Mankotia Dipanker Singh, Satyarthee Guru Dutta, Singh Bhoopendra, Sharma Bhawani Shankar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
J Pediatr Neurosci. 2016 Oct-Dec;11(4):344-347. doi: 10.4103/1817-1745.199471.
Association of Dandy-Walker syndrome with occipital meningocele (OMC) is extremely rare and about thirty cases are reported till date in the Western literature. However, OMC is classified by Talamonti . into small, large, and giant categories with respective diameters were upto 5 cm in small, large with 5-9 cm, and giant with >9 cm. Usually the size of OMC progressively increases as raised intracranial pressure leads to compensatory cerebrospinal fluid escape into sac with the growth of children. Authors report an interesting case of an 18-month-old female child with extra-gigantic OMC, whose size was almost same since birth, representing the first case of its kind, who underwent successful surgical repair. Clinical presentation, radiological features, and surgical management options in literature are reviewed briefly for this rare disease association.
丹迪-沃克综合征与枕部脑膨出（OMC）的关联极为罕见，截至目前西方文献报道了约30例。然而，塔拉蒙蒂将OMC分为小、大、巨大三类，小的直径达5厘米，大的为5至9厘米，巨大的大于9厘米。通常，随着颅内压升高导致脑脊液代偿性地流入囊内，随着儿童生长，OMC的大小会逐渐增大。作者报告了一例有趣的18个月大女童患有超大OMC的病例，其大小自出生以来几乎相同，这是此类首例成功接受手术修复的病例。本文简要回顾了文献中关于这种罕见疾病关联的临床表现、影像学特征及手术治疗选择。
