Deghedy Marwa, Pizer Barry, Kumar Ram, Mallucci Conor, Avula Shivaram
Department of Oncology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK.
Department of Neurology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK.
Case Rep Pediatr. 2022 Feb 10;2022:9148100. doi: 10.1155/2022/9148100. eCollection 2022.
Post-operative cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome (PFS), is a well-recognized and frequent complication of surgery for posterior fossa tumours in children and young people. Its incidence varies between 8 and 31%, and the pathophysiological mechanisms of delayed onset and resolution of cerebellar mutism are not clear, but axonal damage, oedema, and perfusion defects may be involved. Magnetic resonance imaging has failed to reveal a universal anatomical substrate or a single definite mechanism of injury. We present a case of 16-year-old boy who developed CMS three days after resection of a medulloblastoma, a primary fourth ventricular tumour. Early post-operative imaging showed bleeding in the posterior fossa which required evacuation. CT angiography seven days after surgery demonstrated basilar artery vasospasm. Magnetic resonance brain angiography confirmed persistent narrowing of a segment of the basilar artery closely related to a left cerebellopontine (CP) angle peri-operative haematoma. The patient was treated with nimodipine and hypervolemia. The patient started vocalisation without speech five days later with reversal of radiological lesions. Further recovery of post-operative neurological deficits occurred over a protracted period of several months. This case represents a rare cause of post-operative CMS, with rapid initial recovery that occurred after specific treatment directed at the cause. To our knowledge, this is the first reported case showing mutism associated with basilar artery vasospasm with imaging evidence. This case may suggest the need to undertake urgent vascular imaging in selected cases of post-operative CMS.
术后小脑缄默综合征（CMS），也称为后颅窝综合征（PFS），是儿童和青少年后颅窝肿瘤手术中一种公认的常见并发症。其发生率在8%至31%之间，小脑缄默延迟发作和缓解的病理生理机制尚不清楚，但可能涉及轴突损伤、水肿和灌注缺陷。磁共振成像未能揭示普遍的解剖学基础或单一明确的损伤机制。我们报告一例16岁男孩，在髓母细胞瘤（一种原发性第四脑室肿瘤）切除术后三天出现CMS。术后早期影像学检查显示后颅窝出血，需要进行引流。术后七天的CT血管造影显示基底动脉血管痉挛。磁共振脑动脉造影证实与左小脑桥脑角（CP）角围手术期血肿密切相关的一段基底动脉持续狭窄。患者接受了尼莫地平和高血容量治疗。五天后患者开始发声但无言语，影像学病变逆转。术后神经功能缺损在数月的漫长时间里进一步恢复。该病例代表了术后CMS的一种罕见病因，在针对病因进行特定治疗后出现了快速的初始恢复。据我们所知，这是第一例有影像学证据显示与基底动脉血管痉挛相关的缄默症报告病例。该病例可能提示在某些术后CMS病例中需要进行紧急血管成像。
