Category: Cleft and craniofacial

​When a baby spends a lot of time in one position, it can cause the shape of their head to change. This is called a positional skull deformity.

For about 20% of babies, a positional skull deformity occurs when they are in the womb or in the birth canal. More often, it happens in the first 4 to 12 weeks of life. This is when babies are not able to sit up or move on their own. By 6 months of age, many babies become more mobile and can turn their heads on their own more regularly.

What causes a baby’s head shape to change?

A baby’s skull has soft, bony plates that haven’t yet fused together. The bony plates can move a bit, which helps the baby’s head to pass through the birth canal. The plates also allow room for the brain to grow during the first year of life.

There are many things that can cause a positional skull deformity, such as:

• Preferred head position. Some babies like sitting or sleeping with their heads turned a certain way.
• Not enough tummy time. Tummy time is for babies who are awake and being watched. It helps babies master basic milestones like head lifting, turning over, sitting up, and crawling.

• Twin or triplet. Cramped or unusual positions in the womb can lead to changes in head shape.
• Prematurity. Lying flat on their backs in the hospital can cause a preemie’s head to become misshapen. Preemies also have softer bones that are not as fully formed as the bones of full-term babies.
• Birth complications. The baby’s position when moving through the birth canal can affect his or her head shape. Some other things that affect a baby’s head shape at birth are using forceps, vacuum extraction, or even a difficult labor.
• Torticollis. An estimated 85% of babies with torticollis, a condition where the neck muscles are tight or imbalanced, have a positional skull deformity. Infants who have torticollis also will need physical therapy.

3 common misshapen head types

A baby’s head shape change usually is related to the position in which they spend the most time. Your pediatrician can determine whether your baby’s head shape is caused by a positional skull deformity or an uncommon but more serious condition called craniosynostosis. Common positional skull deformities do not require surgery.

• Deformational brachycephaly is when the head is symmetrically (evenly) flat in the back and wide from side to side. The bone above the ears might seem to stick out. This is often seen in babies who spend a lot of time on their backs and do not get enough tummy time.
• Deformational plagiocephaly is when the head is asymmetrically (unevenly) flat in the back on one side. The forehead may be more prominent causing the head to look like a parallelogram. The ear may also shift forward on the flat side. This is often seen in babies who prefer to sleep with their heads turned to one side and those with torticollis.
• NICUcephaly is a common condition in preemies who spend their first few months of life in the neonatal intensive care unit (NICU). This positional skull deformity causes preemies to develop long, narrow heads.

A good time to check your baby’s head is after bath time when his or her hair is wet.

• The back of your baby’s head should be evenly round.
• Your baby’s ears should be even.
• The width of your baby’s head and forehead should be even and balanced.

If you notice any changes or have any concerns, talk to your pediatrician.

What if my baby has a positional skull deformity?

The best treatment is to prevent a positional skull deformity. And when it is found early, simple changes to your baby’s position will help. For example:

• Avoid too much time in a car seat, bouncy seat, baby swing, or other carrier. These positions put pressure on the back of your baby’s head.
• Increase tummy time. It is important to put babies on their backs to sleep. But a baby needs supervised time on their tummy to play during the day. This also lets your baby exercise their neck, back, shoulders, arms and hips. Start with short spurts of time. Gradually work up to an hour per day in several short sessions.
• Switch or alternate arms when holding and feeding your baby.
• Alternate which end you of the crib you place your baby down for sleep.

Positional skull deformities do not affect brain growth or intellectual development. They are purely cosmetic, and the majority do not require surgery.

Early intervention & therapy

In some cases, your baby’s doctor may recommend treatment for positional skull deformity, particularly those with moderate or severe flattening.

Physical therapy. Your pediatrician may refer your baby for early intervention services and an evaluation from a pediatric physical therapist. The therapist will check your baby for delayed motor skills caused by poor head and neck control, and for torticollis. You will also learn stretching and positioning exercises to do with your baby at home. Depending on how severe the positional skull deformity is, your baby may need weekly therapy.

Helmet therapy. If your baby has moderate or severe flattening that does not respond to treatment by 5 or 6 months of age, he or she may need helmet therapy. Molding helmets work by re-shaping the baby’s head and are fitted by a specialist.

Surgery should only be considered when all other options have been exhausted, and after consulting with a neurosurgeon or pediatric plastic surgeon who specializes in these types of cases.

Remember

Talk with your pediatrician if you have any questions or concerns about your baby’s growth and de​​velopment.

It is not unusual for a baby’s head to look a little lopsided. Because the individual bones of a newborn’s skull aren’t yet fused together, pressure from resting in the same position can cause an infant’s head to be misshapen. This may include a flattened area.  

