There has been confusion in the classification of terminal myelocystocele (TMCC) due to its diverse morphology and vague pathoembryogenesis. TMCC could be summarized as having the essential features of an elongated caudal spinal cord extruding out of the dorsal extraspinal space that fuses with the subcutaneous fat, which is in the shape of a trumpet-shaped cerebrospinal fluid-filled cyst. The extraspinal portion of the extruded spinal cord is nonfunctional. The morphological features suggest that TMCC is formed during secondary neurulation, specifically the failure of the degeneration of the secondary neural tube near the time of the terminal balloon. This review discusses the definition, as well as the clinical and surgical features, of TMCC with special emphasis on its pathoembryogenesis.
Keywords: Congenital anomalies; Nervous system; Neurulation.