Cervical split cord malformation (diastematomyelia) with associated Klippel-Feil deformity presenting in adulthood with bimanual synkinesis

Eur Spine J. 2024 Jan 16. doi: 10.1007/s00586-023-08068-7. Online ahead of print.

Abstract

Background: Split cord malformation (SCM) is a rare congenital malformation of the spinal cord in which the cord is split longitudinally. Identification and diagnosis in adulthood is rare, with the majority of cases diagnosed in the paediatric population. Isolated segmental cervical SCM is rarer still.

Case presentation: Here, the authors present the case of a 26-year-old female who presented with neck pain and longstanding bimanual synkinesis secondary to an isolated type II SCM in the C4-C7 region. The authors present this novel presenting symptom in adulthood and finding of isolated cervical SCM with associated blocked cervical vertebrae, in an otherwise normal neuroaxis and spinal column.

Conclusions: The case serves to highlight the importance of knowledge of this rare congenital condition to surgeons, physicians and radiologists involved in the care of both adult and paediatric patients presenting with spinal pathology.

Keywords: Adult; Diastematomyelia; Dysraphism; Split cord; Synkinesis.