While an asymmetrical head shape is a common cause of concern for new parents, a baby’s head typically rounds out after birth. Flat spots may also improve, especially with position changes and extra tummy time during play. If the deformation is moderate or severe and not responding to position changes, a helmet may help.

Less commonly, an uneven head shape happens when the bones of the skull fuse together too soon. This rare condition, called craniosynostosis, may require surgery both to correct the head shape abnormality and in some cases to give the baby’s brain room it needs to grow. It is estimated that about 1 in every 2,000 U.S. babies is born with craniosynostosis. 

Baby’s head: a brainy ​​design 

The soft, flexible spaces between a baby’s six separate bones of the head, called “sutures,” do more than help the head squeeze through the birth canal.  They also allow the skull to expand rapidly during the first year of life, when the baby’s brain more than doubles in size. 

With craniosynostosis, two or more bones fuse together before a baby’s brain growth is completed—sometimes even before birth. Depending on which of the sutures close early, and where they are located, the shape of the skull, brain and face can become distorted.  

How is craniosynostosis diagn​​osed?

To help determine what’s causing a baby’s uneven head shape, pediatricians will talk with parents to piece together a developmental and family history.  They will also conduct careful examinations, looking at the baby’s head and face from several different angles as they search for raised or flattened areas and noting any asymmetry.  This may be easier when the baby’s hair is wet.

These observations and information will help differentiate positional plagiocephaly (caused by simple pressure in one area) from craniosynostosis (caused by bony fusion).

One sign a baby’s uneven head shape was caused by position alone is the classic parallelogram shape. One side of the head may appear to be pushed forward, with the back of the head being flattened, the ear moved slightly forward, and the forehead pushed slightly forward on the same side compared with the opposite side.

Your doctor may gently place a finger in each ear and note the position of their fingers relative to each other as observed from above the baby’s head. In the case of positional plagiocephaly, the two fingers will not line up opposite each other. Plagiocephaly is more common on the right side, and also shows up more often in male infants.

Types of craniosyno​stosis

Skull bone sutures that fused too soon may be felt as ridges that run along various parts of the baby’s head. The baby’s particular head shape will also point to the specific suture involved.  Some of the more commonly seen types of head shapes, with the involved sutures, include:

• Scaphocephaly: A long, narrow head is common with midline “sagittal” synostosis, when the suture extending from front to back over the top of the head fuses too soon. Sagittal synostosis is the most common form of craniosynostosis, accounting for about 40% to 45% of cases.
• Trigonocephaly: A forehead that is pinched on the sides with a ridge running from the bridge of the nose to the soft spot on top of the head signals a “metopic” synostosis.
• Anterior Plagiocephaly: An asymmetric head and face with a flattened forehead, a raised eyebrow, and a deviated nose can result from “coronal” synostosis on one side.     
• Brachycephaly: A flatted and/or tall forehead, usually with pronounced flattening of the cheeks, a small upturned or “beaked” nose, and bulging eyes from “coronal” synostosis on both sides. This is often present in medical syndromes that involve the face and skull
• Posterior Plagiocephaly: An asymmetric head with a pronounced ridge on the back of the head and a large bump behind the ear may result from “lambdoid” synostosis, which is the rarest form.

Surgery for cra​​niosynostosis

Craniosynostosis usually requires surgery, both to correct the deformity and, in some cases, to avoid pressure inside the skull as the baby’s brain grows. While extra tummy time and “molding” with a helmet can correct positional skull deformities, they are not, in themselves, effective in correcting craniosynostosis (although they are sometimes used after surgery).

Children with craniosynostosis are referred to specialists that include plastic craniofacial surgeons and pediatric neurosurgeons, and other experts who work as a team.   These specialists are often able to make the correct diagnosis by examining your child but will arrange detailed imaging procedures, such as computed tomography (CT scans) ,before recommending surgery.

Depending how old the baby was when diagnosed with craniosynostosis and which suture is involved, different surgical options are available to either remove or release the fused bone. Additional reconstructive procedures may include placing springs or screws in the affected bones and “distraction” devices that slowly separate bone and let the healing process gradually fill it in.

Surgery for craniosynostosis should be performed in medical centers with experienced craniofacial teams and pediatric intensive care units. After surgery, children stay in the hospital one or more days and are monitored at follow-up doctor visits. Complications such as bleeding, infection and bone loss are relatively rare. Children who have these procedures usually grow and develop normally into adulthood.

Babys’ ears play a big role in helping them take in the world around them. They let them hear vibrations as sound, help them learn to talk, and even balance so they can begin to walk. As children get older, ear shape also can play a role in their social confidence.

For babies born with structural abnormalities of the ears, a range of treatment options can help prevent these conditions from affecting a child’s development.

