Purpose

Labrune syndrome (LS) is a rare white matter disease characterized by leukoencephalopathy with intracranial calcification and cysts (LCC). While the intracranial cyst characteristics of LS are for the most part stable, some may require surgical intervention when they develop associated hemorrhage and/or mass effect. To date, no review of the surgical outcomes of cyst decompression in LS has been performed.

Case presentation

We report the case of a 16-year-old girl with LS who presented with progressive right hemiparesis from an enlarging hemorrhagic left thalamic cyst. The patient underwent frameless stereotactic cyst aspiration and Ommaya reservoir placement and her hemiparesis subsequently improved. Serial monitoring demonstrated stable decompression of the cyst.

Conclusions

The pathophysiology of LS is thought to be diffuse cerebral microangiopathy and it is thought that these microhemorrhages contribute to the formation of intracranial cysts as well as diffuse calcifications. Indications for surgical intervention in LS are not well established and the heterogeneity of lesions compels them to be managed on a case-by-case basis. Based on our literature review, surgery is the standard treatment of choice for patients with progressive symptoms and growing lesions on imaging studies, with outcomes favoring less-invasive stereotactic approaches with contingencies of reservoir placement when cysts recur.

中文翻译：

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Labrune综合征的手术考虑

目的

Labrune综合征（LS）是一种罕见的白质病，其特征是白脑病伴颅内钙化和囊肿（LCC）。尽管LS的颅内囊肿特征在大多数情况下是稳定的，但当它们出现相关的出血和/或肿块效应时，可能需要手术干预。迄今为止，尚未对LS中囊肿减压的手术结局进行任何回顾。

案例介绍

我们报道了一名16岁女孩LS的病例，该女孩因出血性左丘脑囊肿扩大而出现进行性右偏瘫。患者接受了无框立体定向囊肿抽吸和Ommaya储液器放置，其偏瘫随后得到改善。连续监测显示囊肿稳定减压。

结论

LS的病理生理学被认为是弥漫性脑微血管病，并且认为这些微出血有助于颅内囊肿的形成以及弥漫性钙化。LS手术干预的指征尚不明确，病变的异质性迫使他们必须根据具体情况进行管理。根据我们的文献综述，对于影像学研究中具有进行性症状和病灶不断增长的患者，手术是标准的治疗选择，其结局倾向于采用侵入性较小的立体定向方法，并在囊肿复发时可以进行储液器放置。