| Literature DB >> 36131570 |
Yiming Li1, Jiahe Guo1, Huijie Wei1, Cuiyun Sun1,2, Yan Chai3, Xiuwei Fu4, Kai Zhang5, Shengping Yu1, Xuejun Yang1.
Abstract
BACKGROUND: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is an extremely rare, slow-growing hereditary mass lesion that is mainly characterized by both specific neuroradiological features and secondary hydrocephalus. Patients may present with symptoms of cerebellar mass lesion and increased intracranial pressure. As an important part of Cowden syndrome, Lhermitte-Duclos disease in adults is typically marked by PTEN gene mutation. OBSERVATIONS: The clinical management of a 31-year-old woman who suffered Lhermitte-Duclos disease was introduced in this case report. Subtotal resection was performed with the assistance of intraoperative sonography to relieve obstructive hydrocephalus, and prophylactic C1 laminectomy was performed to prevent possible postoperative progression of the residual lesion. Perioperative care and surgical process were clearly revealed in an accompanying video. Intraoperative sonography of Lhermitte-Duclos disease presents hyperechoic distorted thickening cortices surrounded by hypoechoic edema belt. The patient did not report any significant neurological complications or sequelae after the lesion resection. LESSONS: The authors first reported the use of intraoperative sonography in resection of adult-onset Lhermitte-Duclos disease. Hopefully, the educative case report can provide a feasible experience in the diagnosis and treatment of Lhermitte-Duclos disease.Entities:
Keywords: Cowden syndrome; Lhermitte-Duclos disease; PTEN mutation; dysplastic gangliocytoma of the cerebellum; intraoperative sonography; surgical strategy
Year: 2021 PMID: 36131570 PMCID: PMC9563950 DOI: 10.3171/CASE21451
Source DB: PubMed Journal: J Neurosurg Case Lessons ISSN: 2694-1902