An unusual case of oromandibular-limb hypogenesis syndrome
Date
2008
Authors
Perks T.J.
Van Der Walt J.C.
Levin A.I.
Graewe F.R.
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Journal ISSN
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Abstract
The oromandibular-limb hypogenesis syndrome is a rare complex of jaw and limb defects with unclear aetiology. Multiple and variable deformities of the mandible, maxilla and tongue may occur in combination with a variety of limb defects. The wide range and combination of anomalies make classification difficult. A patient with all of the features of oromandibular-limb hypogenesis syndrome (except cranial nerve palsy) is presented. Despite severe micrognathia, synostosis of the jaws and soft-tissue synechiae between the hypoplastic tongue and the cleft palate, this patient had no respiratory difficulty and thrived with nasogastric feeding. The surgical management is discussed with emphasis given to the difficult airway and restoration of normal function of the tongue and jaws. © 2008 Springer-Verlag.
Description
Keywords
Africa, article, case report, cleft palate, clinical feature, cranial nerve paralysis, human, hypoplasia, infant, jaw malformation, jaw synostosis, limb defect, male, micrognathia, nose feeding, oromandibular limb hypogenesis syndrome, physical examination, priority journal, surgical technique, tongue hypoplasia
Citation
European Journal of Plastic Surgery
31
5
31
5