The outcome of 34 infants with a diagnosis of cephalocele was reviewed at the University of Colorado Health Sciences Center, Denver, and was compared with that of previously published series. Of 22 posterior defects, 5 were meningoceles and of 12 anterior defects, 8 were meningoceles. Additional major abnormalities were present in 15 (44%) of 34 infants, 7 (58%) with anterior defects and 8 (36%) with posterior defects. Those with anterior defects had abnormalities of the cranium, while those with posterior defects had extracranial abnormalities with greater frequency. Mortality was 29% (10/34) and confined to infants with posterior cephaloceles. Microcephaly was noted in 7 children all with posterior defects. Nine had hydrocephalus. Seizures occurred in 14 children, 5 with anterior defects and 9 with posterior defects. Outlook depended on site, operability, and additional major abnormalities. Mortality was confined to posterior defects, inoperability, congenital heart disease, infection, and shunt malfunction. Microcephaly, seizures, and hydrocephalus were associated with a worse outcome. Anterior defects have a better prognosis than posterior defects. [1]

COMMENT. Cephaloceles (cranium bifidum) occur most frequently in the midline. The primary morbidity in children with anterior defects has included facial disfigurement, anosmia and visual problems. Although there were no deaths in infants with anterior defects in this series, significant long-term disability was present in 50% and was associated with severe facial clefting, intracranial abnormalities and visual impairments. In infants with posterior defects, severe long-term disabilities occurred in 25% and mild handicaps in 38% of surviving infants.