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Cloverleaf Skull: Kleeblattschädel-Deformity Syndrome

Cloverleaf Skull: Kleeblattschädel-Deformity Syndrome Abstract 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 References 1. Comings, D.E.: The Kleeblattschädel Syndrome: A Grotesque Form of Hydrocephalus , J Pediat 67:126-129, 1965.Crossref 2. Holtermüller, K., and Wiedemann, H.R.: Kleeblattschädel Syndrome , Med Mschr 14:439-446, 1960. 3. Becker, P.E.: Humangenetik , Stuttgart: Georg Thieme Verlag, vol 2, 1964, pp 79-80, 539. 4. Liebaldt, G.: Das "Kleeblatt"—Schädel syndrom, als Beitrag zur formalen Genese der Entwiklangsstörungen des Schadeldaches , Ergebn Allg Path 45:23-38, 1964. 5. Berry, H.K.: Amino Acid Excretion In Urine of Normal Infants and Children , Pediatrics 25: 983-996, 1960. 6. Greig, D.M.: Oxycephaly , Edinburgh Med J 189-218, 1926. 7. Temtamy, S.A.: Carpenter's Syndrome: Acrocephalopoly-Syndactyly, An Autosomal Recessive Syndrome , J Pediat 69:111-120, 1966.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Cloverleaf Skull: Kleeblattschädel-Deformity Syndrome

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References (8)

Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1967.02090230128018
Publisher site
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Abstract

Abstract 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 References 1. Comings, D.E.: The Kleeblattschädel Syndrome: A Grotesque Form of Hydrocephalus , J Pediat 67:126-129, 1965.Crossref 2. Holtermüller, K., and Wiedemann, H.R.: Kleeblattschädel Syndrome , Med Mschr 14:439-446, 1960. 3. Becker, P.E.: Humangenetik , Stuttgart: Georg Thieme Verlag, vol 2, 1964, pp 79-80, 539. 4. Liebaldt, G.: Das "Kleeblatt"—Schädel syndrom, als Beitrag zur formalen Genese der Entwiklangsstörungen des Schadeldaches , Ergebn Allg Path 45:23-38, 1964. 5. Berry, H.K.: Amino Acid Excretion In Urine of Normal Infants and Children , Pediatrics 25: 983-996, 1960. 6. Greig, D.M.: Oxycephaly , Edinburgh Med J 189-218, 1926. 7. Temtamy, S.A.: Carpenter's Syndrome: Acrocephalopoly-Syndactyly, An Autosomal Recessive Syndrome , J Pediat 69:111-120, 1966.Crossref

Journal

American Journal of Diseases of Children – American Medical Association

Published: Aug 1, 1967

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