August 1967

Cloverleaf Skull: Kleeblattschädel-Deformity Syndrome

Author Affiliations

From the Birth Defects Special Treatment Center of Children's Memorial Hospital, the departments of pediatrics (Drs. Angle and McIntire), neurology and psychiatry (Dr. McIntire), and radiology (Dr. Moore), University of Nebraska, and the Department of Pediatrics (Dr. Angle), Creighton University, Omaha.

Am J Dis Child. 1967;114(2):198-202. doi:10.1001/archpedi.1967.02090230128018

KLEEBLATTSCHÄDEL syndrome, translated to cloverleaf skull by Comings,1 is the evocative name given by Holtermüller and Wiedemann2 to the grotesque, trilobed skull resulting from congenital hydrocephalus associated with intra-uterine synostosis of the coronal and lambdoidal sutures. The increased intracranial pressure is manifested as bulging in the areas of the sagittal suture and squamosal sutures with a downward displacement of the ears, severe proptosis or exophthalmos, and facial deformities similar to those of craniofacial dysostosis. Deformities of the long bones resembling achondroplasia have been described in 11 of the 17 reported cases.1-4 The purpose of this communication is to report two additional cases of cloverleaf skull: one with all of the severe bony deformities previously described, the other, manifesting only the cranial abnormalities.

Report of Cases  Case 1.—This white female infant was born Sept 17, 1965, the product of the first pregnancy of a 29-year-old mother after eight

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