Klippel-Feil syndrome in association with Chiari zero malformation in the setting of acute traumatic spinal cord injury

• Published in: Interdisciplinary neurosurgery : Advanced techniques and case management 2017-09, Vol.9, p.1-3
• Main Authors: McDougall, Cameron M, M.D. FRCS(C), Alarfaj, Abdullah K, M.D, Jack, Andrew S, M.D, Souster, Jenny, M.D. FRCS(C), Broad, Robert W, M.D. FRCS(C)
• Format: Article
• Language: English
• Subjects: Medical Education; Neurosurgery
• ISSN: 2214-7519; 2214-7519
• DOI: 10.1016/j.inat.2016.11.001

Abstract Background This case is the first report of a Chiari type zero in association with Klippel-Feil syndrome (KFS). There is a paucity of literature surrounding the management of Chiari malformations in the setting of acute spinal cord injury. Case description An 18 year old male was transferred to our institution with an acute spinal cord injury following an all-terrain-vehicle accident. Imaging demonstrated both KFS and a crowded posterior fossa with a large cervical syrinx. The patient was initially an ASIA (American spinal injury association) grade A and was managed conservatively. He slowly improved and at 1 month post injury underwent a successful posterior fossa decompression. The patient continued to improve and the syrinx regressed. At the time of discharge to a rehabilitation facility the patient was an ASIA grade D, though not able to ambulate. Conclusion This is the first report of a Chiari zero malformation associated with KFS. This patient was managed with an initial conservative approach and delayed posterior fossa decompression. There was steady improvement in his neurological function. This appears to represent a viable treatment approach.