SURGICAL MANAGEMENT OF A PATIENT WITH CROUZON SYNDROME – CASE REPORT

Abstract

Crouzon syndrome is an autosomal dominant inheritance that affects the FGFR2 receptors, manifesting as craniosynostosis of the coronal and sagittal sutures. This clinical case reports the treatment of 47-year-old patient C.A.P. whose main complaint was related to obstructive sleep apnea. He reported other syndromic characteristics such as phonetic difficulty, respiratory and masticatory pain, bilateral pain in the temporomandibular joint, and headaches. Clinically, maxillary hypoplasia was observed, associated with exophthalmos and mandibular prognathism. The patient had a class III dentofacial deformity, also seen in lateral teleradiograph and panoramic radiograph. Polysomnography revealed moderate sleep apnea. The patient was surgically treated in 2 stages: distraction of the maxilla using an internal distracter device and, later, bimaxillary orthognathic surgery. New lateral and panoramic radiography and orthodontic finishing were performed. The patient reported obstructive sleep apnea resolution with improvement in masticatory, phonetic, and respiratory functions as well as facial harmonization with correction of gnathic bones. Crouzon syndrome is an autosomal dominant inheritance that affects the FGFR2 receptors, manifesting as craniosynostosis of the coronal and sagittal sutures. This clinical case reports the treatment of 47-year-old patient C.A.P. whose main complaint was related to obstructive sleep apnea. He reported other syndromic characteristics such as phonetic difficulty, respiratory and masticatory pain, bilateral pain in the temporomandibular joint, and headaches. Clinically, maxillary hypoplasia was observed, associated with exophthalmos and mandibular prognathism. The patient had a class III dentofacial deformity, also seen in lateral teleradiograph and panoramic radiograph. Polysomnography revealed moderate sleep apnea. The patient was surgically treated in 2 stages: distraction of the maxilla using an internal distracter device and, later, bimaxillary orthognathic surgery. New lateral and panoramic radiography and orthodontic finishing were performed. The patient reported obstructive sleep apnea resolution with improvement in masticatory, phonetic, and respiratory functions as well as facial harmonization with correction of gnathic bones.