Unusual split cord with neurenteric cyst and cerebellar heterotopia over spinal cord

To report a case of splitting of a cord, which could neither be classified as type I nor type II according to Pang's classification. The child had associated neurenteric cyst and cerebellar tissue heterotopia.

A 3-year-old girl presented with a tuft of hair and dermal sinus at her upper dorsal spine. She had splitting of cord into two halves by two intradural horny osseocartilaginous spurs leaving a gap of 3 mm in between (two spurs and two halves of cords). A neurenteric cyst was seen passing from the anterior to posterior aspect of the cords through this gap. An associated heterotopic cerebellar tissue mass was encountered over the dorsum of cord proximal to splitting. The dermal sinus, neurenteric cyst, and spurs were excised to detether the cord. The heterotopic tissue was biopsied. The child had no neurological deficit at 2.5 years follow-up.

An unusual splitting of cord by unusual spurs may leave a gap between two spurs and two halves of cords to pass a neurenteric cyst. Rarely, heterotopic tissue may be associated with split cord syndrome. A dermal sinus may lead into underlying neurenteric cyst (rather than dermoid).