July is National Cleft & Craniofacial Awareness and Prevention Month. Here’s What You Need to Know About These Congenital Anomalies
Each year in the United States, approximately 4,400 infants are born with a cleft lip and 2,600 are born with a cleft palate, with some babies some experiencing both abnormalities. As a pediatric craniofacial surgeon, I’m using this month’s blog to raise awareness and deepen the understanding of craniofacial birth defects and other head and face conditions.
What do I do if my child has a craniofacial deformity?
Counseling and team care
If your baby is diagnosed with a craniofacial deformity during pregnancy, I encourage you to come in for prenatal counseling so we can discuss the diagnosis and what it means for the whole family. Having as much information as possible helps families feel more secure in taking the necessary steps to care for and welcome the newest addition.
Whether your baby is diagnosed in-utero or after birth, a specialized treatment plan will be developed by your cleft and craniofacial team, which will include doctors and medical professionals who specialize in treating skull and face conditions. Patients with craniofacial anomalies not only suffer from medical and functional impairments, but have facial appearance concerns to consider. The inclusion of a board certified plastic surgeon in team care can help patients and their families plan for procedures to achieve the most optimal cosmetic results possible. Your support team may also include non-medical professionals such as local speech therapists, school support advocates, and more.
Insurance coverage and funding resources
Healthcare laws vary by state and insurance policy, both of which change constantly—that said, cleft lip and cleft palate repair are both considered to be reconstructive and therefore covered by most insurance plans. The American Cleft Palate-Craniofacial Association (ACPA) has resources to help families understand how to navigate this process, solve payment issues, and find alternative programs that help provide funding for care.
What are the most common types of craniofacial anomalies?
Children can be born with craniofacial conditions or experience an anomaly after a cranial injury. The most common craniofacial anomalies are cleft lip and palate, craniosynostosis, and positional plagiocephaly. These defects and conditions may cause appearance abnormalities, impair feeding, and/or hinder language development. Children with craniofacial anomalies may also be at a greater risk for frequent ear infections, hearing impairment, and complications with their teeth.
Cleft lip and cleft palate
A cleft lip or cleft palate is a birth defect that occurs when the lip and/or palate are not properly formed, most often in the first 9 weeks of pregnancy. This gap or cleft can affect both sides of the lip and palate (bilateral) or just one side (unilateral). Clefting can affect the soft palate, the hard palate, or both. These defects can be experienced singularly or in combination and can impact the baby’s feeding, hearing, speech development, and nose shape and function.
Diagnosing and treating a cleft lip and palate
A cleft lip may be detectable via prenatal ultrasound and can be diagnosed in the womb or at birth. It is surgically repaired when the baby is a few months old. Small incisions are used to create flaps of skin on the upper lip that can be stitched together to close the gap. Whenever possible, incisions are hidden around the contours of the nose. The procedure typically takes 2 to 3 hours and most patients stay in the hospital for one night. It is possible a child may need a later revision as they grow and develop. Depending on the severity of the cleft, and the patient’s individual anatomy, scarring can range from hardly noticeable to more obvious. Children with cleft lips commonly need orthodontic treatment, though significant long-term speech impairments are unlikely.
A cleft palate is difficult to detect via ultrasound and thus is usually diagnosed at birth or shortly after. As the condition can impair swallowing and affect speech development, surgery is typically necessary and is often performed between 10 - 12 months of age, when the baby starts to make speech sounds. The procedure takes a few hours with incisions running along the roof of the mouth, hiding the scars completely. The baby will stay 1-2 nights in the hospital. The child’s development will be monitored; subsequent palate surgery may be called for in time. Orthodontic treatment is almost always needed for children with cleft palates. Speech therapy may also be appropriate.
