Oto‐palatal‐digital syndrome type II with X‐linked cerebellar hypoplasia/hydrocephalus

Abstract
We describe an infant with clinically apparent oto‐palatal‐digital syndrome Type II (OPD II), who, in addition, also has hydrocephalus and cerebellar hypoplasia. This second X‐linked disorder has not been reported previously to occur in association with OPD II. This patient had 2 maternal uncles who died neonatally with congenital hydrocephalus and digital abnormalities consistent with OPD II. We suggest that these 2 entities may be located near one another on the X chromosome, and that both loci are affected in this family.