent kernicterus. The process involves the placement of a catheter via the umbilical vein into the inferior vena cava and removal and replacement of 5- to 10-mL aliquots of blood sequentially, until about twice the volume of the neonate’s circulating blood volume is reached (ie, double-volume exchange). Exchange transfusions are recommended by the American Academy of Pediatrics if total bilirubin levels remain above the transfusion threshold despite intensive phototherapy or if signs of bilirubin encephalopathy are present ¹⁹⁾. If an exchange transfusion is being considered, an albumin level should be measured. Albumin of 3.0 g/dL or less is considered an independent risk factor for hyperbilirubinemia and lowers the phototherapy threshold. Without sufficient albumin to bind bilirubin, the amount of free, unconjugated bilirubin increases, thereby increasing the risk for kernicterus ²⁰⁾.

This process removes approximately 70-90% of fetal red blood cells. The amount of bilirubin removed directly varies with the pretransfusion bilirubin level and amount of blood exchanged. Because most of the bilirubin is in the extravascular space, only about 25% of the total bilirubin is removed by an exchange transfusion. A rapid rebound of serum bilirubin level is common after equilibration and frequently requires additional exchange transfusions. However, continued hemolysis and anemia in spite of multiple exchange transfusions and negative direct antiglobulin test (DAT), should raise the possibility of absorption of IgG anti-D acquired from maternal breast milk leading to hyporegenerative anemia caused by ongoing hemolysis of erythroid precursor and marrow supression ²¹⁾.

The indications for exchange transfusion are controversial, except for the fact that severe anemia and the presence of a rapidly worsening jaundice despite optimal phototherapy in the first 12 hours of life indicate the need for exchange transfusion. In addition, the presence of conditions that increase the risk of bilirubin encephalopathy lowers the threshold of safe bilirubin levels.

Guidelines for exchange transfusion in neonates with hemolytic disease of the newborn are as follows ²²⁾:

• Total serum bilirubin level of more than 20 mg/dL: Weight more than 2500 g (healthy)
• Total serum bilirubin level of more than 18 mg/dL: Weight more than 2500 g (septic)
• Total serum bilirubin level of more than 17 mg/dL: Weight 2000-2499 g
• Total serum bilirubin level of more than 15 mg/dL: Weight 1500-1999 g
• Total serum bilirubin level of more than 13 mg/dL: Weight 1250-1499
• Total serum bilirubin level of 9-12 mg/dL: Weight less than 1250

The following are indications for exchange transfusion ²³⁾:

• Severe anemia (Hb < 10 g/dL)
• Cord bilirubin above 4 mg/dL.
• Rate of bilirubin rises more than 0.5 mg/dL despite intensive phototherapy
• Severe hyperbilirubinemia ²⁴⁾
• Serum bilirubin-to-albumin ratio exceeding levels that are considered safe

Exchange transfusion should be considered in newborns born at more than 38 weeks’ gestation with a bilirubin-to-albumin ratio of 7.2 and in newborns born at 35-37 weeks’ gestation with a bilirubin-to-albumin ratio of 6.8. Exchange transfusion is not free of risk, with the estimated morbidity rate at 5% and the mortality rate as high as 0.5%. Apnea, bradycardia, cyanosis, vasospasm, and hypothermia with metabolic abnormalities (eg, hypoglycemia, hypocalcemia) are the most common adverse effects.

Anemic infants may require blood transfusions with ABO-matched packed red blood cells. If immediate transfusion is thought to be needed, O-type, Rh-negative blood that has been leukodepleted and irradiated should be available at delivery ²⁵⁾.

Other treatment modalities have been considered, but are still controversial. Intravenous immunoglobulin (IVIG) in the infant may block Fc receptors on macrophages, thereby decreasing the breakdown of antibody-coated red blood cells. IVIG is recommended by the American Academy of Pediatrics if total serum bilirubin continues to rise despite intensive phototherapy or is within 2-3 mg/dL of the exchange transfusion level ²⁶⁾. Intravenous immunoglobulin (IVIG) has been shown to reduce the need for exchange transfusion in hemolytic disease of the newborn due to Rh or ABO incompatibility. The number needed to treat to prevent one exchange transfusion was noted to be 2.7 and was estimated to be 10, if all the infants with strongly positive direct Coombs test were to receive the medication ²⁷⁾. In addition, it also reduced the duration of hospital stay and phototherapy ²⁸⁾. Although it was very effective as a single dose, multiple doses were more effective in stopping the ongoing hemolysis and reducing the incidence of late anemia.

A randomized, controlled trial by Smits-Wintjens et al ²⁹⁾, however, failed to show the benefit of prophylactic single-dose IVIG at 0.75 g/kg within 4 hours of life in severely sensitized neonates with prior IUT due to Rh alloimmunization. Although IVIG has been proven to be safe, a retrospective review reported almost 30-times increased risk of necrotizing enterocolitis (NEC) in late preterm and term infants ³⁰⁾.

Administration of IVIG to mothers prior to delivery has not been shown to be efficacious and is not currently recommended. Other agents such as albumin, phenobarbital, metalloporphyrins, zinc, clofibrate, and prebiotics have been studied as possible treatment options for hyperbilirubinemia, but none are currently recommended ³¹⁾. In a recent randomized control trial of 70 infants with Rh-alloimmunization, delayed cord clamping was shown to improve anemia without increasing the incidence of adverse events. Delayed cord clamping had no significant impact however, on the need for exchange transfusion or duration of phototherapy ³²⁾.

ABO incompatibility prognosis

The severity of this condition can vary. Some babies have no symptoms. In other cases, problems such as hydrops can cause the baby to die before, or shortly after, birth. Severe hemolytic disease of the newborn may be treated before birth by intrauterine blood transfusions.

The overall prognosis of hemolytic disease of the newborn is good if identified and treated promptly. While permanent neurologic dysfunction may result from delays in care, this is now a rare occurrence with the advancements in monitoring as well as prophylaxis against hemolytic disease of the newborn.

Baby hunger cues

Babies instinctively let you know when she/he needs to be fed. Your baby’s body language can tell you how he’s feeling and what he needs from you. All babies give cues to how they’re feeling and what they need from you. But each baby develops her own mix of signs to tell you what she wants. Eventually you’ll get to know your baby’s individual cues and what they tell you about your baby’s feelings.

A hungry baby often will cry. But it’s best to watch for hunger cues before the baby starts crying, which is a late sign of hunger and can make it hard for them to settle down and eat.

And as you and your baby get to know each other, you’ll figure out the best way to respond to your baby’s individual cues too. For example, your grizzling baby might look relaxed when you smile at him, or he might seem to like it better when you sing and talk to him. This helps you know how to respond the next time he grizzles.

Your baby’s body language gives you important cues about whether she’s:

• tired
• hungry
• wide awake and ready to play
• needing a break.

Newborns need to feed every 2-3 hours. Here are signs your baby might be hungry:

1. Bringing his hands to his face.
2. Licking lips
3. Sticking tongue out
4. Opening her mouth
5. Sucking on everything around
6. Rooting (moving jaw and mouth or head in search of breast)
7. Making sucking motions and noises.
8. Turn towards your breast
9. Putting his/her hand to mouth repeatedly
10. Sucking on his fingers or putting his fist in his mouth.
11. Fussiness
12. Flexing his hands, arms and/or legs.
13. Breathing fast.

You can start to look for these cues every 1-2 hours in newborns or every 3-4 hours for an older baby.

When you notice your baby’s body language and respond to it, he feels safe and secure. This helps you to build a strong relationship with your baby. And a strong relationship with you and other main caregivers is vital to your baby’s development.

Watch the video to see what hunger cues look like in real babies.

