Winter 2009

Early Diagnoses of Misshapen Heads Can
Reshape Futures

William Hoffman, M.D.

"When children have misshapen heads due to craniosynostosis or deformational plagiocephaly, it's critical for pediatricians to diagnose early, usually within the first three to five months of life," says William Hoffman, M.D., chief of Plastic and Reconstructive Surgery at UCSF Benioff Children's Hospital and director of the Craniofacial Center there.

Early diagnosis — usually with a physical exam only or, occasionally, with plain films or a CT scan — reduces the need for helmet treatment for plagiocephaly and expands the options available to craniofacial surgeons for correcting deformities due to craniosynostosis.

Deformational plagiocephaly is the more common of the two conditions, due in part to sleep positioning programs recommended in the early 1990s to address legitimate SIDS concerns, but which caused a dramatic increase in the incidence of deformational plagiocephaly. Hoffman sees between five and 10 of these cases each week.

He typically recommends an aggressive positioning program. "We get them off the flat part of their skull and usually see great improvement," he says. "Only about 10 percent of children need to have molding helmets."

Surgical Options for Craniosynostosis

In the case of craniosynostosis, total reconstructive surgery is the most common treatment option. At UCSF Benioff Children's Hospital, which performs about 30 such procedures each year, Hoffman usually works with pediatric neurosurgeon Nalin Gupta, M.D., PhD. Gupta removes the bone to separate the fused sutures, and Hoffman reshapes the skull to allow for normal development.

"With younger children (under 4 months), we also do endoscopic-assisted synostectomy," says Hoffman. This minimally invasive procedure releases the fused sutures, but requires postsurgical treatment with a molding helmet. In contrast, total reconstruction does not.

Maxillofacial distraction osteogenesis is a newer surgical option that a specialty clinic like the one at UCSF Benioff Children's Hospital can offer for a variety of craniosynostosis syndromes. "By using distraction after cutting the bones in the face, we can overcome soft tissue constriction to gradually correct severe facial deformity," says Hoffman.

Correcting Microtia

William Hoffman, M.D., chief of Plastic and Reconstructive Surgery at UCSF Benioff Children's Hospital, has worked with Lawrence R. Lustig, M.D., implant surgeon and director of the UCSF Douglas Grant Cochlear Implant Center, to create a new, combined clinic for correcting microtia, a congenital deformity of the outer ear. By working together, the surgeons combine a full cosmetic reconstruction of the external ear with creation of a new canal to improve hearing.

"We're happy to see microtia patients early on, but we have to wait to do the procedure until they're about 6 or 7 years old because we carve the ear out of one of their ribs and we need enough to make an adult-sized ear," says Hoffman. The surgery occurs in several stages, but provides a durable ear reconstruction that can be strikingly normal in appearance. In addition, in a number of these patients, Lustig can successfully reconstruct the ear canal; alternatives include the bone-anchored hearing aid.

For more information, contact William Hoffman, M.D., at (415) 353-4287.

Related Information

News Releases

UCSF Neurointensive Care
Nursery Opens

Infants who show signs of brain damage at birth and who are at risk for cerebral palsy, mental retardation and other cognitive disorders are being treated at the first Neurointensive Care Nursery in the country.

UCSF MD Wins Ewing
Sarcoma Grant

A pediatric cancer specialist has won two-year, $80,000 Hope Street Kids research grant to study a new technique to detect circulating tumor cells in patients with Ewing sarcoma.