Parikh Parth, Nayak Naren, Goyal Shubham, Ghosh Neelanjana, Pandey Rahul
Department of Neurosurgery, Lokmanya Tilak Municipal General Hospital, Mumbai, Maharashtra, India.
Department of General Surgery, Pandit Deen Dayal Upadhyay Medical College, Rajkot, Gujarat, India.
Asian J Neurosurg. 2025 Mar 18;20(2):413-416. doi: 10.1055/s-0045-1805088. eCollection 2025 Jun.
Lhermitte-Duclos disease (LDD) is a rare, autosomal dominant, dysplastic gangliocytoma of the cerebellum. It is a slow-growing benign tumor. The challenges in the surgical resection of these tumors lie in accurately delineating the tumor margin from the normal cerebellar parenchyma. Intraoperative ultrasound has the potential to overcome these limitations. A 30-year-old woman was diagnosed as having LDD showing a typical "tigroid" appearance on MRI. Intraoperative ultrasound was used to delineate the tumor margins and near-total resection was done via right suboccipital craniectomy. The diagnosis was confirmed on histopathological examination. Postoperatively there were no neurological deficits, and the patient is on regular follow-up for screening of Cowden's syndrome. We report this case to highlight the undervalued utility of intraoperative ultrasonography while dealing with patients with LDD. This modality serves as an effective tool to maximize the extent of resection without adding to postoperative morbidity.
Lhermitte-Duclos病(LDD)是一种罕见的常染色体显性遗传性小脑发育异常性神经节细胞瘤。它是一种生长缓慢的良性肿瘤。这些肿瘤手术切除的挑战在于准确区分肿瘤边界与正常小脑实质。术中超声有可能克服这些限制。一名30岁女性被诊断为LDD,磁共振成像(MRI)显示典型的“虎斑样”外观。术中使用超声来勾勒肿瘤边界,并通过右枕下颅骨切除术进行了近全切除。组织病理学检查证实了诊断。术后无神经功能缺损,患者正在定期随访以筛查考登综合征。我们报告此病例以强调术中超声在处理LDD患者时被低估的效用。这种方法是一种有效的工具,可在不增加术后发病率的情况下最大限度地扩大切除范围。