Complex forms of spinal dysraphism.

INTRODUCTION

Spinal dysraphisms are a heterogeneous group of congenital malformations involving the bony component of vertebrae or spinal cord or both. Simple forms include the contiguous, solitary malformations such as myelomeningocele and diastematomyelia, and can be either aperta or occulta type.

MATERIAL

Complex forms include various types of spinal malformations occurring in combination, one type of malformations occurring at multiple levels in noncontiguous manner or spinal dysraphisms with other organ anomalies. Anomalous development in gastrulation, primary neurulation, and secondary neurulation stage in variable proportions give rise to these types of complex malformations. These cases can be diagnosed postnatally in early infancy by the strong clinical suspicion, supplemented by whole spinal axis and cranial magnetic resonance imaging (MRI) screening, to detect any occult spinal dysraphisms like split cord malformations, neurenteric cyst, lipoma, or cranial anomalies like Chiari malformations and hydrocephalus. Computed tomography and MRI are the gold standard to detect bony anomalies and cord malformations, respectively. Complex spinal dysraphisms, to our mind, should always be tried to be corrected in single operative stage, however, in case of ruptured defects, correction through multiple operations can be considered.

DISCUSSION

Overall, prognosis of complex forms of spinal dysraphism is poor in comparison to their simple forms and depends on preoperative neurological status and the nature of complex dyraphism. We discuss the classification, embryogenesis, investigation, and treatment protocol with review of literature of such complex anomalies.