Shiohama Tadashi, Ando Ryo, Fujii Katsunori, Mukai Hiroki, Naruke Yuki, Sugita Katsuo, Kato Eiji, Shimojo Naoki
Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan.
Division of Pediatric Neurosurgery, Chiba Children's Hospital, Chiba, Japan.
Case Rep Pediatr. 2017;2017:3861608. doi: 10.1155/2017/3861608. Epub 2017 Oct 25.
Dandy-Walker malformation (DWM) is a posterior fossa anomaly characterized by hypoplasia and upward rotation of the cerebellar vermis and cystic dilation of the fourth ventricle. The cyst of DWM rarely extends posteriorly to almost completely fill the entire posterior fossa, which mimics primary cerebellar agenesis, a cerebellar porencephalic cyst, and an arachnoid cyst due to the lack of clarity of the thin cystic wall. A 10-month-old female born at 23 weeks' gestation with cerebellar hemorrhage in the neonatal period was admitted to our hospital with dysphagia and side-to-side head bobbing. The detection of hemosiderin deposits enveloping the cyst wall by T2 star-weighted angiography (SWAN) was useful for the differential diagnosis of an acquired form of DWM from primary cerebellar agenesis. Cyst fenestration successfully improved dysphagia and head bobbing. A pathological specimen of the perforated cyst consisted of collagen fibers with hemosiderin deposits but lacked congenital cyst components. In infants with posterior fossa cysts, SWAN will be useful for a differential diagnosis between DWM and primary cerebellar agenesis.
丹迪-沃克畸形（DWM）是一种后颅窝异常，其特征为小脑蚓部发育不全并向上旋转以及第四脑室囊性扩张。DWM的囊肿很少向后延伸至几乎完全填满整个后颅窝，由于薄囊壁不清晰，这会模仿原发性小脑发育不全、小脑穿通性囊肿和蛛网膜囊肿。一名在孕23周出生的10个月大女性，新生儿期有小脑出血，因吞咽困难和头部左右摆动入住我院。通过T2*加权血管造影（SWAN）检测到囊肿壁周围有含铁血黄素沉积，这有助于鉴别后天性DWM与原发性小脑发育不全。囊肿开窗术成功改善了吞咽困难和头部摆动。穿孔囊肿的病理标本由含有含铁血黄素沉积的胶原纤维组成，但缺乏先天性囊肿成分。对于患有后颅窝囊肿的婴儿，SWAN有助于鉴别DWM和原发性小脑发育不全。