Figure 1. Baby feeding cues

General guidelines for baby feeding

It is important to remember all babies are different―some like to snack more often, and others drink more at one time and go longer between feedings. However, most babies will drink more and go longer between feedings as they get bigger and their tummies can hold more milk:

• Most newborns eat every 2 to 3 hours, or 8 to 12 times every 24 hours. Babies might only take in half ounce per feeding for the first day or two of life, but after that will usually drink 1 to 2 ounces at each feeding. This amount increases to 2 to 3 ounces by 2 weeks of age.
• At about 2 months of age, babies usually take 4 to 5 ounces per feeding every 3 to 4 hours.
• At 4 months, babies usually take 4 to 6 ounces per feeding.
• At 6 months, babies may be taking up to 8 ounces every 4 to 5 hours.

Most babies will increase the amount of formula they drink by an average of 1 ounce each month before leveling off at about 7 to 8 ounces per feeding. Solid foods should be started at about 6 months old.

Do I need to wake my baby for feeds?

Newborns have tiny tummies and so need to feed frequently. For example, the Academy of Breastfeeding Medicine supplementation protocol ¹⁾ reports that the average intakes of colostrum per feed by healthy, term breastfed is 2-10 mL on day 1, 5-15 mL on day 2, 15-30 mL on day 3 and 30-60 mL on day 4. Feeding frequently in the early days and weeks helps make sure you have a good milk supply.

Because breastmilk is easily digested, it is common for breastfed babies to feed 8–12 times in a 24-hour period. This usually means that your baby will breastfeed on average every 2–3 hours around the clock (day and night), with possibly one longer stretch (eg up to about 5 hours) between feeds somewhere in a 24-hour period. The length of time between feeds can vary greatly from baby to baby. The time between feeds is from the beginning of the last feed, not the end. Newborns also tend to have periods each day where they feed very frequently for a few hours (cluster feeds). Often these cluster feeding periods are followed by a longer stretch of sleep.

In most cases, healthy, thriving newborns will wake of their own accord for feeds and so get all the milk they need. Feeding your baby when he is showing early feeding cues can help him to feed better. However, there are some reasons why a newborn may be extra sleepy. It may be necessary to wake the baby for feeds to ensure he gets the milk he needs. Some of the reasons are medical (eg jaundice, infection, heart problem), or perhaps medications for pain relief used during the birth are still affecting the baby. It is important to be guided by your doctor in these situations.

Feeding cues can be hard to pick in a sleepy baby so it is important to keep your baby close to you so you can learn.

There are many ways to tell if your baby is getting enough milk (see below).

If you need to wake your baby for feeds, here are some tips:

• Change your baby’s nappy.
• Breastfeed in a different position eg some mothers find the underarm helps keep the baby awake.
• Undress your baby down to his nappy and place him on your chest with as much skin-to-skin contact between you and him as possible.
• Give your baby a warm bath.
• Talk to him and make eye contact.
• Place your baby on your lap with his legs near your tummy and head on your knees. Gently support your baby, raise him into a sitting position, like doing a ‘sit-up’. Repeat a few times.
• Gently massage his back in circular motions.
• Stroke his feet and hands.

Babies can breastfeed even when not fully awake. Once your baby is feeding, some of the ideas above can be used to keep him awake and feeding. In addition, it can help to switch breasts each time your baby slows in his sucking and starts to doze off. Take him off, burp him and wake him again to start on the other side. He can have each side more than once if he stays awake.

It can also be helpful to use breast compressions — ie squeeze your breast while he is sucking at the breast but is not swallowing milk. Breast compressions increase the flow of breastmilk which helps encourage the baby to suck and swallow, and thus get more milk.

Even if you have a sleepy newborn who needs waking for feeds in the early weeks, this will change. Soon enough you will be able to relax and let your baby set his own individual feeding pattern.

How do I know if my baby is getting enough breastmilk?

If your baby shows the following signs then it is likely that you do have enough milk.

• At least 6 very wet cloth nappies or at least 5 very wet disposable nappies in 24 hours. The urine should be odorless and clear/very pale in color. Strong, dark urine suggest that the baby needs more breastmilk and you should seek medical advice.
• A young baby will usually have 3 or more soft or runny bowel movements each day for several weeks. An older baby is likely to have fewer bowel movements than this. Formed bowel motions suggest that the baby needs more breastmilk and you should seek medical advice.
• Some weight gain and growth in length and head circumference.
• Good skin color and muscle tone. Does she look like she fits her skin? If you gently ‘pinch’ her skin, it should spring back into place.
• Your baby is alert and reasonably contented and does not want to feed constantly. It is however normal for babies to have times when they feed more frequently. It is also normal for babies to wake for night feeds. Some babies sleep through the night at an early age while others wake during the night for some time.

A newborn’s nappies

Wide variation exists in what is seen in a newborn’s nappies. The following gives a general guide as to what might be seen.

The first bowel motions a baby has are black and sticky. This is from the meconium present in the baby’s digestive tract before birth. By day 2, the bowel motions should be softer but still dark in color. Over the next few days, the bowel motions change to a greenish-brown and then to a mustard-yellow. As the color changes they become less sticky and larger in volume.

A baby should have at least 1 wet nappy on day one, at least 2 on day two, at least 3 on day three, at least 4 on day four and at least 5 on day five. From day 5 onwards, the information above is relevant.

Over the first few days, salts of uric acid in your baby’s urine may leave a rusty, orange-red stain on the nappy. This is normal during this time. If you see this after day 4, consult a doctor.

Daily diapers

A newborn’s diaper is a good indicator of whether he or she is getting enough to eat. In the first few days after birth, a baby should have 2 to 3 wet diapers each day. After the first 4 to 5 days, a baby should have at least 5 to 6 wet diapers a day. Stool frequency is more variable and depends whether your baby is breast or formula fed.

Growth charts

During regular health check-ups, your pediatrician will check your baby’s weight and plot it on a growth chart. Your baby’s progress on the growth chart is one way to tell whether or not he or she is getting enough food. Babies who stay in healthy growth percentile ranges are probably getting a healthy amount of food during feedings.

Glue ear

Glue ear also called otitis media with effusion, is a build-up of sticky fluid (or glue) in your middle ear that can cause problems with hearing. This can cause temporary hearing loss. Glue ear is common in children. It usually clears up within 3 months and then gets better on its own without treatment, but see a doctor about any hearing problems. If glue ear hasn’t clear up within 3 months, and you or your child develops severe hearing problems, then an operation may help.

Glue ear can affect hearing because the middle ear can’t move freely, but the level of hearing loss is mild. Some children have recurrent glue ear, meaning it returns after treatment over a long period. Because of the hearing problems that come with glue ear, this can cause speech and language development problems.

Around eight out of 10 children have glue ear at least once by the time they reach the age of 10. Glue ear tends to affect children under the age of seven and your child is most likely to get it between the ages of two and five. They’re more likely to get it during the winter months and it often comes and goes.

Some children develop glue ear after a cold, so it can be more common during the winter months. It’s also more common in boys than girls, but scientists don’t know why yet.

When the tube between your middle ear and the back of your throat, the Eustachian tube, becomes blocked, air can’t get in and it starts to fill with fluid. This makes it harder for sounds to pass through the middle ear to the inner ear, which is what affects your child’s hearing.

Glue ear usually gets better over a few months and most children will no longer have it after a year. For a few children it carries on longer and can cause hearing loss, which can affect their education, language development and behavior.

There are lots of things you can do to help your child. If you smoke, then your child is more likely to develop glue ear so now is a good time to stop or to smoke outside, well away from your children.

How do you hear?

The ear consists of three parts, the outer ear, the middle ear and the inner ear. Sound waves enter the ear canal and cause the eardrum to vibrate. The sound then passes through the middle ear through the three small bones of hearing (ossicles) on to the inner ear, which is filled with fluid.

The movement of the fluid in the cochlea stimulates the hair cells inside it to trigger a nerve impulse, which is carried to the brain by the auditory nerve. The brain then interprets these nerve impulses as sound.

Figure 1. Ear anatomy

Glue ear in adults

Adults can get glue ear, though it’s much less common than in children. Treatment is generally the same as for children. You’re likely to notice some hearing loss, but may also hear popping sounds.

If you think you may have glue ear as an adult, get it checked out by your doctor.

