Craniosynostosis. I. Sagittal synostosis; Its genetics and associated clinical findings in 214 patients who lacked involvement of the coronal suture(s)

Abstract

The clinical and genetic findings in 214 patients with sagittal synostosis are described; 73% of the patients were male. Children with sagittal synostosis were treated earlier than those with coronal synostosis. Major malformations occurred in 22%, and 8.9% were mentally retarded. The retardation was clearly unrelated to the synostosis in almost 1/2 the patients. The remaining retarded patients had a significantly lower mean birth weight, higher frequency of malformations, and later age at operation than the control group. The late age at operation was probably due to bias in the ascertainment of this group of retarded children, and sagittal synostosis was apparently one of a number of malformations that can occur in children with intrinsic retardation. Familial data and the skull measurements of a sample of parents of affected children were compatible with multifactorial inheritance. There is need for prospective family studies and parental measurements on ethnically uniform groups.