Cervicomedullary decompression for foramen magnum stenosis in achondroplasia.
OBJECTIVE
Achondroplasia is the most common hereditary form of dwarfism, and is characterized by short stature, macrocephaly, and a myriad of skeletal abnormalities. In the pediatric population, stenosis and compression at the level of the cervicomedullary junction commonly occurs. The goal in this study was to assess the outcomes in children with achondroplasia who underwent cervicomedullary decompression.


METHODS
Forty-three pediatric patients with heterozygous achondroplasia and foramen magnum stenosis underwent 45 cervicomedullary decompressions at the authors' institution over an 11-year period. After surgical decompression, complete resolution or partial improvement in the preoperative symptoms was observed in all patients. There were no deaths in the treated patients. The surgical morbidity rate was low and usually consisted of a cerebrospinal fluid (CSF) leak in patients in whom the dura mater had been opened (either intentionally or accidentally). This problem was successfully managed in all cases with local measures (wound oversewing) or CSF diversion.


CONCLUSIONS
In this review the authors demonstrate that decompression of the cervicomedullary junction in the setting of achondroplasia may be accomplished safely with significant clinical benefit and minimal morbidity.
Department of Pediatric Neurosurgery, The Johns Hopkins University, Baltimore, Maryland 21287, USA.
Carlos A
CA
Bagley
Jonathan A
JA
Pindrik
Markus J
MJ
Bookland
Joaquin Q
JQ
Camara-Quintana
Benjamin S
BS
Carson
D000130:Achondroplasia / Q000150:complications*; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D019299:Decompression, Surgical / Q000379:methods*; D005260:Female; D005539:Foramen Magnum / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D009017:Morbidity; D019635:Neurosurgical Procedures / Q000379:methods*; D012189:Retrospective Studies