title: Clinical and surgical management of a congenital Type II split cord malformation presenting with progressive cranial neuropathies: case report.
abstract: 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.
authors.affiliation: Departments of 1 Neurosurgery.
authors.forename: Patrick R
authors.initials: PR
authors.lastname: Maloney
authors.affiliation: Departments of 1 Neurosurgery.
authors.forename: Meghan E
authors.initials: ME
authors.lastname: Murphy
authors.affiliation: Otolaryngology, and.
authors.forename: Molly J
authors.initials: MJ
authors.lastname: Sullan
authors.affiliation: Otolaryngology, and.
authors.forename: Kathryn M
authors.initials: KM
authors.lastname: Van Abel
authors.affiliation: Otolaryngology, and.
authors.forename: Shelagh A
authors.initials: SA
authors.lastname: Cofer
authors.affiliation: Pathology, Mayo Clinic School of Medicine, Rochester, Minnesota.
authors.forename: John C
authors.initials: JC
authors.lastname: Cheville
authors.affiliation: Departments of 1 Neurosurgery.
authors.forename: Nicholas M
authors.initials: NM
authors.lastname: Wetjen
mesh_terms: D003389:Cranial Nerve Diseases / Q000000981:diagnostic imaging* / Q000601:surgery*; D003937:Diagnosis, Differential; D019468:Disease Management; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D009436:Neural Tube Defects / Q000000981:diagnostic imaging* / Q000601:surgery*; D013118:Spinal Cord Diseases / Q000000981:diagnostic imaging / Q000601:surgery
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