以眶尖综合征为首发症状的鼻-眶-颅底侵袭性曲霉菌感染5例

Five cases of invasive rhino-orbital-skull base aspergillosis with orbit apex syndrome initial presentation

目的:探讨以眶尖综合征为首发症状的免疫功能低下患者鼻-眶-颅底侵袭性曲霉菌感染的早期诊断、外科干预和全身抗真菌药物的应用。方法:回顾性分析5例以眶尖综合征为首发症状的鼻-眶-颅底侵袭性曲霉菌感染患者的临床资料、诊治经过、随访结果。结果:5例均以眶尖综合征为首发症状,无明显鼻-鼻窦症状,均首诊于神经内科。其中4例行内镜经鼻鼻窦-眶尖-颅底探查术,3例同时行视神经减压术。病理检查为侵袭性曲霉菌病变。1例术后即联合应用两性霉素B、伊曲康唑和卡泊净芬,术后第4天头疼明显缓解,术后3个月复查视力由术前1尺指数恢复至0.1,随访65个月症状得到控制。1例术后即开始应用两性霉素B,术后1个月头疼减轻,随访43个月视力无提高,病变无进行性发展。其余3例死亡,其中1例死于颅内真菌扩散,1例死于脑干出血,1例死于败血症。结论:眶尖综合征常是侵袭性鼻-眶-颅底曲霉菌病的早期临床表现。对免疫功能低下、以眶尖综合征为典型临床表现的患者应用类固醇激素治疗之前应考虑行内镜经鼻探查和活检,术中尽可能清除病变。早期诊断和外科干预有助于提高视力、缓解症状,该病预后差。

Objective：Although rare, invasive rhino-orbital-skull base aspergillosis usually is fatal and necessitates prompt diagnosis,surgical intervention and antifungal treatment.Method：A case series of 5 patients, with review of treatments and outcomes.Result：Five patients initially presented with orbital apex syndrome without apparent rhino-sinus disease.Histologic examination revealed invasive aspergillosis.Four patients underwent transnasal endoscopic surgical exploration of rhino-orbital-skull base, and three of them simultaneously underwent optic nerve decompression. One patient underwent the rhinopharyngeal biopsy. Following surgical debridement and antifungal drug, Two patients achieved various degree of recovery, 1 patient（case 5） showed vision improvement, from counting fingers to 0.1 and symptom-free during 65 months follow-up.One patient（case 1） showed no recovery of vision from no light perception, but symptom-free during 43 months follow-up.Other three patients respectively died of massive intracranial spread（case 3）, postoperative brainstem hemorrhage（case 2） and sepsis（case 4）.Conclusion：Invasive sino-orbital-skull base aspergillosis often may resemble inflammatory conditions. Early diagnosis and surgical intervention are required to improve vision and survival. The endoscopic surgical exploration and biopsy often are necessary to rule out fungal sinus infection before considering steroid use, especially in immunocompromised individuals. The patients with invasive rhino-orbital-skull base aspergillosis usually have a poor prognosis.