Beitrag zum Kleeblattsch�del-Syndrom

Abstract

Cloverlea] Skull Syndrome Abstract.A case report is presented describing an infant with systemic skeletal malformation including a "cloverleaf skull" (Kleeblattschgdel) cleft formation of the facial bones, mMformation of tile brain, hydrocephalus, and symmetrical notching of hands and feet. This entity has been described as "clover leaf skull syndrome" by Holtermfiller and Wiedemann. The etiology of the syndrome is discussed with special reference to lower abdominal X-ray examinations of the mother in early pregnancy as a possible cause of the disorder. The malformations result most likely from a disturbance in the development of the primitive segments involving mainly cranial somites and extremity buds. Key-Words:Clover Leaf Skull --Cleft Formation of the Facial Bones --Malformation of the Brain --Other Malformations X-ray Examinations of the Mother in Early Pregnancy.14"

“ADAM complex” (amniotic deformity, adhesions, mutilations)—A pattern of craniofacial and limb defects

“…Amniotic deformity complexes sometimes have been grouped together with malformations of developmental origin [Berndorfer, 1970;Verschuer, 19411 ; some cases of the "Kleeblattschadel-Syndrom" reported earlier may be of amniotic origin [Schuch and Pesch, 1971;Weingartner, 1961, Case 11. Except for our Cases 6 and 7 we are not aware of any familial observation of the ADAM complex in the literature. The familial occurrence in our patients may therefore be explained by chance, but might, on the other hand, stress the significance of genetic factors acting in the etiology of amniotic anomalies.…”

Cloverleaf Skulls

“…Intrauterine amniotic bands may cause amputations of digits or limbs and may also become attached to the head and face, causing bizarre asymmetric encephaloceles, oblique clefts, and rarely a cloverleaf skull 12,33,34 (Fig. 5).…”

Genetic perspectives on craniosynostosis and syndromes with craniosynostosis

“…Levin, et al (Levin LS, Perrin JCS, Ose L, et al: A heritable syndrome of craniosynostosis, short thin hair, dental abnormalities, and short limbs: cranioectodermal dysplasia. J Pediatr 90: [55][56][57][58][59][60][61]1977) reported an autosomal recessive condition consisting of dolichocephaly with craniosynostosis, sparse, slowgrowing hair, epicanthal folds, hypondontia, short limbs, brachydactyly, brachypodia, and other abnormalities.…”