What are grommets?

Grommets are tiny tubes inserted into the eardrum. They allow air to pass through the eardrum, keeping the air pressure on either side equal. The surgeon makes a tiny hole in the eardrum and inserts the grommet into the hole. It usually stays in place for six to 12 months and then falls out. This is normal and won’t affect your child.

What care should I take with grommets?

Your surgeon is likely to ask you to keep your child’s ears dry for a few weeks after the operation. This helps to stop water getting into the ear and causing an infection while it’s healing.

You’ll be asked to take care when bathing, showering or washing your child’s hair. You’ll also be asked not to take your child swimming.

Surgeons give different advice about how long you’ll need to do this, so ask for information. After the first few weeks, there’s no evidence that wearing earplugs or not swimming or bathing will make any difference to the likelihood of getting an infection.

My child has glue ear and we’re going on holiday, will we be able to fly?

It’s usually OK to fly if your child has glue ear. However, if your child has an ear infection it can sometimes cause problems.

As an airplane takes off, the air pressure in the cabin drops and that causes gases to expand. As the airplane comes down to land, the air pressure increases and gases contract. These changes affect the air in your middle ear and if your child has a middle ear infection, it can be painful and can cause injury. It’s a good idea to check with your doctor before your child travels. They may prescribe decongestant nose drops to use before take-off and landing.

Glue ear causes

Doctors aren’t completely sure why glue ear happens. Different factors are involved, but in more than a half of children glue ear follows an infection of the middle ear.

Other possible causes include:

• problems with the Eustachian tube – the tube between the middle ear and the back of the throat
• an infection (a virus or bacteria)
• inflamed middle ear
• infected adenoids

Your child is more likely to get glue ear if they:

• live with someone who smokes
• have repeated colds and throat infections
• have allergies
• are fed from a bottle when lying down
• have contact with lots of children, such as at a nursery, or have older siblings
• have a cleft palate or Down’s syndrome

Glue ear symptoms

The most common symptom of glue ear is temporary hearing loss. It can affect both ears at the same time. But sometimes the symptoms of glue ear can be hard to spot, especially if your child is very young.

Other symptoms may include:

• earache or ear pain
• hearing sounds like ringing or buzzing (tinnitus)

Glue ear is much more common in children, but adults with glue ear have the same symptoms.

You might see this as some of the following behavior in kids.

• Mishearing what you say.
• Not responding when you talk to them, especially from behind.
• Having the television volume up high.
• Having trouble concentrating.
• Preferring to play on their own, rather than in a group.
• Becoming tired.
• A change in behavior.

Other symptoms include feeling like the inside of the ear is blocked, mild ear pain and hearing ‘popping’ in the ear. Some children have repeated ear infections, coughs and colds before they get glue ear.

If hearing loss is severe, or it’s been a symptom for a while, your child may have problems with speech and language. Their listening skills can also be affected and these things together can affect their progress at nursery or school.

Glue ear diagnosis

Your doctor will ask about your child’s symptoms and medical history. Your doctor can usually diagnose glue ear by looking for fluid inside your or your child’s ear and eardrum.

They’ll use a small scope with a magnifying glass and a light called the otoscope. This should not be painful.

If your child has had glue ear for more than 3 months, they may be referred to a specialist for hearing tests. There are two main types of hearing test. Tympanometry assesses how well their eardrum reacts to sound and audiometry tests their hearing. The type of test your child has will depend on how old they are and their stage of development.

Hearing tests can help find out how severe any hearing loss is and what’s causing it.

Your doctor may monitor your child for three months and then order another hearing test.

If after three months your child’s symptoms haven’t got any better, your doctor may refer them to an ear, nose and throat (ENT) specialist. If your child has Down’s syndrome or a cleft palate, they’re likely to be referred to a specialist straightaway.

Glue ear treatment

Non-surgical treatments

Glue ear is not always treated. Your doctor will usually wait and see if the symptoms get better on their own. This is because there’s no effective medicine for glue ear, and it often clears up on its own within 3 months.

They may still monitor your child for up to a year in case the symptoms change or get worse.

Your doctor may suggest trying a treatment called autoinflation, while waiting for symptoms to improve. Autoinflation helps to open the Eustachian tube to let air into the middle ear so fluid in the middle ear can drain out.

Autoinflation is done by either:

• blowing up a special balloon using one nostril at a time
• swallowing while holding the nostrils closed

As autoinflation has to be done several times a day, it’s not usually recommended for children under 3 years old.

Your child may need to wear hearing aids if they have hearing loss in both ears and surgery isn’t an option. Wearing a hearing aid can make some children anxious, so your doctor will talk to you about your choices.

If glue ear causes an ear infection, your doctor may prescribe antibiotics.

Antihistamines, steroids and decongestants don’t work as treatments for glue ear. There is also no evidence that complementary therapies such as homeopathy or special diets will help.

Nasal balloon instructions

The nasal balloon comes in a treatment pack (Otovent) containing 5 medical balloons, a connecting nozzle and a small carrying case.

Watch videos demonstration of nasal balloon autoinflation.

Step by step instructions:

1. Take one of the balloons and stretch it using your hands or blow it up with your mouth to release some of the tension, otherwise the first inflation may be quite difficult.
2. Attach the balloon to the connecting nozzle.
3. To make sure your child understands what they need to do either show them the instruction video or demonstrate the nasal balloon yourself.
4. Ask your child to hold the nozzle up to one nostril and close off the other nostril with their fingers.
5. Instruct your child to keep their mouth closed, slowly blow out of the nose and inflate the balloon to about the size of a large orange. Repeat with the other nostril.
6. Give lots of feedback and encouragement. Using a sticker chart can help you and your child to remember to use the balloon regularly

Figure 2. Nasal balloon autoinflation method

Surgery for glue ear

Your child may be referred to a specialist in hospital if:

• glue ear symptoms are affecting their learning and development
• they already had severe hearing loss before glue ear
• they have been diagnosed with Down’s syndrome or a cleft lip and palate, as glue ear is less likely to get better by itself

Your doctor may suggest surgery if the glue ear is no better after three months and your child has severe hearing loss. Surgery is also an option if your child’s learning or development is affected.

The main surgery for glue ear is to make a tiny cut in the ear drum and put in a plastic tube called a grommet. These work by allowing air to get in and out of the middle ear, which helps to drain the fluid. Grommets are usually put in with a general anesthetic, which means your child will be asleep during the procedure.

Grommets can improve hearing for up to nine months, but they have little effect on speech or language development in the long term. Different grommets stay in for different lengths of time, but eventually they fall out and the eardrum closes.

In rare cases, surgery may be recommended to remove some glands at the back of the nose (adenoids). This is known as an adenoidectomy.

The specialist in hospital will help you decide on the best treatment option.

Figure 3. Glue ear grommets

Grommets for treating glue ear

Grommets are small temporary tubes that are placed in your child’s ear during surgery. They help drain fluid away and keep the eardrum open.

The grommet should fall out naturally within 6 to 12 months as your child’s ear gets better.

Are there any risks to grommets?

Every operation carries some risk of infection and bleeding but the hole in the eardrum is tiny, so this risk is reduced. Your ear, nose and throat (ENT) surgeon give your child a course of antibiotic eardrops to reduce the risk of infection further. Around one in every 100 children may develop a perforated eardrum. If this continues, your surgeon can repair it later. Every anesthetic carries a risk, but it’s very small. Modern anesthetics are safe and your child’s anesthetist will be an experienced doctor trained to deal with any complications.

Glue ear prognosis

Once your child’s glue ear is diagnosed and treated glue ear, the outlook is good. Most children with speech and language delays catch up and go on to have a normal school life. A small proportion of children may need extra help from a speech and language therapist.

Bleach bath

Bleach baths are recommended in patients with frequent skin infections to reduce skin colonization by pathogenic bacteria, particularly Staphylococcus aureus. Bleach baths have also been reported to reduce the severity of atopic dermatitis (eczema) and to reduce the need for topical steroids and antibiotics. Bleach baths also seem to reduce inflammation and itching in eczema. The current clinical guidelines for management of eczema or atopic dermatitis now recommend bleach baths. This requires simply the addition of regular household bleach to twice-weekly baths.

