Son Ji H, Marshall Danielle C, Valiathan Manish, Otteson Todd, Ferretti Gerald, Grigorian Paula A, Rosen Carol, Becker Devra, Rowe David, Soltanian Hooman, Lakin Gregory
1 Division of Plastic and Reconstructive Surgery, University Hospitals Case Medical Center, Cleveland, OH, USA.
2 Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Cleft Palate Craniofac J. 2019 Jan;56(1):90-93. doi: 10.1177/1055665618774008. Epub 2018 May 22.
Cherubism is an autosomal dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration and fibrous tissue hyperplasia. Conservative management is the preferred treatment as cherubism has a self-limiting course. Functional or emotional disturbances may, however, demand surgical intervention. We report a patient who underwent surgical intervention.
METHOD/DESCRIPTION: He had significant enlargement of lower cheeks and bilateral lower lid scleral show. On computed tomography of the face, the patient had significant fibrous tissue involving bilateral maxilla and mandible. The mandibular tumor was excised. Given normal inferior border, bilateral sagittal split osteotomy was performed to infracture and inset the outer cortex. During the procedure, patient required blood transfusion intraoperatively, so the maxillary portion of the procedure was delayed until 6 months later. For the maxilla, bilateral transconjunctival approach was used to resect parts of the orbital floors that were concave, resulting in 1 × 2 cm defects bilaterally which were reconstructed using resorbable plates. Then the anterior maxillary tumor was excised.
The patient and his parents were satisfied with his appearance after surgery. The patient was noted to have improvement in contour and decreased scleral show. He has most recently followed up 15 months after the initial surgery. There were no long-term complications.
Severity of cherubism influences the type of surgical intervention. The present case is innovative because this is the first reported case of recontouring orbital floors with resorbable plates and infracturing of the mandible using sagittal split osteotomies for surgical treatment of cherubism.
cherubism是一种常染色体显性综合征，其特征为双侧上颌下颌骨过度骨质退化和纤维组织增生。由于cherubism具有自限性病程，保守治疗是首选治疗方法。然而，功能或情绪障碍可能需要手术干预。我们报告了一名接受手术干预的患者。
方法/描述： 他的下脸颊明显肿大，双侧下睑巩膜外露。面部计算机断层扫描显示，患者双侧上颌骨和下颌骨有大量纤维组织。切除了下颌肿瘤。鉴于下颌下缘正常，进行了双侧矢状劈开截骨术以骨折并嵌入外侧皮质。手术过程中，患者术中需要输血，因此上颌部分的手术推迟到6个月后进行。对于上颌骨，采用双侧经结膜入路切除部分凹陷的眶底，导致双侧出现1×2 cm的缺损，使用可吸收板进行重建。然后切除上颌前部肿瘤。
患者及其父母对术后外观满意。患者的轮廓得到改善，巩膜外露减少。他最近一次随访是在初次手术后15个月。没有长期并发症。
cherubism的严重程度会影响手术干预的类型。本病例具有创新性，因为这是首例报道的使用可吸收板重塑眶底并通过矢状劈开截骨术骨折下颌骨来手术治疗cherubism的病例。
