Fadle Khalid Nasser, Hassanein Ahmed Gaber, Kasim Abdin K
*Neurosurgery Department†Maxillofacial Surgery Unit, Faculty of Medicine, Sohag University, Sohag, Egypt.
J Craniofac Surg. 2016 Nov;27(8):e719-e723. doi: 10.1097/SCS.0000000000003033.
Fibrous dysplasia (FD) is a non-neoplastic developmental fibro-osseous disease. It represents 2.5% of all bone tumors and 5% to 7% of the benign bone tumors. Orbitocranial region is involved in about 20% of the patients. The main presentations are craniofacial deformity and headache. Loss of vision is the most devastating result of this disease. There is no medical treatment to cure or prevent FD. Radiation therapy is contraindicated. Surgery for the orbitocranial FD is often challenging because of the proximity of neurovascular and ocular structures. Conservative surgical shaving and recontouring is always associated with suboptimal results. Radical excision is potentially curative with no extra morbidity. Orbital hypertelorism, dystopia, or proptosis can be corrected only by radical excision and reconstruction.
The aim of the study was to evaluate the outcome of radical excision of the orbitocranial FD and immediate reconstruction using titanium mesh and pericranial flap.
This prospective study had been conducted on 22 patients with orbitocranial FD with age range from 17 to 52 years (mean 29.5). Radical excision of the lesions was done for all patients through transcranial approach. Immediate reconstruction was achieved using titanium mesh and pericranial flap.
Intraoperative dural tears and cerebrospinal fluid leak were reported in 2 patients and repaired with galeal graft. Supraorbital anesthesia occurred in 6 patients. Of these, 2 patients were transient, while the remaining 4 patients were permanent. Wound infection was noticed in 1 patient who improved by medical treatment. Temporary postoperative diplopia occurred in 1 patient and temporary postoperative impaired vision in 1 other patient. In all patients, acceptable or good aesthetic results were observed. No recurrence was detected in our series during the follow-up period that ranged from 24 to 58 months (mean 37.5 months).
Radical excision of orbitocranial FD is potentially curative with no extra morbidity. It can achieve good aesthetic and functional results with no recurrence.
骨纤维异常增殖症(FD)是一种非肿瘤性发育性纤维-骨疾病。它占所有骨肿瘤的2.5%,良性骨肿瘤的5%至7%。眶颅区域受累的患者约占20%。主要表现为颅面畸形和头痛。视力丧失是该疾病最严重的后果。目前尚无治愈或预防FD的药物治疗方法。放射治疗是禁忌的。由于神经血管和眼部结构相邻,眶颅FD的手术治疗往往具有挑战性。保守的手术刮除和重塑效果总是不尽人意。根治性切除可能治愈疾病且不会增加额外的发病率。只有通过根治性切除和重建才能纠正眶距增宽、异位或眼球突出。
本研究的目的是评估眶颅FD根治性切除并立即使用钛网和颅骨膜瓣重建的效果。
本前瞻性研究对22例年龄在17至52岁(平均29.5岁)的眶颅FD患者进行。所有患者均通过经颅入路对病变进行根治性切除。立即使用钛网和颅骨膜瓣进行重建。
2例患者术中出现硬脑膜撕裂和脑脊液漏,用帽状腱膜移植修复。6例患者出现眶上神经麻醉。其中,2例为短暂性,其余4例为永久性。1例患者出现伤口感染,经药物治疗后好转。1例患者术后出现暂时性复视,另1例患者术后出现暂时性视力受损。所有患者均观察到可接受或良好的美学效果。在随访期24至58个月(平均37.5个月)内,我们的系列研究中未检测到复发。
眶颅FD根治性切除可能治愈疾病且不会增加额外的发病率。它可以实现良好的美学和功能效果,且无复发。