Bleach is the generic name for any chemical product which is used industrially and domestically to clean, and to remove stains. Bleach often refers, specifically, to a dilute solution of sodium hypochlorite (NaOCl or NaClO), also called “liquid bleach”, a household chemical widely used (since the 18th century) as a disinfectant or a bleaching agent. The compound in solution is unstable and easily decomposes, liberating chlorine, which is the active principle of such products. Indeed, sodium hypochlorite is the oldest and still most important chlorine-based bleach.

Bleach baths are dilutions of household bleach with water in a bath. The concentration of chlorine is similar to that of a swimming pool. It has antimicrobial action.

• The active compound in commercial household bleaches is sodium hypochlorite (NaOCl).
• Bleach contains 3–6% available chlorine (Cl2), approximately 0.5–1.5% sodium hydroxide (NaOH) as a stabilizer, small quantities of surfactants, and perfume.
• At pH 11–13, it is an alkaline aqueous solution with major oxidizing properties.
• Sodium hypochlorite 0.0006% is also available as a convenient wash suitable to use in a shower.

Bleach bath for your skin

Mix 2 ml of 2.2% household bleach for every 1 liter of water to make a 0.005% solution. Brands vary strength, so check the label. If the concentration is higher, use less bleach, and if the concentration is lower, use more bleach.

• Soak trunk and limbs in the bath for 10–15 minutes. Do not submerge head.
• Rinse off with warm tap water and pat dry with a towel. Avoid sharing towels.
• Apply topical steroid and moisturizers if prescribed.
• Bleach baths are recommended at least twice a week.
• Keep bleach out of reach from children.

Bleach bath side effects

Exposure to bleach baths can lead to:

• Dry skin
• Rash/dermatitis/skin irritation
• Asthma exacerbation
• Nasal irritation.

These symptoms are often due to added perfumes or detergents in the bleach. If troublesome, try another brand. Do not apply the bleach directly to the skin.

Toxicity or poisoning is rare but potentially very serious. It is usually due to ingestion of undiluted solution. Keep bleach in its original container with a child-proof top and make sure young children are not able to open it.

Ingestion of small volumes of bleach bath water may lead to:

• Irritation of the oropharynx,
• Burning sensation in the mouth and throat
• Thirst
• Nausea
• Vomiting
• Vomiting blood (hematemesis).

Ingestion of large amounts or more concentrated solutions can lead to:

• Corrosion of the mucous membranes
• Chest and abdominal pain
• Watery diarrhea
• Melena (blood in feces)
• Ulceration or perforation of the esophagus or stomach
• Hemorrhage and shock.

Bleach bath for staph infection

Staphylococcal infections of the skin and soft tissue are common, especially in children, and can be severe. Despite treatment, more than 20% of patients with skin and soft tissue infections have a recurrence within three months. Primary staphylococcal skin infections include:

• Impetigo
• Boils
• Folliculitis.

Secondary staphylococcal infections include:

• Infected eczema
• Infected wounds, including surgical wounds and trauma.

Predisposing factors for skin and soft tissue infections include:

• Bacterial colonization of the patient’s skin with Staphylococcus aureus
• The colonization of a family member’s skin
• Colonization with methicillin-resistant Staphylococcus aureus (MRSA)
• Dermatitis, particularly atopic eczema, discoid eczema and venous stasis eczema.

Attempts to reduce Staphylococcus aureus (S. aureus) by decolonization of the skin include combinations of:

• Bleach baths or shower washes ¹⁾
• Mupirocin nasal ointment
• Oral antibiotics (eg, flucloxacillin, rifampicin, doxycycline)
• Chlorhexidine solution as a bath or shower antiseptic cleanser
• General hygiene and wound care.

Bleach bath benefits

In conjunction with other methods of decolonization, bleach baths reduce rates of recurrent primary and secondary skin and soft tissue infections.

Bleach baths have a broad spectrum of action against:

• Bacteria (Gram-positive and Gram-negative)
• Fungi and spores
• Viruses.

No microbial resistance has been reported to sodium hypochlorite. The effect of bleach baths on decolonizing the patient’s skin is only temporary, so must be repeated regularly.

Bleach baths also seem to reduce inflammation and itching in eczema ²⁾.

Bleach bath for eczema

Clinical trials have actually proven that bleach baths to be very effective. In one of the first formal studies ³⁾, conducted at Northwestern University’s Feinberg School of Medicine, 31 children between the ages of 6 months and 17 years suffering from moderate to severe eczema were treated. Additionally, the children had signs that indicated they had some form of bacterial infection that aggravated their condition.

Half of the patients bathed in a tub of water diluted with household bleach twice a week, and the other half took baths without bleach twice a week. The bleach baths used one-half cup of 6% bleach in an average-size bathtub that was full of water (40 gallons). The final concentration of bleach to water was 0.005%. All patients also took an oral antibiotic (cephalexin) and had mupirocin ointment applied in their noses. Additionally, all patients were instructed to use anti-inflammatories and emollients as they normally would.

After 3 months of treatment, the children who took the bleach baths had a dramatic reduction in the severity of their eczema, 5 times greater than those who took the placebo baths. No patients withdrew from the study because of intolerance to the baths; however, one patient who received the bleach baths reported skin irritation and itching. That patient dropped out of the study and then developed a multidrug-resistant Staphylococcus aureus skin infection (commonly known as a staph infection) that resulted in hospitalization and IV antibiotics. After discharge from the hospital, the patient started bleach baths again and had no adverse effects.

Part of the reason the bleach bath therapy may be so effective is that 9 out of 10 people with eczema have Staphylococcus aureus on their skin, including some with methicillin-resistant Staphylococcus aureus (MRSA). Only about 2 out of 10 people in the general population do. Frequent scratching by eczema sufferers breaks the skin, which increases the likelihood of Staphylococcus aureus causing an infection. The diluted bleach helps to kill the microbes that cause these infections.

What is so extraordinary about this treatment is that it is not an expensive topical cream or new drug. For a few cents’ worth of household bleach, patients showed remarkable results comparable to more sophisticated and expensive medical treatment. For those with very sensitive skin, it is advisable to rinse off in the shower after a bleach bath.

While this may not be good news for pharmaceutical companies, it certainly is for eczema sufferers. Doctors now routinely recommend bleach baths to their patients with moderate to severe eczema in addition to regular gentle skin care and appropriate medication as needed. However, as with all treatments, people respond differently, so be sure to consult with your dermatologist before trying this therapy.

Vascular ring

A vascular ring occurs when the aorta (the large artery that carries oxygenated blood out of the heart) or its branches form a complete ring around the trachea (the tube that carries air to the lungs) and the esophagus (the tube that carries food to the stomach) ¹⁾. This happens when certain parts of the aorta that normally disappear during fetal development persist abnormally. The aorta starts normally at the heart as one large vessel, but then divides into two arches: one on each side of the trachea and esophagus. The two arches then come back together into one vessel (the descending aorta).

“Vascular” means relating to blood vessels (veins and arteries). The condition is called “vascular ring” because the aorta or other, related arteries — encircles the trachea and esophagus.

Normally, the aorta starts at the left ventricle of the heart as one large vessel: it arches up (the aortic arch) to the left of the trachea and then down (the descending aorta). Arteries that deliver blood to the head, arms and other parts of the upper body branch off at the top of the arch. Arteries that deliver blood to the abdomen, legs and other parts of the lower body branch off from the descending aorta.

As their name suggests, vascular rings are caused by malformed blood vessels. But the vessels themselves aren’t really the issue, and some people without symptoms may live their entire lives not even realizing that they have a vascular ring. Instead, symptoms occur when a vascular ring puts pressure on a child’s esophagus, trachea, or both.

