Endoscopic Transsphenoidal Resection of Suprasellar Histiocytosis in a Patient with Erdheim-Chester Disease: A Case Report.

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-re

SUBMITTER: Hong S 

PROVIDER: S-EPMC8769389 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature