Summer 2011

Case Study: Bilateral Coronal Synostosis

"You know you're doing the right thing when your kid is happy going to see her doctors," said Sharyn Ruso.

Her daughter, Samantha, was diagnosed at 2 months old with bilateral coronal synostosis, a congenital disorder in which the bones of the skull fuse prematurely. By the time she was 1 year old, she had had several craniofacial operations at a university hospital near her home to treat the condition.

Her craniosynostosis recurred, creating ongoing complications. When she was 4, Samantha began losing motion on one side. Her parents brought her to UCSF Benioff Children's Hospital, where she was evaluated by Professor Warwick Peacock, M.D., a neurological surgeon. He diagnosed a Chiari type I malformation, a downward displacement of the brain at the base of the skull.

The condition, caused by the re-fusing of her skull plates, constricted the brain's ability to grow normally. Peacock and Professor William Hoffman, M.D., chief of plastic and reconstructive surgery, performed bony decompression surgery that same evening.

Since then, Samantha has been followed in the Center for Craniofacial Anomalies at UCSF to monitor her facial growth and development. This allowed coordination of orthodontic and surgical care.

13-hour Surgery

When she was 11, Hoffman performed a LeFort III advancement of the midface. The 13-hour surgery involved cutting the upper jaw and cheekbones as a unit to advance them forward, then bone grafting from her hip to fill in the spaces.

The surgery rebuilt the eye sockets, allowing Samantha to close her eyes during sleep as well as correcting her bite.

"I just love UCSF,” said Sharyn. "The doctors listen to the patients and the parents. I can call and get Dr. Hoffman or someone else on the phone. If something abnormal comes up, Dr. Hoffman will say, 'Bring her on up.' "

After one surgery, Samantha developed a localized infection, which might have required further surgery for drainage. "Samantha started crying, and I started crying," said Sharyn.

Hoffman offered an alternative. If the family could bring her in every day for two weeks, he would drain the infection himself, thus avoiding surgery. "I said, 'We’ll do it,' " said Sharyn.

The continuing premature fusion of her cranial plates created septal deviation, resulting in difficulty in nasal breathing and possibly sleep apnea. When she was 14, Hoffman performed a septorhinoplasty to straighten her nasal septum. He also performed osseous genioplasty to correct her retruded chin.

In these procedures Hoffman applies techniques that are also used in cosmetic surgery; for example, to correct residual flat contour of the cheeks, he used autologous fat injection from her abdomen.

Despite Samantha's many surgeries, her facial scars are not visible.

"My main concern is that she's able to breathe and see," Sharyn said. "Dr. Hoffman not only helped her breathe better, he also helped her look better. Kids don’t tease her any more. She's a totally different girl now, and her self-esteem is really high."

Samantha recently started high school, where she is a straight-A student and a star volleyball player.

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