Vascular ring types

There are different types of vascular rings. The most common are:

Double aortic arch

Double aortic arch is a congenital (present at birth) abnormal formation of the aorta, where both the left and right aortic arches are present, resulting in a complete circle around the trachea and esophagus ²⁾. Double aortic arch is the most common cause of tracheoesophageal compression, with an incidence of 46% to 76% in reports of vascular rings ³⁾.

The fetal heart starts with two symmetrical branches of the aorta. Parts of the aortic arch regress in the second month of fetal life, usually resulting in a left aortic arch with three artery branches: the innominate artery, the left common carotid artery, and the left subclavian artery. The ductus arteriosus is present from the left pulmonary artery to the aorta, near the left subclavian origin. Double aortic arch occurs when there is abnormal regression of parts of the original two aortic arch branches. A left and a right aortic arch are present, resulting in a complete circle around the trachea and esophagus. The right aortic arch is usually larger than the left aortic arch. The right arch gives rise to the right common carotid artery and right subclavian artery and the left arch gives rise to the left common carotid artery and left subclavian artery.

Generally, incidence of double aortic arch and vascular rings is unknown, although vascular rings comprise an estimated 1% of cardiovascular malformations that are managed surgically. In most surgical series, 45-65% of patients undergoing repair of a vascular ring have a double aortic arch ⁴⁾.

Figure 1. Double aortic arch

Right aortic arch with left ligamentum arteriosum

The aorta starts off to the right, instead of the left (as is normal), and gives off an abnormal branch from the descending aorta. This abnormal branch passes behind the esophagus and gives off the vessel to the left arm (the left subclavian artery) and the ligamentum arteriosum. The ligamentum arteriosum is a remnant of a blood vessel that exists normally in the fetus; it runs between the aorta and the pulmonary artery, and in doing so completes the ring and also might constrict the trachea and/or esophagus.

The structure of the vascular ring and its relationship to the trachea and esophagus varies from child to child.

As viewed through a left thoracotomy, the descending thoracic aorta is visible and can be traced proximally to the area of its merger with the right aortic arch, which joins it from behind the esophagus. In this area, the ductus diverticulum and ligamentum arteriosum can be found.

Figure 2. Right aortic arch with left ligamentum arteriosum

Right aortic arch with retroesophageal left subclavian artery and left ligamentum arteriosum

The aortic arch travels to the right and behind the esophagus, joining the left-side descending aorta. As it takes this course, it gives off the left carotid artery first. Then, it sequentially gives off the right carotid, the right subclavian, and the left subclavian arteries. This last branch often has a retroesophageal position. The ligamentum arteriosum courses from the base of the left subclavian artery to the left pulmonary artery.

Through a left thoracotomy, the structures visible in normal position are the descending thoracic aorta and the distal portion of the left subclavian artery. These structures can be traced proximally to identify the site where the left subclavian artery exits the right arch as it joins with the descending aorta. The ligamentum arteriosum can be found at the base of the subclavian artery and traced towards the pulmonary artery.

Figure 3. Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum

Left aortic arch with right descending thoracic aorta and right ligamentum arteriosum

The arrangement of this anomaly is the mirror image of right aortic arch with retroesophageal left subclavian artery and left ligamentum arteriosum. Approached through a right thoracotomy, structures are identified and traced in the same manner.

Figure 4. Left aortic arch with right descending thoracic aorta and right ligamentum arteriosum

Left aortic arch with right descending aorta and atretic right arch

In a retroesophageal position, the left arch passes to the left of the trachea to join the right descending thoracic aorta. The surgical view is via a right thoracotomy. At the level of this junction and immediately superior to a right ligamentum arteriosum, an atretic right arch is found. The brachiocephalic vessels in this case exit the left arch in a normal sequence.

Anomalous innominate artery

The brachiocephalic vessels exit the left arch in the normal sequence. The innominate artery may be more distally positioned and leftward on the arch than normal.

Retroesophageal right subclavian artery with left aortic arch and left ligamentum arteriosum

Rather than an innominate artery exiting the arch as the normal first brachiocephalic branch, the right carotid is the first brachiocephalic vessel. It is followed by the left carotid and then the left subclavian artery. The right subclavian artery is the last brachiocephalic branch to exit the arch, and it takes a course rightward and posterior to the esophagus. The ligamentum arteriosum is normally positioned on the left.

Figure 5. Retroesophageal right subclavian artery with left aortic arch and left ligamentum arteriosum

Anomalous left pulmonary artery (pulmonary artery sling)

Instead of the normal pulmonary artery configuration, in which the main pulmonary artery gives rise to the right and left pulmonary arteries, the main pulmonary artery continues behind the ascending aorta and rightward as the right pulmonary artery, which then gives off the left pulmonary artery. The left pulmonary artery passes from right to left between the trachea and esophagus in its course to the left lung. The ligamentum arteriosum takes a course from the junction of the main and right pulmonary arteries posteriorly to the aorta.

Figure 6. Pulmonary artery sling

Vascular ring causes

Normally, the aorta develops from one in a series of symmetrical arches (six embryonic branchial arches). By the end of the second month of fetal development, the other arches are naturally broken down or formed into arteries. When a vascular ring occurs, certain arches that should have disappeared still remain and form a ring structure. Several papers have reported the close association of band 22q11 deletion with anomalies of the aortic arch, as well as other congenital cardiac abnormalities ⁵⁾.

In normal development, each primitive arch either progresses into a functional vascular structure or involutes as follows ⁶⁾:

• The right and left first and second arches become a portion of the arterial supply to the face
• The third arches develop into the carotid arteries
• The dorsal aorta between the third and fourth arches involutes
• The fourth arches are the primary contributors to the aortic arch itself, with the proximal right fourth arch developing into the proximal right subclavian artery; the distal portion of the right fourth arch involutes at the point where it joins the dorsal arch; the left fourth arch remains as the aortic arch in normal development
• The fifth arches involute bilaterally
• The ventral right sixth arch becomes the proximal right pulmonary artery; the ventral portion of the left sixth arch develops into the left pulmonary artery, while the dorsal portion becomes the ductus arteriosus
• The entire left and the distal right subclavian arteries arise from the seventh segmental arterial branches of the dorsal aorta

Abnormal arch development results when one or more of the necessary involutions or other changes in embryonic arch structures does not occur.

Tracheal or tracheobronchial malacia and stenosis may develop in association with some of these lesions in the areas where the greatest degree of compression exists. This is particularly true in cases of anomalous left pulmonary artery.

Other congenital cardiac anomalies may be present in association with aortic arch anomalies.

The presence of a right aortic arch should always raise the suspicion of additional congenital cardiac disease. Right aortic arch has been reported in as many as 34% of cases of tetralogy of Fallot. Other intracardiac lesions found in association with a right aortic arch include double-outlet right ventricle, truncus arteriosus, ventricular septal defect with pulmonary atresia, transposition of the great vessels, tricuspid atresia, and absent left pulmonary artery.

Anomalous left pulmonary artery has also been associated with other cardiac defects in as many as 50% of cases. These include the aforementioned lesions, as well as persistent left superior vena cava, atrial septal defect, and ventricular defect. Complete tracheal rings (congenital absence of the membranous trachea) resulting in tracheal stenosis are another anomaly reported in as many as 50% of infants with anomalous left pulmonary artery. The combination of these two lesions is often termed the ring-sling complex.

Vascular ring signs and symptoms

In most cases, the symptoms of a vascular ring are mild. Some people without symptoms may live their entire lives not even realizing that they have a vascular ring. Instead, symptoms occur when a vascular ring puts pressure on a child’s esophagus, trachea, or both. That means the condition may go undiagnosed until your child is older, or even an adult. Symptoms range widely depending on the severity of the compression. If the condition is severe and your child has trouble breathing, a vascular ring may be diagnosed at several weeks or months of age.

