Todo T, Usui M, Araki F
Department of Neurosurgery, Aizu Central Hospital, Fukushima, Japan.
Neurol Med Chir (Tokyo). 1993 Dec;33(12):845-50. doi: 10.2176/nmc.33.845.
A boy was born with Dandy-Walker syndrome associated with a giant occipital meningocele, cleft lip, and cleft palate. The meningocele was actually a component of the giant posterior fossa cyst which communicated with the fourth ventricle. A cyst-peritoneal shunt achieved a considerable decrease in the size of the meningocele, but decubital ulcers developed due to restricted head movement caused by the occipital lesion. Cranioplasty removed a wide area of the inferior occipital bone, and the boundary between the superior occipital and parietal bones was thinned to allow free bending of the bone flap. The meningocele was removed totally in the third operation, but infection of the wound and pneumonia developed, causing death. The coexistence of Dandy-Walker syndrome and occipital meningocele, together with midline facial anomalies, may suggest a later pathogenesis of Dandy-Walker syndrome than previously believed.
一名男婴出生时患有丹迪-沃克综合征，伴有巨大枕部脑膨出、唇裂和腭裂。脑膨出实际上是与第四脑室相通的巨大后颅窝囊肿的一部分。囊肿-腹腔分流术使脑膨出的大小显著减小，但由于枕部病变导致头部活动受限，发生了褥疮性溃疡。颅骨成形术切除了枕骨下部的大片区域，枕骨上部和顶骨之间的边界变薄，以使骨瓣能够自由弯曲。在第三次手术中完全切除了脑膨出，但伤口感染和肺炎发生，导致死亡。丹迪-沃克综合征与枕部脑膨出以及中线面部异常并存，可能提示丹迪-沃克综合征的发病机制比以前认为的要晚。