Taking Shape

Of the roughly four million babies born in the United States each year, an estimated 15 percent or 600,000 of them have some abnormally shaped ears. Doctors used to believe that misshapen ears would correct on their own as a baby grew. Current research shows this is not the case, with about 70 percent of untreated ear deformities lasting into childhood.

A better understanding of the cartilage that forms the outer ear, as well as advances in biomedical engineering, have led to more treatment options to correct ear abnormalities in children. Some treatments help a child’s ears work properly, while others are more about making them look better.

For example, surgery can rebuild parts of the ear that didn’t form fully and help a baby hear properly. For other cases, simple molding devices designed for infants to wear can help correct ear shape deformities.

Types of Ear Abnormalities

There are many different types of ear abnormalities, including:

• Prominent Ear: Ear sticking out more than 2 cm from the head
• Constricted (lop/cup) Ear: The outer rim of ear is rolled or folded.
• Cryptotia: When the upper rim of the ear is tucked underneath scalp skin.
• Stahl’s Ear: Pointy shaped ear.
• Microtia: The outer ear is underdeveloped or small.
• Anotia: Missing ear.
• Congenital Earlobe Deformities: Split or double earlobes, sometimes with skin tags.
• Accessory Tragus (ear tag): Bump of skin or cartilage in front of the ear.

Non-Surgical Treatments

Ears that are folded, stick out or are otherwise irregularly shaped can be a source of embarrassment and teasing for young children. It’s often why they may want surgical correction (called otoplasty). However, there are non-surgical treatments that can be done shortly after birth that can avoid the need for corrective surgery, which is often postponed until a child is around five years old and requires an operation under general anesthesia.

Ear Molding. Using custom devices, pediatric plastic surgeons can mold the ears into a more “normal” shape, proportion or position. Generally, the earlier the ear is molded, the more successful the outcome. Less than three weeks of age is considered the ideal time to begin ear molding, though some improvement can still be achieved if started later.

There are several ear molding methods. One of the more common techniques involves placing a soft silicone cradle around the ear and applying plastic retainers to reshape the ear cartilage. The device is held on using tape and is worn for a period of 4-6 weeks. After the molding devices are removed, double sided tape may be placed to hold the position for another 2 weeks. An estimated 90 percent of all ear deformities can be corrected using this non-surgical method.

When Surgery Can Help

Otoplasty. Older children with ear malformations may not benefit much from simple techniques such as ear molding. For these children, surgical options, such as otoplasty, are available. Otoplasty reshapes the outer ear using permanent stitches placed through a hidden scar behind the ear. This procedure is performed as an outpatient surgery in early childhood after the ear has completed most of its growth.

Reconstructive surgery. Ear deformities such as microtia (small ears) may need more complex reconstruction procedures. Microtia reconstruction is often performed in two or three operations by pediatric plastic surgeons. The surgeons use either cartilage from the rib or a synthetic material such as porous polyethylene to build or shape the ears.

Ear reconstruction is usually delayed until a child is 6 to 10 years old when using rib cartilage, and 3 to 5 years old if synthetic material is used. An artificial, prosthetic ear may also be an option. The ear canal may be missing in children with microtia. As a result, these children may benefit from a bone anchored hearing aid (BAHA) to improve the quality of hearing.

Sometimes babies are born with a small lower jaw. It is called micrognathia, which means “small mandible.” It can occur in isolation, but is also a symptom of a variety of craniofacial conditions. Micrognathia may interfere with a child’s feeding and breathing. For example, infants with this condition may need special nipples and positioning in order to feed properly. Outcomes for children with micrognathia are generally good, but can vary depending on the severity of the condition.

Infants

Micrognathia can appear in infants by itself or as part of a syndrome (where more than one body system is involved). A syndrome may be inherited (passed down from the parents) or come about spontaneously.

Pierre Robin Sequence

A small jaw is often seen as part of Pierre Robin Sequence. Pierre Robin occurs in about one per 8,500 live births. It is called a “sequence,” because while the baby is growing in the womb, the lower jaw does not grow enough. This causes the tongue to be pushed back and the palate bones to remain separated in the middle. Babies born with Pierre Robin may have difficulty with breathing, feeding, and/or sleeping. It can be a very mild problem to quite severe.

• Babies born with mild forms of Pierre Robin will be discharged home with close follow up in the pediatrician’s office. Parents are advised to keep a close watch on their baby due to partial airway blockage associated with obstructive sleep apnea. These infants may also have trouble feeding and suffer from gastrointestinal reflux.
• Babies born with more severe forms Pierre Robin can’t leave the hospital after birth and need extra measures to protect their breathing. A breathing tube attached to a ventilator may be needed temporarily.

Obstructive Sleep Apnea

The airway is at the most risk for blockage at night when all the muscles are relaxed. When a child’s tongue is in a backward position due to a small jaw, his or her attempts to breath when sleeping can be met with partial or complete blockage. This is called obstructive sleep apnea. It occurs in about 2% of children.

