Cleft and cranial conditions

HCA Midwest Health - May 15, 2023

Abnormal head shape in children

A baby’s skull is made of several bony plates loosely joined by areas called sutures. Sutures are the locations where new bone grows as the skull develops. This special design allows the baby’s skull to mold and pass more easily through the birth canal. After birth, a baby can have a cone-shape or uneven appearance to the skull. Typically, this is not a cause for concern and the rapidly growing brain will help normalize the shape of the head. The unfused area between the bony plates allow for the skull to expand with the growing brain. Over time, these areas fill in and solidify so that once separated plates fuse together. Sometimes the head shape does not normalize which causes concern.

What causes abnormal head shape in children?

When the skull shape does not normalize or becomes more uneven as a baby grows, an evaluation is appropriate. Prolonged external pressure causes the most common abnormal head shapes in infants and leads to flat spots on the skull called ‘deformational plagiocephaly’ or ‘positional plagiocephaly’. Another less common cause is ‘craniosynostosis’, the premature closure of the sutures between the bones which prevents the skull from expanding.

Positional plagiocephaly is the flattening of one or both sides of the skull from repeated or prolonged pressure on the back of the head. The recommendation to put infants to sleep on their backs to decrease risk of sudden infant death has resulted in more infants with pressure on the back of their head as they sleep at night. Other infants have a problem called torticollis where the muscles on one side of the neck do not move freely. This can cause the baby to always tilt their head or strongly favor looking to one side. When the baby lays down, their head continues to stay tilted which can lead to flattening. If this pressure is not relieved or changed in some way, the skull may eventually remain in this abnormal shape.
Craniosynostosis is the early fusion of one or more of the sutures between the skull bones which leads to problems with normal brain or bone growth. By preventing normal growth in one area of the skull, the growing brain causes overgrowth in other areas which can lead to predictably uneven head shapes. We do not understand the cause of craniosynostosis completely. Some believe the cause is prolonged abnormal positioning inside the womb or a localized growth abnormality in that part of the skull. In other cases, an unknown familial or genetics factor may cause the condition. There are several well-described genetic syndromes that include craniosynostosis as a feature. These conditions include Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, Saethre-Chotzen syndrome and Muenke syndrome.

What could an abnormal head shape look like?

Positional plagiocephaly, also called ‘slanted’ head, gets its name from the skull’s appearance. Looking from one side or both sides of the head, the back of the head looks slanted. Looking from the top the head, the head’s shape looks slanted into a diamond-type shape.By laying too long in one position that area of the head flattens and the opposite area bulges in response and leaves an uneven skull shape. This can cause the back of the skull to look flat and the opposite forehead to look prominent. The ear position can also be different as a result. Despite the abnormal shape of the head, these infants typically do not have problems with their brain or developmental differences.

Craniosynostosis is usually apparent as an abnormal head shape at birth or shortly after. Restricted skull growth also leads to problems such as elevated brain pressures, developmental delays and changes in the growth and position of facial bones. Increasing pressures inside the head can eventually cause irritability, nausea, vomiting, headaches, decreased appetite, eye abnormalities and increased sleepiness. The abnormal head shape of craniosynostosis takes different forms depending on which suture is affected:

Sagittal suture: The sagittal suture runs in the midline from front to back. The most common type of single suture craniosynostosis (55-60 percent) occurs with early fusion of this suture. Early closure creates a very narrow and elongated shape of the skull termed ‘scaphocephaly’.
Metopic suture: The metopic suture runs beneath the middle of the forehead. The second most common type of single suture craniosynostosis (20-25 percent) occurs with early fusion of this suture. Early closure creates a triangular shape of the forehead known as ‘trigonocephaly’. This can create closely spaced eyes (hypotelorism) and widening of the back of the head.
Coronal suture: The coronal suture runs side to side towards the front of the head. When one of these sutures is affected (‘unicoronal synostosis’), there is flattening of the forehead on the affected side and bulging on the non-affected side. The conditions raises the eye socket on the affected side and the tip of the nose turns away. When both coronal sutures are affected (‘bicoronal synostosis’), there is a tall towering forehead and shortened skull in a front to back direction.
Lambdoid Suture: The lambdoid suture runs side to side along the back of the skull. Early fusion of this suture is the rarest type of synostosis (3-5 percent), and can be mistaken for the simpler, positional plagiocephaly. The primary difference includes downward displacement of the ear with a bulge in the back of the skull.

