PDF(Pubmed)
Abstract：
Erdheim-Chester disease (ECD) is a rare systemic disease characterized by non-Langerhans histiocytosis. Pituitary involvement, often manifesting as diabetes insipidus, is the most common central nervous system (CNS) lesion. However, significant mass formation compressing the optic apparatus is rarely reported. We present a case of ECD-related suprasellar mass treated with an endoscopic transnasal approach, with emphasis on the surgical strategy and the intraoperative findings. The mass was fibrous, significantly hard, and strongly adhered to the optic nerves, causing visual impairment. A subtotal resection was performed with preserving the adhesion between the mass and the optic nerves, and her visual symptoms improved remarkably after surgery. We highlight the surgical procedure of ECD-related suprasellar mass, from an endoscopic point of view. Due to strong adhesion of the mass to the surrounding optic apparatus and perforators, complete resection may be harmful; judicious mass reduction with preserving such adhesion would contribute to better visual outcomes.
摘要：
Erdheim-Chester病（ECD）是一种罕见的全身性疾病，其特征是非朗格汉斯组织细胞增生症。垂体受累,经常表现为尿崩症，是最常见的中枢神经系统（CNS）病变。然而,压缩光学设备的大量质量形成很少报道。我们介绍了一例ECD相关的鞍上肿块，经鼻内窥镜入路治疗，重点是手术策略和术中发现。质量是纤维状的，非常困难，并牢固地粘附在视神经上，导致视力障碍。在保留肿块和视神经之间的粘连的情况下进行了次全切除术，手术后她的视觉症状明显改善.我们强调ECD相关鞍上肿块的外科手术，从内窥镜的角度来看。由于质量与周围光学设备和穿孔器的牢固粘附，完全切除可能是有害的;明智的肿块减少并保留这种粘连将有助于更好的视觉效果。
