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一名8岁男孩的大型青少年鼻咽血管纤维瘤侵犯颞下窝并累及颅内硬膜外鞍旁区域的手术治疗

Surgical management of large juvenile nasopharyngeal angiofibroma invading the infratemporal fossa with intracranial extradural parasellar involvement in an 8-year-old boy.

作者信息

Yamada Masato, Tsunoda Atsunobu, Hagino Koji, Aoyagi Masaru, Kawano Yoshihisa, Yano Tomoyuki, Tanaka Kentaro, Kishimoto Seiji

机构信息

Department of Otolaryngology, Tokyo Medical and Dental University, Japan.

出版信息

Auris Nasus Larynx. 2012 Jun;39(3):341-4. doi: 10.1016/j.anl.2011.07.017. Epub 2011 Aug 31.

Abstract

We present a rare case of a large juvenile nasopharyngeal angiofibroma (JNA) in an 8-year-old boy. Preoperative imaging revealed that the tumor had widely extended to the sphenoid sinus, infratemporal fossa, and cavernous sinus. Following embolization of the feeding vessels, the tumor was successfully removed by a combination of an orbitozygomatic approach and Le Fort I osteotomy under frontolateral craniotomy. An endoscope assisted in the surgery. At 15 months follow-up, the patient was free of the disease with no facial palsy, scars, or malocclusion. JNA is a benign tumor that typically affects adolescent males and is rarely observed during prepuberty. Complete removal of JNA by surgery, the initial therapy, is generally required. However, as in the present case, a large JNA with wide extension requires extended surgery, and such a surgery is more invasive for prepubertal patients. Using an appropriate combination of surgical approaches, a large JNA developed during prepuberty can be safely removed with reduced morbidity.

摘要

我们报告了一例罕见的8岁男孩巨大青少年鼻咽血管纤维瘤(JNA)病例。术前影像学检查显示肿瘤广泛延伸至蝶窦、颞下窝和海绵窦。在栓塞供血血管后,通过眶颧入路联合Le Fort I截骨术在前额开颅下成功切除肿瘤。手术中使用了内镜辅助。随访15个月时,患者无疾病复发,无面瘫、瘢痕或咬合不正。JNA是一种良性肿瘤,通常影响青春期男性,在青春期前很少见。手术作为初始治疗,一般需要完整切除JNA。然而,如本病例所示,广泛延伸的巨大JNA需要扩大手术范围,而这种手术对青春期前患者的侵袭性更大。采用适当的手术入路组合,青春期前发生的巨大JNA可以安全切除,同时降低发病率。

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