Obstructive sleep apnea causes decreases in the blood’s ability to carry oxygen to the developing organs, so it can interfere with development. In children with obstructive sleep apnea and micrognathia, surgical treatment of the airway can be an emergency.

Treatment of Micrognathia

Early diagnosis and ongoing monitoring help with a multi-disciplinary team of specialists will determine the best time to medically or surgically intervene. If your baby has micrognathia, you may meet with:

• A pediatric otolaryngologist (ENT)
• A craniofacial plastic surgeon
• A pediatric geneticist
• A pediatric pulmonologist

If the child can tolerate it, a sleep study is done to see how bad his or her airway is obstructed. If the results are severe, then a formal airway evaluation by the pediatric ENT is done in the operating room to assess the anatomy of the upper airway.

Mandibular Distraction Osteogenesis

Mandibular Distraction Osteogenesis (MDO) is a surgical procedure that makes the lower jaw larger by slowly lengthening the lower jaw bone.  The surgeon makes cuts on both sides of the lower jaw and attaches turning devices which expand the space in the bone cut. By turning the devices, the jaw moves forward (and with it, the tongue). The airway will then get bigger, and within a couple of weeks from the surgery, the breathing/sleeping problem will be improved.

Risks for MDO surgery include damage to tooth buds, sensory nerve damage, facial nerve damage, relapse of the original condition, and infection. After 6 weeks, the new bone has formed and the devices are removed. Usually, the child can go home during this period of bone formation. Additional benefits of MDO are improved feeding, decreased reflux, and avoidance of a permanent breathing tube called a tracheostomy.

Childhood Concerns

Regardless of whether or not a child undergoes MDO as an infant, he or she will need to be followed for:

• Jaw growth
• Dental (tooth) development
• Lower lip sensation
• Airway health

Sometimes, a second (or even first) MDO is needed during childhood if breathing and sleep apnea problems persist. Many children born with micrognathia will eventually need orthodontic care, as well. Some eventually require corrective jaw surgery after growth is complete in adolescence.  

​Head tilt is a condition that causes a child to hold her head or neck in a twisted or otherwise abnormal position. She may lean her head toward one shoulder and, when lying on her stomach, always turn the same side of her face toward the mattress. This can cause her head to flatten on one side and her face to appear uneven or out of line. If not treated, head tilt may lead to permanent facial deformity or unevenness and to restricted head movement.

Most cases of head tilt are associated with a condition called torticollis, although in rare instances a head tilt can be due to other causes such as hearing loss, misalignment of the eyes, reflux (a flowing back of stomach acid into the esophagus), a throat or lymph node infection, or, very uncommonly, a brain tumor.

Acquired torticollis (due to injury or inflammation)

This is more likely to occur in older children, up to the age of nine or ten. This type of torticollis usually results from an inflammation of the throat caused by an upper respiratory infection, a sore throat, an injury, or some unknown factor. The swelling, for reasons still not known, causes the tissues surrounding the upper spine to loosen, allowing the vertebral bones to move out of normal position. When this happens, the neck muscles go into spasm, causing the head to tilt to one side. Onset of this condition is typically sudden and very painful.

Congenital muscular torticollis

By far the most common cause of head tilt among children under age five is congenital torticollis. This condition commonly occurs due to positioning while the baby is still in the womb and rarely may occur during birth (particularly breech and difficult first-time deliveries).Whatever the cause, this condition usually is detected in the first six to eight weeks after birth, when the pediatrician notices tightness in one of the muscles of the neck. About half the time there may be a small lump in the muscle. The affected muscle is the sternocleidomastoid muscle, which connects the breastbone, head, and neck. Later the muscle contracts and causes the head to tilt to one side and look toward the opposite side.

Treatment

Each type of head tilt requires different treatment. It is very important to seek such treatment early, so that the problem is corrected before it causes permanent deformity. Your pediatrician will examine your child’s neck and may order X-rays of the area in order to identify the cause of the problem. X-rays or ultrasound of the hip also may be ordered, as some children with congenital muscular torticollis also have an abnormality known as developmental dysplasia of the hip.

If the doctor decides that the problem is congenital muscular torticollis, you will learn an exercise program to stretch the neck muscles. The doctor will show you how to gently move your child’s head in the opposite direction from the tilt. You’ll need to do this several times a day, very gradually extending the movement as the muscle stretches.

When your child sleeps, it is best to place her on her back with her head positioned opposite to the direction of the tilt. In rare instances, your pediatrician may suggest adjustments to her sleep position. When she is awake, position her so those things she wants to look at (windows, mobiles, pictures, and activity) are on the side away from the injury. In that way, she’ll stretch the shortened muscle while trying to see these objects. Your pediatrician may also recommend placing her on her stomach while awake and turning her face away from the affected side.

