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October 1969

Kleeblattschädel Syndrome

Author Affiliations

From the Center for Genetic Counseling and Birth Defect Evaluation (Dr. Feingold); Tufts-New England Medical Center Hospitals (Boston Floating Hospital for Infants and Children), and the departments of pediatrics (Dr. Feingold) and radiology (Dr. Darling), Tufts University School of Medicine; Tufts University School of Dentistry (Dr. Berkman); and the Department of Pediatric Radiology, Boston City Hospital and Boston University Medical School (Dr. O'Connor), Boston. Dr. Berkman is a postdoctoral fellow in oral pathology, Tufts University School of Dentistry.

Am J Dis Child. 1969;118(4):589-594. doi:10.1001/archpedi.1969.02100040591011

IN 1960, Holtermueller and Wiedemann1 described a new case of cloverleaf skull and reviewed the literature. They named the entity the Kleeblattschädel syndrome. Comings2 and Angle et al3 subsequently described the only three reported cases in the American literature.

The major diagnostic features of this syndrome include a grotesque, trilobed skull resulting from congenital premature synostosis of the coronal and lambdoidal sutures (plus other sutures as described in our patients); severe exophthalmos; deformities of the long bones resembling achondroplasia; mental retardation; and an early demise.1-5

Two new cases of the Kleeblattschädel syndrome are reported. The first a 14-year-old girl, is the oldest recorded patient, and the second represents the typical long bone changes.

Report of Cases  Case 1.—The first patient, a 14-year-old white girl, was the product of a 33-year-old, gravida 4, para 2, abortus 2 mother and a 34-year-old father. The pregnancy was not complicated