title: Severe snoring in a child with pycnodysostosis treated with a bilateral rib graft.
abstract: Pycnodysostosis is a rare osteopetrotic disorder. This report describes a case of a 3(1/2)-year-old boy with pycnodysostosis who presented with severe snoring. The snoring was the result of a pharyngeal narrowing due to a hypoplastic mandible. Surgery consisted of a bilateral rib graft, which allowed an enlargement of the pharynx by acting both on an anterior projection of the chin and a decrease of the glossoptosis. A significant reduction of the snoring and an excellent mouth aperture were obtained. This technique is ideal for such patients presenting a high risk of infection, nonunion, and secondary fracture.
authors.affiliation: Head and Neck Department, Rebert Debré Hospital, Paris, France.
authors.forename: Natacha
authors.initials: N
authors.lastname: Teissier
authors.forename: Marie-Line
authors.initials: ML
authors.lastname: Jacquemont
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Blancal
authors.forename: Monique
authors.initials: M
authors.lastname: Elmaleh-Bergès
authors.forename: Thierry
authors.initials: T
authors.lastname: Van Den Abbeele
authors.forename: Selim
authors.initials: S
authors.lastname: Bennaceur
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000601:surgery; D016025:Bone Transplantation / Q000379:methods*; D002675:Child, Preschool; D006801:Humans; D008297:Male; D008334:Mandible / Q000601:surgery*; D008342:Mandibulofacial Dysostosis / Q000150:complications* / Q000601:surgery*; D019647:Oral Surgical Procedures / Q000379:methods; D010614:Pharynx / Q000473:pathology; D012272:Ribs / Q000637:transplantation; D012913:Snoring / Q000209:etiology / Q000601:surgery*