مجله جهانی جراحی پلاستیک
مجله جهانی جراحی پلاستیک
پنجشنبه 23 مهر 1405
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چکیده: (17 مشاهده)
Multiple Endocrine Neoplasia Type 2B (MEN2B) is a rare RET-associated syndrome characterized by mucosal neuromas, craniofacial anomalies, and life-threatening endocrine tumors. Reports of orthognathic surgery in the MEN2B population are extremely limited, despite the functional and aesthetic burden of skeletal deformities. This case highlights intraosseous neural hyperplasia of the jaws in MEN2B, a rare finding with direct implications for surgical planning and outcomes. A 20-year-old Iranian woman with genetically confirmed MEN2B, previously treated with thyroidectomy, presented for correction of severe craniofacial deformities. Clinical findings included double lips, mucosal neuromas, and hypertrophic eyelids. Cephalometric analysis revealed vertical maxillary excess, maxillary prognathism, and a Class II skeletal pattern. Following multidisciplinary evaluation, she underwent Le Fort I osteotomy, bilateral sagittal split osteotomy, and genioplasty. Intraoperatively, unusually dense cortical bone, cortical cracking, and intraosseous neural hyperplasia complicated fixation, requiring modified techniques. Orthognathic surgery in MEN2B patients may be complicated by dense, brittle cortical bone, intraosseous neural hyperplasia, and abundant submucosal soft tissue, which can affect osteotomy and fixation. Multidisciplinary preoperative evaluation, careful surgical planning, and flexible intraoperative techniques are essential to optimize outcomes in this rare population.
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