title: Craniovertebral junction anomalies in achondroplastic children.
abstract: The natural history of CVJ stenosis in achondroplastic children is only partially known. Achondroplastic children have a foramen magnum that is significatively smaller at birth, and it does not follow the normal growth during the first 18 months of life, leading to CVJ stenosis and, for the most severe of them, to neurological and developmental impairment and delay and even sudden death due to cervicomedullary narrowing. We reviewed our experience based on 37 patients operated for cervicomedullary decompression between 1970 and 2010 and performed a literature review. The indication for surgery should be taken on very strict clinical and radiological parameters as well as sleep studies. Under those criteria, surgical decompression of CVJ leads to neurological and developmental improvement, despite non-negligible mortality and morbidity.
authors.affiliation: Pediatric Neurosurgical Departement, Hôpital Necker-Enfants Malades, APHP, 149, Rue de Sèvres, Paris, 75743, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Reina
authors.forename: Genevieve
authors.initials: G
authors.lastname: Baujat
authors.forename: Brigitte
authors.initials: B
authors.lastname: Fauroux
authors.forename: Vincent
authors.initials: V
authors.lastname: Couloigner
authors.forename: Elise
authors.initials: E
authors.lastname: Boulanger
authors.forename: Christian
authors.initials: C
authors.lastname: Sainte-Rose
authors.forename: Philippe
authors.initials: P
authors.lastname: Maroteaux
authors.forename: Martine Le
authors.initials: ML
authors.lastname: Merrer
authors.forename: Valérie
authors.initials: V
authors.lastname: Cormier-Daire
authors.forename: Laurence
authors.initials: L
authors.lastname: Legai-Mallet
authors.forename: Michel
authors.initials: M
authors.lastname: Zerah
authors.forename: Federico
authors.initials: F
authors.lastname: Di Rocco
mesh_terms: D000130:Achondroplasia; D002648:Child; D003251:Constriction, Pathologic; D019299:Decompression, Surgical; D005539:Foramen Magnum; D006801:Humans; D009017:Morbidity