These simple strategies cure this type of head tilt in the vast majority of cases, preventing the need for later surgery. (Your pediatrician may refer your child to a physical therapist to help work on this condition.)

If the problem is not corrected by exercise or position change, your pediatrician will refer you to a pediatric neurologist or orthopedist. In some cases, it may be necessary to lengthen the involved tendon surgically. If your child’s head tilt is caused by something other than congenital muscular torticollis and the X-rays show no spinal abnormality, other treatment involving rest, a special collar, gentle stretching, massage, traction, application of heat to the area, medication, or, rarely, further imaging or surgery may be necessary.

For treating torticollis due to injury or inflammation, your doctor may recommend applying heat, as well as using massage and stretching to ease head and neck pain. Your pediatrician can refer you to a specialist for a definitive diagnosis and treatment program.

​Having prominent ears may negatively affect a child’s self-image because he looks different and may be teased by peers. This can lead to poor development of interpersonal relationships, social withdrawal, and even depression.

For minor degrees of deformity, no intervention may be needed.

Surgical Correction

For severe degrees of congenital deformity or birth defect, otoplasty (surgical correction) is warranted in children who are not at excessive risk of under anesthesia and surgery. If the auricle (outer projecting portion of the ear) is 85% of adult size by the time a child is 4 years old, otoplasty can be considered. The procedure is not very painful or risky and generally yields good results, although occasionally, revision surgery is needed.

Insurance Coverage for Otoplasty

Unfortunately, health insurance companies frequently consider otoplasty to be a cosmetic procedure and will not provide payment for the operation. Check your plan for specifics.

​All people have asymmetric faces. When one looks closely, these differences become more apparent. However, there are conditions in children in which the normal minor differences are much more significant.

Craniofacial Microsomia 

Children born with this congenital condition have one half of the face that didn’t develop as well as the other. It occurs during fetal development. 

Common affected areas:

• Ear: Anything from a change in shape to an absent ear
• Ear canal: May be absent with decreased hearing as a result
• Lower jaw (mandible): Less growth, chin point to side of decreased growth, and abnormal bite
• Facial movement: A single nerve (facial nerve) with many branches controls facial movement, and one or more of these may be affected
• Eye socket and/or eye: May be small or absent. Whatever the child is born with, he/she will maintain, but it will not get worse with growth.

These children are cared for by a craniofacial team, including a pediatric plastic surgeon, ENT surgeon, speech therapist, dentist, and orthodontist. Surgery may be needed to help the jaw grow and to reconstruct the ear. Why this condition occurs is not fully understood, although genetic and environmental factors are both likely.

Congenital Facial Nerve Palsy

On each side of the face, there is one main facial nerve with several branches that travel to the muscles of facial expression. Children can be born with a deficiency of this nerve, usually on one side. Sometimes both nerves are affected. This is a rare problem.

The causes of congenital facial nerve palsy are usually birth trauma or developmental. 

• ​Developmental: When one side is affected, it is typically seen as muscular weakness, with difficulties raising the eyebrow, closing the eye, feeding difficulties, or smiling on the affected side. A bilateral (both sides) form of this problem is known as Mobius Syndrome. Mobius syndrome is a congenital paralysis of the muscle of facial expression, usually due to deficiency of the facial nerve as well as one of the nerves controlling movement of the eye.
• Birth trauma: 90% of the cases related to birth trauma will improve without treatment. If it does not go away spontaneously, then based on the location of the nerve damage, a surgical procedure called a neurorrhapy (stitching nerve ends together) or nerve graft may be performed to restore normal nerve function.

​Aside from the inability to communicate to others with facial expression, children with facial nerve weakness/paralysis may have difficulty closing the eye on the affected side, and thus have eye irritation and/or corneal ulceration. Prolonged exposure can lead to infection or loss of vision.​

There are two forms of treatment:

• Eye protection: A small weight is surgically placed into the upper eyelid on the affected side. This will make it easier to close the eye, with the goal of keeping the cornea protected, and the eye free of infection.
• Smile recreation: The ability to smile can be restored either by transferring one of the surrounding muscles of the face to the corner of the mouth, or by implanting a new muscle taken from another part of the patient’s body. With new muscle, arteries, veins, and nerves must be reconnected under a microscope to restore function. The nerve that powers the new muscle comes either from a chewing muscle on the same side of the face or from a normal facial nerve branch on the patient’s other side.