Risk factors for cleft lip and palate
The following risk factors may increase the likelihood of developing a cleft lip and palate:
Genetic predisposition
Maternal diabetes or obesity
Substance exposure in the womb (ie; alcohol, certain medications, or cigarettes)
Race (cleft lip and palate are most common in Native Americans and least common in African Americans)
Gender
Female infants are more likely to have a cleft palate without a cleft lip
Male infants are more likely to have a cleft lip without a cleft palate
Craniosynostosis
Craniosynostosis occurs when one or more bones of the skull prematurely fuse in the womb. In normal development, there are gaps or sutures between a baby’s skull bones which first provide flexibility as the baby moves through the birth canal; after birth, they also make space for the brain to grow as a child develops. When craniosyntosis occurs, the skull will expand unevenly, spreading at open sutures but not along fused sutures, which will ultimately result in a misshapen head. If multiple sutures are fused, pressure inside the head will be particularly problematic and worsen as the child grows.
Diagnosing and treating craniosynostosis
Craniosynostosis is diagnosed after physical examination of the baby and/or with cranial x-rays and CT scans at birth. Repairing craniosynostosis often takes 4 to 5 hours; the fused sutures are opened to ease pressure and allow room for brain growth. There are also cosmetic benefits: after repair, the child will develop a more normal skull and face shape. Incisions are thoughtfully placed to seamlessly blend in with the hair. After surgery, the patient will typically spend the night in the Pediatric Intensive Care Unit (PICU) followed by a few nights in the general Pediatric ward to be monitored for any signs of complications.
Craniosynostosis can be an isolated condition or part of a known syndrome. These are 3 of the most common syndromes:
Pfeiffer, seen in one of every 25,000 babies with varying degrees of severity
Crouzon, seen in one out of 100,000 babies with varying degrees of severity
Apert, seen in one out of 100,000 babies and causing syndactyly (webbed) hands and feet
Timing for corrective surgery can vary: we take the severity of the particular syndrome, treatment plan steps, and other associated issues into consideration when planning craniosyntosis surgery and follow-up procedures.
Risk factors for craniosynostosis
Craniosynostosis is thought to be caused by a combination of genetic abnormalities and certain exposures in-utero, though research for definitive answers is still on-going. New research suggests that these factors may increase the likelihood of developing craniosynostosis:
Maternal thyroid disease and/or treatment
Certain medications (ie; clomiphene citrate to treat infertility)
Limited womb space (ie; carrying multiples)
Plagiocephaly
Positional plagiocephaly is caused by continuous pressure on the bones of the skull which flattens that part of the head.
Diagnosing and treating plagiocephaly
Positional plagiocephaly is diagnosed after a physical examination by a craniofacial surgeon, sometimes with the aid of x-rays to rule out craniosynostosis syndromes. Positional plagiocephaly can be evident or hardly noticeable. If caught early enough, mild positional molding may resolve organically within the first 6 months of life with simple position changes. Moderate to severe positional molding in children under 14 months of age can be improved with helmet therapy. Each helmet is specifically designed to fit comfortably on the baby’s head as it helps reshape the skull. The helmet is worn continuously for an average of 3 months and typically goes unnoticed by the baby after a few days.
Risk factors for plagiocephaly
The following may increase the likelihood of developing positional plagiocephaly:
Positional preference: The baby sleeps in the same position
Torticollis (muscle tightness): The baby holds their head in a particular position to alleviate discomfort
Premature birth: The baby’s head molds to the isolette bed's flat surface instead of forming more naturally in the womb
Uterine crowding: Carrying more than one baby, uterine fibroids, or a misshapen uterus putting pressure on the skull bones.
Birth canal decent: Pressure from the birth canal can mold the head, especially if the baby is positioned in the canal for an extended period
Craniofacial care in Richmond
Dr. Sharline Zacur Aboutanos is a cleft and craniofacial specialist and board certified plastic surgeon who has earned numerous awards for her dedication to patient care and surgical excellence, including being designated by her peers as a Richmond Top Doctor in both Cosmetic Plastic Surgery and Reconstructive Surgery for the last four consecutive years.
As a member of the highly-qualified Cleft and Craniofacial Team at St. Mary’s Hospital, Dr. Aboutanos is passionate about caring for her patients and their families before, during, and after craniofacial surgery. If your baby has been diagnosed with a craniofacial condition, we are ready to both support you and coordinate with your baby’s medical team to ensure an excellent functional and aesthetic outcome. We invite you to schedule a consultation online or call Aboutanos Plastic Surgery at 804.355.3410.