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December 1964

Craniotelencephalic Dysplasia: An Unusual Example of Dysplasia of Frontal Bone

Author Affiliations

J. T. Jabbour, MD, the University of Oklahoma Medical Center, 800 NE Thirteenth St, Oklahoma City, Okla 74104.; From the departments of pediatrics and radiology, Children's Memorial Hospital, University of Oklahoma Medical Center.

Am J Dis Child. 1964;108(6):627-632. doi:10.1001/archpedi.1964.02090010629008

In 1958, Daum et al1 described a 6-month-old infant with frontal bone protrusion, encephalocele, craniostenosis, and developmental retardation. The presence of either encephalocele or craniostenosis in an infant is not unusual; however, the combination of these two anomalies does constitute an unusual disorder. The purpose of this report is to describe the clinical and radiological features of a similar disorder—that of frontal bone dysplasia, craniostenosis, and retardation of neurological development without an encephalocele.

Report of a Case  A white female, 6 weeks of age, was presented at the Children's Memorial Hospital because of a conspicuous malformation of the skull. She was the product of an eight-month pregnancy from a gravida 7 para 6 mother. During the fourth week of gestation, the mother was injured in an automobile accident during which she had momentary loss of consciousness and lost two teeth. Subsequently, she had no pelvic or cranial complaints. Fetal