For many parents, discovering that their baby has a disease or abnormality can be a source of fear, concern, or anxiety. This initial feeling of shock and despair is often present for parents of children born with the birth defect craniosynostosis, which occurs in 1 out of every 2000 births.
About Craniosynostosis
The infant skull is made up of a series of bones that are connected by fibrous tissues called cranial sutures. These cranial sutures are the growth centers of the infant skull.
Craniosynostosis is a condition where a cranial suture fuses prematurely, creating a “sticky point” that restricts normal skull growth in infants. As a result, the skull grows parallel to the fused area, leading to an unusual head shape and potentially placing pressure on the developing brain. Some common craniofacial deformities and the associated sutures involved include:
Scaphocephaly (Sagittal Suture)Derived from "scapho," meaning "boat," scaphocephaly describes a head shape that appears elongated and narrow. This occurs when the sagittal suture fuses prematurely, leading to a long, boat-like skull shape.
Plagiocephaly (Unilateral Coronal Suture)From "plagio," meaning "oblique" or "twisted," plagiocephaly is characterized by asymmetry in the skull. It occurs when one side of the coronal suture fuses prematurely, resulting in a twisted or oblique head shape.
Trigonocephaly (Metopic Suture)"Trigono" means "triangular." This deformity arises when the metopic suture fuses early, leading to a triangular-shaped forehead with a pointed ridge along the center of the forehead.
Brachycephaly (Bilateral Coronal Suture)Stemming from "brachy," meaning "flat," brachycephaly results in a shortened head shape. This occurs when both sides of the coronal suture fuse prematurely, giving the head a broad, flat appearance.
Lambdoidal Deformity (Posterior Plagiocephaly)In cases involving the lambdoidal suture, multiple sutures may fuse, often leading to a more complex and pronounced deformity. This type of fusion is sometimes referred to as a "cloverleaf deformity" due to its distinctive appearance.
Each of these craniofacial conditions results from the premature fusion of specific cranial sutures, affecting the shape and symmetry of the skull. Early diagnosis and intervention can be critical for managing these conditions and supporting healthy growth and development.
Craniofacial Surgical Solutions
When your infant is born with craniosynostosis or another craniofacial abnormality, its important to consult a highly trained craniofacial surgeon as soon as possible.
“With this condition, the goal of surgery is to remove the fused cranial suture to allow the brain to grow without restriction and to reverse the cranio-orbital deformity,” says Dr. Frank Ciminello, a board-certified plastic surgeon with advanced training in craniofacial and pediatric plastic surgery. “There are two different surgical treatment options we perform to achieve this: endoscopic minimally invasive strip craniectomy or traditional cranial vault remodeling.”
Endoscopic Minimally Invasive Strip Craniectomy
According to Dr. Ciminello, this surgical option is extremely effective if the child is diagnosed and evaluated by a craniofacial surgeon before they are three months old. With this technique, the fused cranial suture is removed though a small incision(s). Following the procedure, the child is placed in a custom cranial-molding helmet to mold the head into a more normal shape over time.
The advantages of this technique are smaller scars, shorter hospital stay, potential for less blood loss, and less post-operative swelling. The disadvantage is that the deformity takes more time to correct. But if this procedure is done early in life, the long-term results are comparable to more traditional techniques.
Traditional Cranial Vault Remodeling
This operation is typically performed when the infant is between 4 and 8 months old. With this technique, the fused suture is also removed. In addition, the deformity in the head shape is corrected during the surgery. So, when the surgery is done, the child’s skull will appear to have a normal shape.
The major advantages of this technique are that the deformity is corrected immediately following the surgery and there is no need for a post-operative helmet.
“This procedure provides a better long-term result for infants that missed the window of treatment before reaching three months of age,” says Dr. Ciminello.
The disadvantages are a larger scar in the hair-bearing scalp, greater potential for blood loss, and a somewhat longer hospital stay.
Navigating your child’s treatment journey can feel overwhelming and isolating. At Cimisurgical, our team is here to assist you every step of the way. Providing compassionate care to patients and their parents is a distinction we are proud of at our practice.
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Dr. Ciminello has served as the Section Chief of Craniofacial & Pediatric Plastic Surgery for the last two decades at the Hackensack University Medical Center, as well as the Director of Craniofacial Surgery at Morristown Medical Center and Co-Director of Craniofacial Surgery at Barnabas Health. His work and research have been published extensively in the specialized field of Plastic & Reconstructive Surgery, and he has lectured nationally and internationally as an expert on numerous Plastic & Reconstructive Surgery subjects. Dr. Ciminello has been a board certified plastic & reconstructive Surgeon since 2008, with a sub-specialty in craniofacial and pediatric plastic surgery. He has been recognized by his peers by being selected as a Castle Connolly Top Plastic Surgeon, an America's Best Plastic Surgeon, a NJ Top Doc, and a Favorite Kids Doc.
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