Fronto-orbital advancement, Le Fort III distraction osteogenesis, and bimaxillary orthognathic jaw surgery with and without osteochondral graft treatment approaches of three siblings with Crouzon’s syndrome

2017 
Abstract Objectives Crouzon’s syndrome (CS) is a rare autosomal dominant, first branchial arch disorder characterized by craniosynostosis, most commonly coronal and sagittal, leading to distinctive malformations of the skull (brachycephaly) and face (exophthalmos, maxillary hypoplasia, mandibular prognathism), with obstructive sleep apnoea (OSA) and raised intra cranial pressure (ICP). We present three siblings affected by CS with various degree of severity and the appropriate treatments carried out for the correction of the ensued afflictions. The objectives are; (1) to describe the various treatment approaches of three siblings affected by various degree of CS and, (2) to emphasize that multidisciplinary approach is deemed essential in the successful management of the syndrome. Methods Based on the severity of the presentation various surgical interventions are planned; from fronto-orbital advancement to relieve raised ICP, Le Fort III midfacial distraction for the correction of exophthalmos and maxillary hypoplasia, and OSA; to bimaxillary orthognathic surgery with osteochondrol bone harvest for the correction of prognathic mandible and hypolastic maxilla. Conclusion To our knowledge, this is the first crouzon’s syndrome case series showing comprehensive treatment of three siblings affected by varying degree of severity. A thorough planning along with phased treatment is essential for successful management of Crouzon’s syndrome.
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