How do you diagnosis the cause of an abnormal head shape?

When an infant has an abnormal head shape, the next step is an evaluation conducted by someone comfortable with the different underlying causes and typical head shapes associated with each. When physicians suspect craniosynostosis, getting an evaluation by a neurosurgeon or craniofacial surgeon is essential. They will perform a detailed physical exam to identify craniosynostosis features like sutural ridging and characteristic facial differences. X-ray imaging may be ordered to help show any fused suture(s).

How do you care for a child with an abnormal head shape?

Treatment for positional plagiocephaly initially involves focused repositioning of the baby’s head. By relieving the repeated pressures, the skull growth and shape can normalize over time. As the baby grows and gets stronger and starts to turn over or sit up, they naturally decrease the time spent on their back. Awake ‘tummy time’ is important for young infants before they reach this stage. When the head shape does not improve or show growth, then doctors recommend molding helmet therapy to help reshape the head.

For babies diagnosed with craniosynostosis, surgery is the primary treatment. Surgery helps correct the head shape while also decreasing the elevated brain pressures and creating room for the brain to grow and develop normally. A craniofacial surgeon and neurosurgeon typically work together to perform the procedure. These specialists may recommend different surgery options depending on the type of craniosynostosis, the age of the child and the associated symptoms.

Cleft lip and cleft palate

A cleft lip is a condition seen at birth where a child has a split in the skin and muscle of the upper lip. The split can extend in any length including into the base of the nose. Often, the split also involves the gumline just behind the lip.

A cleft palate is also a birth abnormality where the split occurs in the roof of the baby’s mouth. It can extend in any length, from the soft palate near the back of the throat to the gumline at the front of the mouth. A cleft lip or palate can occur by itself or in combination.

What causes cleft lip and cleft palate?

A combination of environmental and inherited factors can prevent complete formation of the lip or mouth tissues early in pregnancy. Approximately 1 in 750 babies are born with some type of clefting. Sometimes, clefting results when a child inherits their condition from one or both parents, but other factors such as medications, drugs or dietary deficiencies can also increase the risk for clefting. Often, we do not know the exact cause. If a baby is born with a cleft and also has other birth differences, then physicians may recommend genetic testing to see if a specific genetic reason can be determined.

What are the effects of a cleft lip and cleft palate?

The effects of a cleft condition depends on the severity of the split in the tissue. An infant with a cleft lip may have difficulty latching to the breast or bottle for feeding in infancy. Some simple feeding change may be all the adjustment needed to help a baby feed effectively. A cleft lip also creates a visible difference in facial symmetry with the lips and nose. As a child grows, an unrepaired split can also cause some difficulties with speech.

The effects of a cleft palate include difficulty feeding and normal speech development. An infant with a cleft condition may also have difficulty latching onto or extracting milk from the breast or bottle at feeding. These feeding challenges, if not addressed, could lead to malnutrition and poor weight gain. There are some specialized bottle and feeding interventions that can help a baby feed effectively. As the infant grows, an unrepaired split in the roof of the mouth can also cause difficulties with speech and hearing.

How do you care for a child with a cleft lip or cleft palate?

If your child was born with a cleft condition, the ideal care setting is a clinic with a ‘cleft team’ where a group of surgeons, nurses, genetic counselors, speech and feeding therapists and many other experts come together to provide cleft-related care. By working together, your child’s cleft team can provide the most efficient and coordinated care. The Kansas City Cleft and Craniofacial Care Program housed at Overland Park Regional Medical Center offers this type of comprehensive care for children and adults.

Will my child need surgery?

We typically recommend surgery to repair cleft conditions. Repairing a split in the lip will creates a more typical lip appearance and motion. There are rare instances where surgery is not an option for treatment. Surgery most often happens when the infant is 3 months to 6 months of age.

Our physicians recommend surgery for infants born with a cleft palate to repair the mouth and help the tissue function more normally for speech and hearing. Cleft palate surgery most often happens between 9 months and 15 months of age. Timing for surgery depends on the baby’s growth and overall health.