The following symptoms may be present in children with vascular rings:

• Stridor (noisy breathing) or labored breathing, especially with eating
• Difficulty eating and swallowing: the child regurgitates milk or other food
• Difficulty feeding when you introduce solid foods
• Swallowing difficulties (dysphagia): the child feels like something is “stuck in the throat”; the child keeps choking or feels like he or she is choking on food
• Persistent cough or wheezing
• Acid reflux or gastroesophageal reflux (GERD)
• Respiratory distress
• Respiratory infections

Children with vascular rings often also have an airway disorder called tracheomalacia. In this condition, the trachea narrows or collapses when your child exhales, which makes it feel hard to breathe and may lead to a vibrating noise or cough. Like vascular rings, tracheomalacia can be present at birth, or it can develop in response to vascular rings or other anomalies.

Vascular ring diagnosis

If your child has any symptoms of a vascular ring — particularly noisy breathing or a weak pulse — their doctor may refer you for testing.

Diagnosis of a vascular ring may require some or all of these tests:

• Chest X-ray
• Cardiac MRI : a three-dimensional image that shows heart and vessel abnormalities
• Chest CT (CAT scan)
• Echocardiogram : sound waves create an image of the heart
• Bronchoscopy: a doctor looks at the trachea using an instrument called a bronchoscope (while patient is under anesthesia)
• Gastrointestinal tests such as barium swallow or barium esophagram. Barium (contrast) fluid is consumed by mouth and coats the esophagus. Fluoroscopy (x-ray) is done at the same time the barium is consumed, which reveals compression or obstruction of the esophagus

The gold standard for evaluating and identifying vascular rings is a computed tomography (CT) scan. This technique allows physicians to visualize your child’s vascular anatomy properly. Vascular rings can be diagnosed at any age, including while a child is still in the womb.

Vascular ring treatment

Children who with vascular rings but no symptoms usually don’t need surgical treatment. However, those who have symptoms from vascular rings typically require to surgery to relieve pressure on their airway and esophagus. Surgeons may use a variety of techniques to accomplish this, including:

• Complete resection of the diverticulum of Kommerell, which is typically left as an out-pouching of the aorta and compresses the esophagus and airway from the back
• Descending aortopexy, which moves the descending aorta to the side of the spine so it doesn’t compress the airway
• Rotation esophagoplasty, which moves the esophagus out of the airway so it doesn’t contribute to airway compression and can’t be compressed itself by the aorta and airway
• Posterior tracheobronchopexy, which keeps the airways open in children with vascular rings who also have tracheobronchomalacia below the narrow, compressed region of the airway. Occasionally, these children will also need anterior airway support to completely open the airways.
• Aortic uncrossing, which reroutes the aorta and can be combined with the airway procedure

Vascular surgeons repair vascular rings with a combination of these procedures in one comprehensive surgical procedure. In most cases, the surgeon makes an incision on the left side of the chest and goes between the ribs to access the area.

Double aortic arch surgery

Children with double aortic arch that are asymptomatic do not require surgical intervention. Timing of surgical intervention is determined by the severity of associated symptoms. Double aortic arch requires surgery (not open heart) if there are symptoms. In most cases, the surgeon makes an incision on the left side of the chest and goes between the ribs to access the area. The surgeon will cut and stitch closed one of the arches (usually the left) so the aorta no longer encircles or puts pressure on the trachea or esophagus. Children need careful respiratory management in the postoperative period. Normal life expectancy and development for children without other congenital anomalies.

The surgeon will cut and stitch closed one of the arches (usually the left) so the aorta no longer encircles or puts pressure on the trachea or esophagus.

Figure 7. Double aortic arch repair

Follow-up care

Through age 18

In most cases, children with double aortic arch recover completely after surgery and won’t need additional procedures. It is not uncommon for noisy breathing to continue for several weeks or months after surgery as the trachea slowly takes on a more normal shape. Rarely, symptoms such as coughing or labored breathing continue after surgery. Your child’s surgeon will monitor your child to make sure recovery is as complete and rapid as possible.

Pediatric cardiologists will follow your child until she is a young adult, coordinating care with her primary care physician. She will need to carefully follow doctors’ advice. In rare cases, pediatric cardiologists will suggest that your child limit certain types of exercise.

Into adulthood

Children born with double aortic arch who do not require surgery should follow up with a cardiologist.

Right aortic arch with left ligamentum arteriosum

The surgeon divides the ligamentum arteriosum so that it no longer constricts the trachea or esophagus. The right aortic arch remains, as a person can live with this irregularity.

In some cases, a child has no symptoms of vascular ring and the condition is discovered during an unrelated medical test, procedure or evaluation. In some of these cases, instead of surgery, a cardiac surgeon will monitor the child to make sure symptoms don’t appear and the condition isn’t harming the the child’s health.

The procedure to correct vascular ring is considered low-risk. In most cases, surgery is recommended.

Vascular ring prognosis

Because of enormous strides in medicine and technology, today most children with heart defects such as vascular rings will go on to lead productive lives as adults. About 95% of patients who undergo surgical correction of a vascular ring survive for long periods, and most of these are soon relieved of their symptoms. In those infants with no intracardiac or extracardiac defects, surgery for vascular rings carries essentially no mortality.

Among those with less optimal long-term results are patients with an anomalous left pulmonary artery with and without complete tracheal rings and those with severe associated congenital cardiac defects. In those patients with a severely deformed trachea or tracheomalacia, additional reconstruction procedures may be required in the future.

In a series reported by Backer et al, reoperation proved necessary for 26 of 300 patients who had undergone surgical intervention for vascular rings ⁷⁾. The four primary indications for reoperation were Kommerell diverticulum (18 patients), circumflex aorta (two patients), residual scarring (two patients), and tracheobronchomalacia necessitating aortopexy (four patients).

A number of patients continue to show evidence of some pulmonary function abnormalities years after surgery. Several studies report that measured pulmonary function studies show some degree of airway obstruction in as many as 50% of patients 7-8 years postoperatively. Also, a large number of patients appear to have a pronounced bronchial responsiveness to histamine.

Naimo et al ⁸⁾ reported long-term outcomes of complete vascular ring division in 132 children from a 36-year experience at a single institution (median follow-up, 11.4 years; range, 44 days to 36 years). In-hospital mortality was 1.5% (2/132), and no late deaths occurred. Overall survival was 98.3 ± 1.2% at 20 years. After surgical treatment, three patients experienced persistent tracheal compression and 16 had tracheomalacia. The freedom-from-reoperation rate was 88.6 ± 4.0%. None of the patients required tracheal surgery during the follow-up period.

François et al ⁹⁾ assessed early and late outcomes (mean follow-up, 7.8 ± 5.8 years) in 62 patients (median age, 1 year) who underwent surgical treatment of a vascular ring (most commonly a double aortic arch [53%]). Median extubation time was 4 hours, and median hospital stay was 5 days. Early mortality was 8% and was associated with the anatomic diagnosis, concomitant anomalies, and the need for preoperative intubation. At 1 month, 63% of patients were free of residual symptoms; at 6 months, 82% were. At final follow-up, the rate of freedom from inhalation therapy was 82%.

Low birthweight

Low birthweight is when a baby is born weighing less than 2500 grams (less than 5 pounds, 8 ounces). In contrast, the average newborn weighs about 8 pounds (3628 grams). Some babies with low birthweight are healthy, even though they’re small. But being low birthweight can cause serious health problems for some babies. A baby with low birthweight may have trouble eating, gaining weight and fighting off infections. Some low-birthweight babies may have long-term health problems too, with an increased risk of physical and mental disabilities. This risk becomes significant for those babies born weighing less than 1500 g. About 1 in 12 babies (about 8 percent) in the United States is born with low birthweight. The overall rate of these very small babies in the United States is increasing. This is primarily due to the greater numbers of multiple birth babies who are more likely to be born early and weigh less. Over half of multiple birth babies have low birthweight compared with only about 6 percent of single birth babies.

Low birth weight infants can be further classified into ‘very low birth weight’ (VLBW) if they weigh 1000–1500 g and ‘extremely low birth weight’ (ELBW) if they weigh less than 1000 g. Such low birth weights are seen in infants who are born extremely prematurely.

