Basicranial diplomyelia: an extension of the split cord malformation theory

Abstract

Basicranial diastematomyelia is an extremely rare congenital disorder. A review of the literature indicates only one reported case of basicranial diastematomyelia in which an osseous peg divided the brainstem in two. The authors present the first reported case of basicranial diplomyelia split by a fibrous band and correlate its pathogenesis with that of split cord malformation (SCM). The patient described in the present report had a fibrous stalk dividing the brainstem, and therefore the condition was categorized as a diplomyelia, or SCM Type II. Because the occipital dermatomes behave similarly to the spinal dermatomes early in development, they may be subject to the same embryonic error that results in SCM. The authors propose that the mechanism leading to SCM is the same as that found in basicranial split malformations and that the theory explaining it be modified to include the posterior fossa.