Hayat Fakhar, Ismail Mohamed, Alqhtani Muhanned M, Almayman Talal, Sardar Noor, Ismaeel Abdullah, AlJohani Mohammed, Alruwaili Rayan S
Neurosurgery, King Hamad University Hospital, Busaiteen, BHR.
Neurological Surgery, King Hamad University Hospital, Busaiteen, BHR.
Cureus. 2023 Dec 10;15(12):e50262. doi: 10.7759/cureus.50262. eCollection 2023 Dec.
Dandy-Walker syndrome (DWS) is a rare congenital brain malformation defined by the presence of an expanded posterior fossa, full or partial absence of the cerebellar vermis, and a cystic expansion of the fourth ventricle. We report an 18-month-old girl with DWS presenting with atypical clinical manifestations and unusual symptoms. She initially presented with persistent vomiting and abdominal pain for four days, not responding to antiemetic medication. In addition, she was found to have abnormal postural arching of the back, extension of the lower limbs, and neck extension. MRI and CT head suggested Dandy-Walker syndrome with hydrocephalus (the lateral ventricle, third ventricle, and fourth ventricle are all significantly dilated with evidence of trans-ependymal cerebrospinal fluid permeation, severe compression anterior displacement of the brain stem). The patient underwent urgent, lifesaving right sub-occipital craniotomy, evacuation, and decompression of the posterior fossa cyst and external ventricular drain (EVD) insertion along with left supra-tentorial EVD insertion. A series of brain magnetic imaging and CT brain post-procedure studies showed a significant reduction in the size of the ventricular system and mass effect on the brain stem.
丹迪-沃克综合征（DWS）是一种罕见的先天性脑畸形，其特征为后颅窝扩大、小脑蚓部完全或部分缺失以及第四脑室囊性扩张。我们报告一名18个月大患有丹迪-沃克综合征的女孩，其临床表现不典型且症状异常。她最初持续呕吐和腹痛四天，使用止吐药物无效。此外，发现她有背部姿势性异常拱起、下肢伸直和颈部伸展。头部MRI和CT提示丹迪-沃克综合征合并脑积水（侧脑室、第三脑室和第四脑室均显著扩张，有经室管膜脑脊液渗透的证据，脑干严重受压并向前移位）。患者接受了紧急的、挽救生命的右枕下开颅手术，后颅窝囊肿引流和减压，并插入了外部脑室引流管（EVD），同时在左幕上插入了EVD。术后一系列脑部磁共振成像和脑部CT研究显示脑室系统大小显著减小，对脑干的占位效应减轻。