Acquired Facial Nerve Palsy

Children can develop facial nerve weakness/paralysis during life due to trauma, tumors, or from a third condition called Bell’s palsy. Bell’s palsy is thought to result from a viral infection that affects the facial nerve, and therefore often results in a unilateral (one-sided) facial paralysis. With time, the nerve can regain function. Many children get full function back, while others regain only partial function. In the case of no return of function, treatment can be offered as described above for congenital cases.

Of the 1% to 2% of babies born with congenital defects, 10% are born with malformations to the hand. These anomalies occur in early pregnancy and are sometimes diagnosed by ultrasound during pregnancy. When they are not, they often come as a surprise to parents. The cause of congenital hand anomalies is unknown.

Depending on the type and extent of a hand malformation, some babies may have little trouble adapting and functioning well. Others, however, may face various challenges as they grow and learn: 

• Developmental problems such as delayed or deficient motor skills
• Difficulties with activities of daily living activities and basic self-care skills
• Limitations on certain types of exercises and sports
• Potential emotional and social harm from childhood teasing about appearance

If child is functioning well and is happy, treatment is not always necessary. If a child is having difficulty doing the things he or she wants to do because of a hand malformation, treatment options may be available.

Treatment Options

The most important goal of any treatment for hand malformations is to help a child function as independently as possible. Your pediatrician will often provide referrals to pediatric plastic surgeons or pediatric orthopedic surgeons. Treatment may involve:

• Orthotics (splints or braces)
• Prosthetics (artificial limbs)
• Physical therapy
• Surgery

Types of Hand Malformations

Pediatric plastic and orthopedic surgeons diagnose and treat children with all types of hand malformations. There are many types. The following are those most often seen.

Extra Digits (Polydactyly)

Polydactyly is the most common congenital hand deformity. It affects boys and girls equally. A baby born with polydactyly has more than five fingers on one hand. An extra finger is often a small piece of soft tissue that can be simply removed. Sometimes, the extra finger contains bones but not joints. Very rarely, the extra finger is a fully functioning digit. A baby may be born with several extra fingers. There are different types of polydactyly:

• Preaxial polydactyly: Involves having an extra thumb. It is more common in Caucasians. A pediatric surgeon can remove the extra bone and skin and repair the tendons.
• Postaxial polydactyly: Involves having extra fingers on the opposite (the “pinkie” side). It is more common in African-Americans. These may occur as complete, separate fingers or just as small stumps. Stumps can be treated by a hand specialist in the office.
• Central polydactyly: Extra fingers can be located between center fingers, although this is less common.

Fused Fingers (Syndactyly)

Syndactyly is one of the most common birth defects of the upper limbs—seen in as many as 1 in every 2,000 live births. This condition occurs when two or more fingers fail to separate when a baby is in the womb—resulting in “webbed” fingers at birth. It usually involves the middle and ring fingers.

Syndactyly affects twice as many boys as girls. There is often a family history, and these cases often involve both fingers and toes.

Surgery is generally needed before a child is 18 months old to separate the fingers or toes and may require extra skin to be taken from a separate area on the body, depending on the extent the fingers were joined. Categories of the condition include:

• Complete syndactyly: The skin is fused all the way to the tip of affected fingers or toes.
• Incomplete syndactyly: The fingers or toes are joined only part of the way to the tips.
• Simple syndactyly: The fingers or toes are joined by skin and soft tissue only.
• Complex syndactyly: The bones of fingers next to each other are fused. 

Underdeveloped Hand (Symbrachydactyly)

Babies born with symbrachydactyly have small or missing fingers. They also may have webbed fingers or a short hand or forearm.

• Mild symbrachydactyly: The hand has slightly short, mobile fingers with minor webbing. The hand bones and some of the finger bones and the thumb are present.
• Moderate symbrachydactyly: Most or all of the finger bones are missing, and the baby has small projections of skin and soft tissue. The thumb is usually present, but it may be short.
• Severe symbrachydactyly: The baby has either a partial thumb or no thumb and no fingers.

Club Hand

Babies born with club hand are partially or completely missing one of the two long bones that make up the forearm—the radius or the ulna. As a result, the forearm may be shorter than normal. The hand turns inward, causing limited range of motion at the wrist. A child with the condition may have trouble performing tasks that require his or her hands.  The condition is usually more prominent on one side of the hand or the other—the radial (thumb) side or the ulnar (little finger) side.

• Radial club hand: Children with club hand on the radial side (radial dysplasia) often have a short forearm and wrist curved toward the thumb side. Once a baby is between 6 and 12 months of age, surgery may be recommended to straighten the forearm bone and to fix the tendons. The condition may also be related to other medical syndromes such as Fanconi anemia, Holt-Oram syndrome, and VATER syndrome.
• Ulnar club hand: This condition is less common than radial club hand and is usually unrelated to other syndromes. It can range from mild to more severe cases with the baby’s wrist to be in a fixed and bent position toward the little-finger side of the hand. The thumb by be deformed or absent.

