Acalvaria is a rare congenital malformation characterized by an absence of skin and skull. The authors describe a newborn at an estimated 38 weeks gestational age who was delivered via cesarean section from a 32-year-old mother. Upon delivery, the child was noted to have a frontal encephalocele and an absence of calvaria including skull and skin overlying the brain. A thin membrane representing dura mater was overlying the cortical tissue. After multiple craniofacial operations, including repair of the encephalocele and application of cultured keratinocytes over the rostral defect, the patient demonstrated significant closure of the calvarial defect and was alive at an age of more than 17 months with near-average development.
Address correspondence to: Ammar H. Hawasli, M.D., Ph.D., Washington University School of Medicine, Department of Neurological Surgery, 660 S. Euclid Ave., Campus Box 8057, St. Louis, MO 63110. email: firstname.lastname@example.org.
Please include this information when citing this paper: published online June 13, 2014; DOI: 10.3171/2014.5.PEDS13688.