Split cord malformation (SCM) is a rare abnormality of notochord development. The majority of cases occur in the thoracolumbar region, with more than 30 cases of cervical SCM reported. The clinical impact of SCMs involving the cervical cord is therefore largely unknown. In addition, the concomitant finding of brainstem involvement is presumably incompatible with life in the majority of patients, resulting in a paucity of data regarding this clinical scenario. In this paper the authors present the first case, to their knowledge, of an incomplete cervical SCM involving the brainstem and discuss its clinical impact, diagnosis, and management.
Keywords: AHI = apnea-hypopnea index; BAER = brainstem auditory evoked response; FEES = functional endoscopic examination of swallowing; SCM = split cord malformation; TCS = tethered cord syndrome; brainstem hypoplasia; cervical cord malformation; diplomyelia; duplicated spinal cord; spine; split cord malformation; unilateral vocal cord palsy.