Cleft lip and cleft palate repairs are surgeries performed under general anesthesia. Typically, a baby will stay overnight in the hospital after surgery to make sure they are not in pain and that they are able to resume feeding well. After a cleft lip repair, infants resume their typical feeding routine from the breast or bottle shortly after surgery. After a cleft palate repair, infants may be on an altered diet for several days to ensure proper healing. Overall, babies heal relatively quickly after surgery.

Do you also care for adults with a history of cleft lip or cleft palate?

Living with a cleft condition may be a life-long endeavor. The challenges for a child born with a cleft abnormality may be significant and require different amounts of care over time. Often cleft care providers focus on managing these challenges during childhood. Once these children become adults, they may still have medical needs. Their medical needs may involve speech, feeding, dental care, breathing or even cosmetic appearance.

If you are an adult with a cleft condition and ongoing medical concerns, we are happy to provide your continued care and help address your concerns. Adults with cleft conditions deserve the same thoughtful, coordinated care that children receive.

Pierre Robin Sequence

Pierre Robin Sequence (PRS) is a condition in which a child is born with a small lower jaw that causes the tongue and other oral tissues to be pushed backwards in the mouth. This can lead to serious breathing and eating difficulties depending on the size of the jaw and the position of the baby’s tongue. Frequently, these infants also have a large split in the roof of the mouths, also called a cleft palate. Pierre Robin Sequence is named for the doctor who first described the problem in detail.

What causes Pierre Robin Sequence?

It is unknown why babies with PRS develop the condition. Some experts think that compression on the baby or limitation of motion in the womb may prevent the lower jaw from growing normally. Physicians often see cleft palate with PRS because the pushed-back positioning of the tongue may physically get in the way of proper formation of the mouth’s roof during early fetal development. Specialists see Pierre Robin Sequence as an isolated birth difference but they also see it in combination with several known genetic syndromes.

What does an infant with Pierre Robin Sequence look like?

Infants with PRS have small lower jaws and underdeveloped chins or marked underbites.

What are the effects of Pierre Robin Sequence?

If the lower jaw is too small to accommodate the tongue, the baby may struggle because the tongue partially or completely blocks the airway when they are laying down or trying to eat. This would mean the baby has difficulty breathing well, has lower oxygen levels and may not be able to eat enough to gain weight. In milder forms of PRS, the baby may only require special positioning to allow the tongue to stay out of the airway during sleeping and feeding. When the condition is more severe, the baby may need to have specialized breathing support or urgent surgery to help reposition the lower jaw.

How do you care for a child with Pierre Robin Sequence?

If your child has a small lower jaw or chin at the time of birth, PRS may be the diagnosis. This finding combined with a cleft palate or symptoms like snoring or gasping for air while eating will prompt medical caregivers to closely look at the baby’s oxygen levels and weight gain. These babies often have more difficulty breathing when lying flat on their backs. Turning the child onto their stomach or on their side may help relieve any tongue-related airway obstruction. If there are continued challenges with feeding or breathing despite positioning, then your doctor may discuss surgical options.

Will my child need surgery?

It is sometimes hard to predict which infants with PRS will benefit most from surgery. As mentioned above, some infants do well with simple non-surgical interventions and will outgrow their challenges as they get bigger. If an infant continues to struggle with breathing or feeding despite conservative interventions, then physicians may perform surgery before the baby goes home after delivery. The most common surgery is one that expands the lower jawbone to improve its position and make more room in the front of the mouth for the tongue. Sometimes a baby will require a tube placed in the neck called a tracheostomy to allow for improved breathing. If the infant also has a cleft palate,, then the care team refers the baby to the cleft team for follow-up after discharge to manage any additional issues including scheduling cleft palate repair near their first birthday.

The cleft and craniofacial team at HCA Midwest Health treats patients with congenital (present at birth) and acquired abnormalities of the head and neck. Whether the diagnosis of a cleft or craniofacial condition occurs before or after birth, we are prepared to offer ongoing supportive care. We understand medical evaluations and procedures can be overwhelming or even scary for young patients and their families. Our compassionate team involves the family in every step of a child's care.

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