Bassily Ramy, Wallace Daniel, Liolios Vasilis, Prinsely Peter, Beigi Bijan
Department of Ophthalmology, Norfolk and Norwich University Hospital NHS Foundation Trust, Norwich, Norfolk, United Kingdom.
Orbit. 2013 Jun;32(3):187-9. doi: 10.3109/01676830.2013.771678. Epub 2013 Mar 12.
To report a rare case of a fronto-ethmoidal mucocele secondary to an ethmoidal schwannoma. To our knowledge this is the first reported case of an ethmoidal schwannoma, a tumour of the peripheral nerve sheath originating from an area not believed to contain peripheral nerves that has presented as a fronto-ethmoidal mucocele.
Our patient is a 23-year-old male presented with a one-year history of progressive proptosis and vertical diplopia with restriction of upgaze. Orbital imaging demonstrated a mass in the right medial ethmoidal air cells extending to the frontal sinus and orbit, consistent with a mucocele. Patient underwent endonasal decompression of the right fronto-ethmoidal mucocele. Histology confirmed a schwannoma and repeat imaging post-operatively revealed residual mass originating from the ethmoidal air cells. A right upper eyelid skin crease approach anterior orbitotomy was performed to successfully excise the mass via the lamina papyracea. Post-operatively the patient's proptosis and diplopia resolved, with a full range of ocular movements. Post-operative imagining at 3 months did not show any residual tumour.
Mucocele formation may be secondary to an underlying schwannoma obstructing the fronto-ethmoidal foramen. In such cases we recommend an open-sky technique for full visualisation and improved chance of total removal.
报告一例罕见的筛窦神经鞘瘤继发额筛窦黏液囊肿病例。据我们所知,这是首例报告的筛窦神经鞘瘤病例,该肿瘤起源于一个通常认为不含周围神经的区域,却表现为额筛窦黏液囊肿。
我们的患者是一名23岁男性,有一年渐进性眼球突出和垂直复视伴上视受限病史。眼眶影像学检查显示右侧筛窦气房有一肿物延伸至额窦和眼眶,符合黏液囊肿表现。患者接受了右侧额筛窦黏液囊肿鼻内减压术。组织学检查证实为神经鞘瘤,术后复查影像学显示仍有残留肿物起源于筛窦气房。遂行经右上睑皮肤皱褶入路的前路眼眶切开术,通过纸样板成功切除肿物。术后患者的眼球突出和复视消失,眼球运动范围正常。术后3个月的影像学检查未显示任何残留肿瘤。
黏液囊肿的形成可能继发于潜在的神经鞘瘤阻塞额筛孔。在这种情况下,我们建议采用开放式手术技术,以实现全面可视化并提高完全切除的几率。