[Hereditary sclerodactyly and syndactyly]. / Hereditäre Sklerodaktylie und Syndaktylie.

A 61-year-old man is described with sclerodactyly of the hands and syndactyly of the second and third toes. Hereditary sclerodactyly is a rare condition, beginning in early youth with flexion contracture of the fingers. In this patient the skin of the fingers was sclerotic and thickened, and the dorsal skin of the hands was atrophic and dry. The condition did not progress nor did it show signs of Raynaud's phenomenon. Both feet showed syndactyly of the second and third toes. The family tree suggested autosomal dominant inheritance, with reduced penetrance since the grandfather of our patient was reported to have had a similar disease.

Exencephaly in Cantrell-Haller-Ravitsch Syndrome.

A case of exencephaly associated with spinal dysraphism and Cantrell-Haller-Ravitsch syndrome is described. Significant findings included exencephaly, multiple subcortical neuroepithelial islets with active proliferation, cerebellar aplasia, and abnormal flexion of the neuraxis with spinal dysraphism of the cervicothoracic region. The various features of exencephaly, anencephaly, and iniencephaly are discussed. The significance of subcortical growth zones is evaluated in the light of experimental observation.s

Congenital ventricular diverticulum in the brainstem. Report of four cases.

This report concerns rare ventricular malformations observed in four fetal brains. Multiple aqueductal diverticula were the only malformation of the central nervous system (CNS) in one case, unilateral diverticula of the 4th ventricle in a holoprosencephaly and a hydrocephaly, and a ventral accessory aqueduct in a fetus with trisomy 18. These findings are the pathologic progression of embryonal ventricular recesses which may be seen in normal brains.