Pediatric Craniosynostosis Surgery: Here's What You Should Know
Though many parents are lucky enough not to have encountered craniosynostosis, it is an increasingly common condition affecting the mental and physical development of children everywhere. As a board-certified plastic surgeon specializing in craniofacial and pediatric reconstructive surgery, Dr. Sharline Aboutanos explains what—and why—you need to know about craniosynostosis.
Key Takeaways
Before diving in, here are a few key takeaways from this post:
Early diagnosis and intervention is critical to patient success.
Compassion, empathy, and clear explanations from doctors and surgeons are important, as the condition is distressing for families.
More research is needed into the causes of craniosynostosis and its increasing rates.
What is craniosynostosis?
Craniosynostosis is a congenital birth defect that occurs in children before their brains are fully grown. A baby is born with 5 plates in its skull that are made of bone and “float” just above its brain. These plates expand as the brain grows and normally begin to fuse by the time a child has reached the age of 2. However, when these plates fuse prematurely in a baby’s skull, the skull—and sometimes the face—can become misshapen, and pressure can build around the brain, leading to developmental delays.
Syndromic vs. nonsyndromic craniosynostosis
Craniosynostosis can either be syndromic or nonsyndromic, depending on whether the condition is isolated or accompanied by genetic syndromes:
Nonsyndromic craniosynostosis occurs within 75% of craniosynostosis patients and typically involves a single suture (the part of the skull where the plates have fused). There are 5 types of nonsyndromic craniosynostosis, categorized by the location of the suture and when they fused. The 2 most common types of nonsyndromic craniosynostosis are sagittal suture synostosis (scaphocephaly) and unicoronal synostosis (frontal plagiocephaly). Unlike syndromic craniosynostosis, nonsyndromic craniosynostosis is typically not associated with other physical deformities or genetic syndromes.
Syndromic craniosynostosis is a much rarer and more severe form of craniosynostosis. Patients with syndromic craniosynostosis often experience other birth defects and abnormalities, such as cleft palate, hearing loss, ear infections, vision problems, fusion of the fingers and toes, cognitive impairment, maladaptive behaviors, learning disabilities, and facial deformities—although these defects can also be present in nonsyndromic craniosynostosis. Syndromic craniosynostosis is associated with genetic syndromes, such as Apert and Crouzon syndromes.
What causes craniosynostosis?
Craniosynostosis is thought to be caused by gene mutations and/or environmental factors, such as a mother’s diet, any medications or supplements used during pregnancy, and whether she’s used nicotine. Craniosynostosis can occur in children whose families have had a history of the condition, as well as those who have not. Recently, the CDC reported that women with a thyroid disease may be more likely to give birth to a child with craniosynostosis.
Why should you care about craniosynostosis?
Rates of craniosynostosis have risen significantly around the world since the 1990s, affecting nearly 1 in 2,500 babies. Although many inroads have been made as to what causes craniosynostosis, the reason for the rise in cases is unknown.
What are the symptoms of craniosynostosis?
Symptoms of craniosynostosis may include:
An abnormally shaped skull.
Absence of a soft spot on top of the baby’s skull.
A raised hard spot on top of the baby’s skull (called a “ridge”) from where 2 or more sutures have fused prematurely.
Increased intracranial pressure (ICP).
Craniosynostosis is often apparent at birth, but it can be difficult to diagnose, especially during the first few months of a baby’s life. Craniosynostosis can be confused with deformational (positional) plagiocephaly, a less severe skull deformity. The difference between craniosynostosis and positional plagiocephaly can be distinguished in most cases by physical exam alone by a craniofacial plastic surgeon. A doctor can also use a CT or CAT scan to help identify craniosynostosis.
Risks associated with craniosynostosis
There are severe risks associated with craniosynostosis, including:
Increased intracranial pressure (ICP) can continue to develop and cause brain damage, blindness, seizures, and even death.
Aesthetic concerns, such as a misshapen skull, sunken face, enlarged brow, eyes spaced too far apart or looking in different directions, and/or cleft palate.
Thankfully, there is treatment for craniosynostosis. Recent medical advances have improved craniosynostosis surgery, and doctors and pediatricians are more aware of—and qualified to treat—this condition than ever.
Treating craniosynostosis
Many types of craniosynostosis require surgery to relieve intracranial pressure, reshape the skull, and allow the brain to grow properly. Surgery is normally performed during a baby’s first year, and early diagnosis and intervention is critical for optimal surgical results. Usually the earlier craniosynostosis is diagnosed, the more surgical options are available.
Surgery for craniosynostosis
Surgery for craniosynostosis depends on a child’s age and their unique craniosynostosis. Cranial vault remodelling (CVR), also called open cranial remodelling, is the oldest surgery for craniosynostosis. It is usually recommended for children ages 5-6 months or older. This is considered an invasive surgery, as a surgeon must remove a portion of the child’s skull to reshape the affected bone and reassemble the skull in a more anatomic head shape. CVR surgery is proven to improve the overall shape of the head and increase space for the developing brain. Surgery takes about 4-6 hours. A cell saver and certain medications are utilized during surgery to minimize the need for a blood transfusion.
Benefits of craniosynostosis surgery
Surgery can significantly reduce a child’s risk of experiencing developmental delays by decreasing pressure in the skull. It can also help a child have a more normal life and avoid bullying. Surgery may not be needed for mild cases of craniosynostosis, in which case, a molding helmet can be used on its own to re-shape the skull.
Board-certified Richmond plastic surgeon Dr. Aboutanos can help
Dr. Sharline Zacur Aboutanos is an award-winning board-certified plastic surgeon serving patients of all ages in Richmond, Virginia. Dr. Aboutanos has over 10 years of experience in pediatric plastic surgery, and is both a Fellow of the American Academy of Pediatrics and a member of the accredited Cleft and Craniofacial Team at St. Mary’s Hospital. Contact Dr. Aboutanos online or by calling 804-355-3410 to discuss your child’s needs.