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Sublabial Approach for the Treatment of Symptomatic Basilar Impression in a Patient With Klippel-Feil Syndrome

Bettegowda, Chetan MD, PhD*; Shajari, Mehdi BS‡; Suk, Ian BMc, BMC*; Simmons, Oliver P MD‡; Gokaslan, Ziya L MD*; Wolinsky, Jean-Paul MD*

doi: 10.1227/NEU.0b013e3182160709
Operative Technique

BACKGROUND: Basilar impression (BI) is an uncommon condition in which there is upward displacement of the elements forming the foramen magnum, causing translocation of vertebral elements into the brainstem. Most commonly a developmental anomaly, BI is often associated with congenital conditions such as Down syndrome. Symptomatic BI is often difficult to treat surgically secondary to the anatomic variants associated with many of the coinciding congenital syndromes.

OBJECTIVE: To present a feasible approach for the treatment of BI.

METHODS: We present an alternative surgical approach for the treatment of symptomatic BI in a 37-year-old woman with Klippel-Feil syndrome. Because of the altered anatomy, traditional approaches such as the transoral-transpharyngeal, transmandibular circumglossal, and transcervical endoscopic routes were not feasible.

RESULTS: We chose a staged sublabial, transnasal, transpalatal route for the anterior brainstem decompression followed by posterior fixation. The patient tolerated the procedures well and at last follow-up had nearly complete resolution of symptoms.

CONCLUSION: The sublabial route is an alternative approach for anterior decompression in patients with symptomatic basilar impression and altered anatomic circumstances such as that caused by Klippel-Feil syndrome.

Departments of *Neurosurgery and ‡Plastic Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland

Received, June 14, 2010.

Accepted, January 4, 2011.

Published Online, March 15, 2011.

Correspondence: Chetan Bettegowda, MD, PhD, Department of Neurosurgery, Johns Hopkins School of Medicine, 600 N Wolfe St, Meyer 8-161, Baltimore, MD 21287. Email:

Copyright © by the Congress of Neurological Surgeons