Although uncommon, variations in split cord malformation (SCM) have been described. However, a combination of SCM Type II and dorsal lipomas has not been reported.
The authors describe the case of a 6-year-old girl who presented with a 1-year history of spastic paraparesis associated with a lipomatous swelling on her lower back. Radiology revealed a Type II SCM with a dorsal lipoma at that level. Intraoperatively, the authors observed a dorsal lipoma for each hemicord; these were excised, and the septum causing the split was cut.
Premature separation of the cutaneous and neural ectoderm is predetermined for each neural fold, and a split at this level would give rise to SCM with twin dorsal lipomas, one for each hemicord.