Orthognathic Surgery in Multiple Endocrine Neoplasia Type 2B Complicated by Intraosseous Neural Hyperplasia: A Case Report

Moradi , Ali; Jafarpour , Kimia; Pilehvar , Sara; Grillo , Ricardo; Samieirad , Sahand

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Orthognathic Surgery in Multiple Endocrine Neoplasia Type 2B Complicated by Intraosseous Neural Hyperplasia: A Case Report

Ali Moradi¹

, Kimia Jafarpour²

, Sara Pilehvar¹

, Ricardo Grillo³

, Sahand Samieirad ^*⁴

1- 1. Department of Oral and Maxillofacial Surgery, School of Dentistry, Mashhad University of Medical Sciences, Mashhad, Iran
2- 2. Dental Research Center, School of Dentistry, Mashhad University of Medical Sciences, Mashhad, Iran
3- 3. Department of Oral & Maxillofacial Surgery, Faculdade São Leopoldo Mandic, Campinas, Brazil
4- 1. Department of Oral and Maxillofacial Surgery, School of Dentistry, Mashhad University of Medical Sciences, Mashhad, Iran , samieerads@mums.ac.ir

Abstract: (16 Views)

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.

Keywords: Multiple endocrine neoplasia type 2b, Orthognathic Surgery, Osteotomy, Le Fort

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Type of Study: case report | Subject: Special