Cleft Hand (Ectrodactyly)

Cleft hand (also called ectrodactyly or split hand) is when the middle part of the hand develops abnormally. Babies with this condition are missing one or more central fingers on the hand. There are generally two types:

• Typical cleft hand: V-shaped and usually missing or partially missing middle fingers. Both hands are usually affected, and sometimes the feet are, too. There is often a family history of this type of cleft hand.
• Atypical cleft hand: U-shaped and usually involves only one hand. This type of cleft hand is not often inherited. Atypical cleft hand may be a symptom of Poland syndrome.

Small (Hypoplastic) Thumbs

A hypoplastic thumb did not fully form in the womb or was missing altogether at birth. Treatment depends on how secure or stable the joint is that connects the base of the thumb to the wrist.

Children with stable joints tend to use the thumbs in regular play. Children whose have unstable joints tend to ignore the thumb.

For stable thumb-wrist joints, reconstructive surgery is generally recommended to build up the thumb and its tendons. For loose or floating unstable joints, the small thumb is removed and the index finger is converted to a thumb.

Trigger Thumb

Sometimes babies are born with an abnormality of the tendon in the thumb that makes it difficult for it to bend it. This condition may disappear on its own by about age 1. Other times, surgical correction may be recommended before age 3 to release the tendon and allow for the thumb to properly function.​

One in every 700 babies is born with a cleft lip, a cleft palate, or both—making it one of the most common birth defects.

Babies born with a cleft lip and/or palate need special care from a team of different health professionals. Their care must be well managed because of the difficult medical, surgical, dental, and social factors important to treatment decisions. To help pediatricians better manage children with cleft lip/palate, the American Academy of Pediatrics (AAP) published the clinical report, “The Primary Care Pediatrician and the Care of Children with Cleft Lift and/or Cleft Palate.“

The following information explains these recommendations to parents and families involved in caring for a baby born with a cleft lip and/or palate.

Different Types of Cleft Lip and/or Palate:

The type of cleft lip and/or palate a child has will determine the kind of care that he or she needs.

• A cleft lip means that there is a gap in the baby’s lip. The cleft may be on one or both sides of the upper lip and may reach all the way to the nose. Often, a baby with a cleft lip may have a gap in the upper gum.
• A cleft palate has a gap in the roof of the mouth that connects to the nose.
• The combination of a cleft lip with a cleft palate is more common than just a cleft lip or just a cleft palate. ​

Detecting Cleft Lip and/or Palate:

Many, but not all, parents know in advance that their newborn infant will have a cleft lip from the prenatal ultrasound. The mother’s doctor or the baby’s future doctor can help parents meet with experts—called a cleft/craniofacial team. Prenatal consultation involves parents meeting various members of the cleft/craniofacial team and learning about the care of children born with cleft lip.

Normally, a cleft palate is not seen until after a baby has been born. A nurse, a doctor, or a parent may notice that the top of the mouth looks different. The cleft palate may involve most or just the farthest part of the roof of the mouth. 

Common Concerns from Parents:

Being the parent of a newborn with a cleft lip, cleft palate, or both can feel overwhelming. Simple things like feeding your baby might now be more complicated. Parents wonder how people may react when they see their baby has a cleft lip. Parents also worry how the cleft lip or cleft palate will affect their child long-term—socially and developmentally.  

If you are worrying about any of these things or others, talk with your child’s pediatrician. He or she can help answer questions and even direct you to a parent support group near you. 

Feeding a Baby with Cleft Lip and/or Palate:

In general, babies with a cleft palate either as cleft lip with cleft palate or cleft palate alone, suck weakly and need a special bottle to feed. Support from a feeding therapist, certified lactation consultant, and/or nurse experienced in feeding children with cleft palate is recommended for parents.

A baby born with a cleft lip without cleft palate may also need extra help with feeding. However, most babies with cleft lip can feed from the breast or a normal bottle. This is also true for babies with cleft lip and cleft gum.

Care and Treatment from the Cleft/Craniofacial Team:  

Various specialists will care for your child at different times and stages. The cleft/craniofacial team usually includes nurses, social workers, nutritionists, audiologists, speech-language pathologists, geneticists, pediatricians, dentists, orthodontists, and pediatric surgeons (otolaryngologists, oral and maxillofacial surgeons, and plastic surgeons). These providers understand common concerns and know the answers to parents’ questions.  

• First Appointment: A newborn should see his or her cleft/craniofacial team within one week (or as soon as possible) from the day he or she first leaves the hospital. In the beginning, the team will make sure the baby is feeding and growing well. The team will also check for other medical problems common in babies with a cleft lip or palate, answer parents’ questions, and discuss the next steps for treatment.
• Surgery: Surgical repair of a child’s cleft is one part of the care that the cleft/craniofacial team provides. ​

When Will My Child Need Surgery?