The primary cause of low birthweight is premature birth (being born before 37 weeks gestation). Being born early means a baby has less time in the mother’s uterus to grow and gain weight. Much of a baby’s weight is gained during the latter part of pregnancy.

Another cause of low birthweight is intrauterine growth restriction (IUGR). This occurs when a baby does not grow well during pregnancy because of problems with the placenta, the mother’s health, or the baby’s condition. A baby can have IUGR and be born at full term (37 to 41 weeks). Babies with IUGR born at term may be physically mature but may be weak. Premature babies that have IUGR are both very small and physically immature.

Who is affected by low birthweight?

Any baby born prematurely is more likely to be very small. However, there are other factors that can also contribute to the risk of very low birthweight. These include:

• Race. African-American babies are two times more likely to have low birthweight than white babies.
• Age. Teen mothers (especially those younger than 15 years old) have a much higher risk of having a baby with low birthweight.
• Multiple birth. Multiple birth babies are at increased risk for low birthweight because they often are premature. Over half of twins and other multiples have low birthweight.
• Mother’s health. Babies of mothers who are exposed to illicit drugs, alcohol, and cigarettes are more likely to have low birthweight. Mothers of lower socioeconomic status are also more likely to have poorer pregnancy nutrition, inadequate prenatal care, and pregnancy complications–all factors that can contribute to low birthweight.

Extremely low birth weight or very low birth weight infants

The following problems are commonly associated with extreme prematurity and very low birth weight (weigh 1000–1500 g) or extremely low birth weight (weigh less than 1000 g).

Respiratory problems

extremely low birth weight infants are more likely to develop bronchiolitis (inflammation of the small airways in the lungs) or recurrent wheeze. Babies with bronchiolitis often need to be treated in hospital.

These infants also usually have reactive airways disease, which tends to cause symptoms similar to asthma. About 20 per cent of all extremely low birth weight infants will have asthma-like symptoms into middle childhood.

Exercise problems

Children who were born very prematurely generally have a lower exercise capacity than those who were born at full term. This is believed to be due to a combination of factors, including reduced lung function, reduced muscle bulk, slim build and coordination problems.

Exercises that help control breathing, such as swimming, are recommended. These may also enhance lung growth. Weight-bearing exercise, such as walking, should also be encouraged, as it will help develop strong bones.

Nutritional problems

Feeding problems are not uncommon in low-birth-weight infants. Iron and multivitamin supplements should be given for the child’s first 6 months, before the introduction of solids.

Once these infants reach the toddler stage, growth tends to occur at a consistent and steady rate. But it’s important to remember that when their height and weight are plotted on standard growth charts, children born prematurely may be below the average for their age.

Functional problems

While severe functional problems that limit a child’s ability to perform everyday tasks such as dressing, toileting and feeding are not common, they do occur more often in children who were born extremely prematurely than in children who were born at full term.

In addition, many extremely low birth weight infants will go on to experience milder functional impairment, such as speech difficulties or trouble understanding simple instructions. As a result, they are more likely to need physiotherapy, speech therapy or occupational therapy. Regular reviews by appropriate health professionals will help in the early detection of any difficulties.

Early detection and subsequent early intervention with the appropriate treatment can help maximize the functional and educational opportunities for these children.

Low birth weight causes

There are two main reasons why a baby may be born with low birthweight:

1. Premature birth. This is birth that happens too soon, before 37 weeks of pregnancy. Being born too early means a baby has less time to grow and gain weight in the womb. The earlier a baby is born, the lower her birthweight may be. About 1 in 10 babies in this country is born prematurely each year. Talk to your health provider about things you can do to help reduce your risk of having a premature baby.
2. Fetal growth restriction also called growth-restricted, small for gestational age (SGA) and small for date. This means a baby doesn’t gain the weight she should before birth. Some growth-restricted babies may have low birthweight simply because their parents are small. Others may have low birthweight because something slowed or stopped their growth in the womb. Your health care provider measures your belly and uses ultrasound to help track your baby’s growth during pregnancy. Ultrasound uses sound waves and a computer screen to show a picture of your baby inside the womb. If your provider thinks your baby is growth-restricted, you may get ultrasounds more often (every 2 to 4 weeks) to track your baby’s growth. Your provider also may do other tests like heart rate monitoring and tests to check for infections. In some cases, treating health conditions in the mom during pregnancy can help improve a baby’s growth.

Premature birth and fetal growth restriction may be caused by conditions that affect your baby in the womb, like infections and birth defects. Birth defects are health conditions that are present at birth. Birth defects change the shape or function of one or more parts of the body. They can cause problems in overall health, how the body develops, or how the body works. They may limit a baby’s development in the womb, which may lead to low birthweight. Babies with birth defects are more likely than babies without birth defects to be born prematurely.

Risk factors for having low birth weight infants

Scientists know some things may make you more likely than others to have a low-birthweight baby. These are called risk factors. Having a risk factor doesn’t mean for sure that you’ll have a low-birthweight baby. But it may increase your chances. Because many premature babies are born with low birthweight, many risk factors for preterm labor and premature birth are the same as for having a low-birthweight baby. Talk to your health care provider about what you can do to help reduce your risk.

Medical risk factors for having a low-birthweight baby

• Preterm labor. This is labor that starts too soon, before 37 weeks of pregnancy.
• Chronic health conditions. These are health conditions that last for a long time or that happen again and again over a long period of time. Chronic health conditions need treatment from a health care provider. Chronic health conditions that may lead to having a baby with low birthweight include high blood pressure, diabetes and heart, lung and kidney problems.
• Taking certain medicines to treat health conditions, like high blood pressure, epilepsy and blood clots. Tell your provider about any prescription medicine you take. You may need to stop taking a medicine or switch to one that’s safer during pregnancy.
• Infections. Certain infections, especially infections in the uterus (womb) during pregnancy can slow a baby’s growth in the womb. These include cytomegalovirus, rubella, chickenpox, toxoplasmosis and certain sexually transmitted infections (also called STIs, sexually transmitted diseases or STDs).
• Problems with the placenta. The placenta grows in your uterus and supplies your baby with food and oxygen through the umbilical cord. Some problems in the placenta can reduce the flow of oxygen and nutrients to your baby, which can limit your baby’s growth.
• Not gaining enough weight during pregnancy. Women who don’t gain enough weight during pregnancy are more likely to have a low-birthweight baby than women who gain the right amount of weight. If you have an eating disorder or have been treated for an eating disorder, tell your provider. Your provider can check on you and your baby carefully throughout pregnancy to help prevent complications and make sure you’re both healthy.
• Having a premature baby or a growth-restricted baby in the past
• Being pregnant with multiples (twins, triplets or more). More than half of multiple birth babies have low birthweight.

Risk factors in your everyday life for having a low-birthweight baby

• Smoking, drinking alcohol, using street drugs and abusing prescription drugs. Pregnant women who smoke are three and a half times more likely to have an SGA baby than women who don’t smoke. Smoking, drinking alcohol, using street drugs and abusing prescription drugs during pregnancy can slow your baby’s growth in the womb and increase the risk for premature birth and birth defects.
• Exposure to air pollution or lead
• Low socioeconomic status. Socioeconomic status is a combination of things like your education, your job and your income (how much money you make).
• Domestic violence. This is when your partner hurts or abuses you. It includes physical, sexual and emotional abuse.

Age and race as risk factors for having a low-birthweight baby

Being a teen mother (especially younger than 15) or being older than 35 makes you more likely than other women to have a low-birthweight baby. In the United States, black women are more likely than others to have a low-birthweight baby. The rates of babies born with low birthweight each year among different ethnic groups are:

• About 1 in 7 black babies (about 13 percent)
• About 1 in 12 Asian babies (about 8 percent)
• About 1 in 13 American Indian/Alaska Native babies (about 7 percent)
• About 1 in 14 Hispanic babies (about 7 percent)
• About 1 in 14 white babies (about 7 percent)

Scientists don’t know exactly why race plays a role in having a low-birthweight baby; researchers are working to learn more about it.

