Diastematomyelia

In this condition the spinal cord is divided longitudinally in the anteroposterior plane by a fibrous or bony structure. The cases are usually isolated but affected sisters were reported by Kapsalakis (1964). Gardner (1973) described a family in which 3 sisters had diastematomyelia and other dysraphic malformations in various combinations.

Balci et al. (1999) reported 2 sisters with diastematomyelia with variable expressivity. They suggested that X-linked dominant inheritance with lethality in hemizygous males or female sex preference of a multifactorial trait may explain the fact that all reported familial cases have been female.