HYPOTHALAMIC HAMARTOMA IN ORAL-FACIAL-DIGITAL SYNDROME TYPE-VI (VARADI-SYNDROME)

Oral-facial-digital syndrome (OFDS) type VI (Varadi syndrome) is an au tosomal recessive trait of orofacial anomalies, cerebellar dysgenesis, and polysyndactyly. Developmental anomalies of the posterior fossa, i ncluding cerebellar hypoplasia and variants of the Dandy-Walker comple x, are the most common central nervous system malformations reported i n patients with this syndrome. We report hypothalamic hamartoma, super numerary maxillary incisor, and precocious puberty in a boy with OFDS type VI. We propose that hypothalamic hamartoma is an occasional manif estation of OFDS type VI. (C) 1994 Wiley-Liss, Inc.