Low birthweight prevention

Because of the tremendous advances in care of sick and premature babies, more and more babies are surviving despite being born early and being born very small. However, prevention of preterm births is one of the best ways to prevent babies born with low birthweight.

Prenatal care is a key factor in preventing preterm births and low birthweight babies. At prenatal visits, the health of both mother and fetus can be checked. Because maternal nutrition and weight gain are linked with fetal weight gain and birthweight, eating a healthy diet and gaining the proper amount of weight in pregnancy are essential. Mothers should also avoid alcohol, cigarettes, and illicit drugs, which can contribute to poor fetal growth, among other complications.

Characteristics of low birth weight baby

Babies with low birthweight look much smaller than other babies of normal birthweight. A low birthweight baby’s head may appear to be bigger than the rest of the body, and he or she often looks thin with little body fat. A baby with low birthweight may have trouble eating, gaining weight and fighting off infections. Some low-birthweight babies may have long-term health problems, too.

Low-birthweight babies are more likely than babies with normal weight to have health problems as a newborn. Some need special care in a hospital’s newborn intensive care unit (also called NICU) to treat medical problems. These include:

• Breathing problems, like respiratory distress syndrome (also called RDS). Babies with RDS don’t have a protein called surfactant that keeps small air sacs in a baby’s lungs from collapsing. Treatment with surfactant helps these babies breathe more easily. Babies with RDS also may need oxygen and other breathing help to make their lungs work.
• Bleeding in the brain also called intraventricular hemorrhage (IVH). Most brain bleeds are mild and fix themselves with no or few lasting problems. More severe bleeds can cause pressure on the brain that can cause fluid to build up in the brain. This can cause brain damage. In some cases, a surgeon may insert a tube into the baby’s brain to drain the fluid.
• Patent ductus arteriosus (PDA). Patent ductus arteriosus is when an opening between two major blood vessels leading from the heart does not close properly. This can cause extra blood to flow to the lungs. In many babies with patent ductus arteriosus, the opening closes on its own within a few days after birth. Some babies with PDA need medicine or surgery to close the opening.
• Necrotizing enterocolitis (NEC). This is a problem in a baby’s intestines. The intestines are long tubes that are part of the digestive system. The digestive system helps the body break down food. Necrotizing enterocolitis can be dangerous for a baby leading to feeding problems, swelling in the belly and other complications. Babies with necrotizing enterocolitis are treated with antibiotics (medicines that kill infections) and fed intravenously (through a vein) instead of by mouth while the intestine heals. In some cases, a baby may need surgery to remove damaged parts of intestine.
• Retinopathy of prematurity (also called ROP). Retinopathy of prematurity is an eye disease that affects many premature babies. ROP happens when a baby’s retinas don’t fully develop in the weeks after birth. The retina is the nerve tissue that lines the back of the eye. ROP usually affects both eyes. If your baby has ROP, getting treatment right away is really important. The disease can develop very quickly and take away your baby’s vision if it’s not checked carefully by his doctor at recommended checkups after he leaves the hospital. Some babies need treatment with medicine or laser surgery to prevent vision loss.
• Jaundice. This is a condition that makes a baby’s eyes and skin look yellow. It’s caused when there’s too much of a substance called bilirubin in the blood. This happens when the liver isn’t fully developed or isn’t working. Babies with severe jaundice may be treated with light therapy, blood transfusion or intravenous immunoglobulin (also called IVIg). During light therapy, a baby is placed under special lights that help her body change bilirubin into a form she can get rid of in her urine. If this doesn’t work, your baby may get a special type of blood transfusion called an exchange transfusion. During an exchange transfusion new blood is put into your baby’s body to help replace your baby’s blood and get rid of the bilirubin in her blood. If you and your baby have different blood types, your baby may get immunoglobulin (a blood protein) through a needle into a vein. This can help treat jaundice so your baby’s less likely to need an exchange transfusion.
• Infections. The immune system protects your body from infection. A premature baby’s immune system may not be fully developed, which can lead to infections.

Low birth weight complications

A baby with low birthweight may be at increased risk for complications. The baby’s tiny body is not as strong and he or she may have a harder time eating, gaining weight, and fighting infection. Because they have so little body fat, low birthweight babies often have difficulty staying warm in normal temperatures.

Because many babies with low birthweight are also premature, it is can be difficult to separate the problems due to the prematurity from the problems of just being so tiny. In general, the lower the birthweight, the greater the risk for complications. The following are some of the common problems of low birthweight babies:

• Low oxygen levels at birth
• Inability to maintain body temperature
• Difficulty feeding and gaining weight
• Infection
• Breathing problems, such as infant respiratory distress syndrome (a respiratory disease of prematurity caused by immature lungs)
• Neurologic problems, such as intraventricular hemorrhage (bleeding inside the brain)
• Gastrointestinal problems, such as necrotizing enterocolitis (a serious disease of the intestine common in premature babies)
• Sudden infant death syndrome (SIDS)

Nearly all low birthweight babies need specialized care in the Neonatal Intensive Care Unit (NICU) until they gain weight and are well enough to go home. Survival of infants with low birthweight depends largely on how much the baby weighs at birth, with the smallest babies (<500 grams) having the lowest survival rate.

Babies born with low birthweight may be more likely than babies born at a normal weight to have certain health conditions later in life, including:

• Diabetes
• Heart disease
• High blood pressure
• Intellectual and developmental disabilities. These are problems with how the brain works that can cause a person to have trouble or delays in physical development, learning, communicating, taking care of himself or getting along with others.
• Metabolic syndrome. This is caused when you have high blood pressure, diabetes and heart disease all together.
• Obesity. This means being very overweight. If you’re obese, your body mass index (BMI) is 30 or higher.

Talk to your baby’s doctor about what you can do to help your baby be healthy. As your child grows, make sure she eats healthy food, stays active and goes to all her health care checkups. Getting regular checkups throughout childhood can help your baby’s provider spot health conditions that may cause problems as your baby grows older. These checkups also help make sure that your child gets all the vaccinations she needs to stay protected from certain harmful diseases.

If my baby has developmental delays, does she need early intervention services?

Yes. If your baby has developmental delays, it’s important to get early intervention services as soon as possible. Developmental delays are when your child doesn’t reach developmental milestones when expected. A developmental milestone is a skill or activity that most children can do at a certain age. Milestones include sitting, walking, talking, having social skills and having thinking skills. Early intervention services can help improve your child’s development. They can help children from birth through 3 years old learn important skills. Services include therapy to help a child talk, walk, learn self-help skills and interact with others.

How is low birthweight diagnosed?

During pregnancy, a baby’s birthweight can be estimated in different ways. The height of the fundus (the top of a mother’s uterus) can be measured from the pubic bone. This measurement in centimeters usually corresponds with the number of weeks of pregnancy after the 20th week. If the measurement is low for the number of weeks, the baby may be smaller than expected. Ultrasound (a test using sound waves to create a picture of internal structures) is a more accurate method of estimating fetal size. Measurements can be taken of the fetus’ head, abdomen, and femur and compared with a growth chart to estimate fetal weight.

Babies are weighed within the first few hours after birth. The weight is compared with the baby’s gestational age and recorded in the medical record. A birthweight less than 2,500 grams (5 pounds, 8 ounces) is diagnosed as low birthweight. Babies weighing less than 1,500 grams (3 pounds, 5 ounces) at birth are considered very low birthweight.

Low birthweight treatment

Specific management for low birthweight will be determined by your baby’s doctor based on:

• Your baby’s gestational age, overall health, and medical history
• Your baby’s tolerance for specific medications, procedures, or therapies
• Your opinion or preference

Care for low birthweight babies often includes:

• Care in the NICU
• Temperature controlled beds
• Special feedings, sometimes with a tube into the stomach if a baby cannot suck, or through an intravenous (IV) line
• Other treatments for complications

Low birthweight babies typically “catch up” in physical growth if there are no other complications. Babies may be referred to special follow-up healthcare programs.