The most common question parents have is, “When will my child have surgery to repair his or her cleft lip or cleft palate?”  The AAP recommends initial reconstructive surgeries for cleft lip or cleft palate occur within the first year of life.

• Cleft lip repair: A cleft lip usually is repaired between 3 and 6 months of age. Many things will determine the right time for surgery; the right time for one child may not be good for another.
• Cleft palate repair: A cleft palate is commonly fixed between 9 and 14 months of age. If there is a separation in the gum line, it usually is repaired when a child is 8-10 years of age. A child’s cleft/craniofacial team will determine the best time for cleft palate surgery.
• Every child is different: Children with cleft lip and/or cleft palate often need additional surgeries and treatments as they grow. For example, they may need braces during childhood. Each child will need different numbers and types of surgeries—why individualized coordination of care is so important. Most children born with clefts will need regular visits to their cleft/craniofacial team until they are adults.​

What Does a Cleft Look Like Before and After Surgery?

It is common for parents to worry about how their child will look before and after the cleft lip and/or palate repair. 

• Below are pictures of a child before surgery and immediately after.

• ​​Below are pictures of a child before surgery and once he has completely healed. 

​​​​​​​​​Babies grow fast, and their heads are made for this quick growth.

Flexible skulls can protect a baby’s growing brain, which roughly doubles in size during the first year. But if a baby’s skull shape seems different, he or she may need helmet therapy.​

What is helmet therapy?

Helmet therapy (also called helmet orthosis) is a treatment that’s prescribed to help mold the baby’s skull into shape.

Babies skulls are made of many bony plates. The plates are joined by fiber-like pieces called sutures. Sutures help the skull protect the baby’s brain. They also allow some flexibility and space so that the brain can grow.

To work, molding helmets must be worn during the time when the skull and brain are actively growing. Helmets will not help after brain growth is done, and three-fourths of brain growth happens by age 2.

What conditions cause a baby to need a helmet?

• Positional skull deformity. Deformational plagiocephaly, brachycephaly and NICUcephaly are three conditions that cause a baby’s skull shape to change. They are caused by pressure on the skull when an infant spends a lot of time in one position.
• Craniosynostosis. This condition is caused when bony plates in the skull are abnormally fused together. As the brain grows in a child with craniosynostosis, the shape of the head becomes abnormal as the brain pushes out on the other parts of the skull. In some cases, the brain growth may be affected, as well. Craniosynostosis requires surgery and may be followed by helmet therapy. Some children with craniosynostosis may have other developmental needs.

Is helmet therapy the only treatment for a child with a positional skull deformity?

It depends. If the condition is not severe, changing your baby’s usual positions or starting physical therapy may help.  If an infant has moderate or severe positional skull deformity that doesn’t respond to changing positions, or if the baby is older, helmet therapy may be prescribed.

A positional skull deformity does not affect a child’s brain development or cause any other medical condition. But not correcting it may affect the child’s social well-being when he or she is older.

Physical therapy and stretching and strengthening exercises are especially helpful for babies with torticollis, which is present in about 20% of babies with positional flattening. This occurs when the baby’s neck muscles are tighter and more contracted on one side. Torticollis causes the baby’s neck to turn in a twisted position, which makes the baby’s head to tilt one way and the chin to point the other way.

What is the first step to get a helmet?

A pediatrician may recommend a helmet after evaluating your baby’s head shape. A referral is to someone who is trained to properly measure and fit the helmet is necessary.

What can I expect during helmet therapy?

A baby’s head shape is measured, and a custom-fitted helmet is designed. This is so the helmet can properly support your baby’s skull while allowing the head to gradually grow and round out on its own.

Babies usually wear their helmets for 23 hours each day. Most children quickly get used to wearing them. The helmet should be taken off when it’s time for your baby’s bath. It should be cleaned at that time. Problems such as skin irritation, discomfort, and bad odor should not occur. If there are problems, the helmet should be adjusted by the specialist who made it.

At what age should a baby with a positional skull deformity begin wearing a helmet?

• A baby that starts helmet therapy at a younger age may wear the helmet for less time. The results of therapy also may be better.
• Babies referred for helmets at a later age (e.g., after 8 months), or after position changes and physical therapy did not help can still get helmets. However, they may have to wear them for a longer time than if they had started at a younger age.

How long does helmet therapy take?

Every child is different. A lot depends on the child’s age when he or she started helmet therapy. In early infancy, a baby’s brain and skull grow very quickly. This means that the helmet can direct growth in less time.

The specialist will check your child’s progress at each visit to see if the head shape is improving. Children may need to wear a helmet for several months. Some children may benefit from more time and a second helmet.