Encephaloceles are cystic congenital malformations in which central nervous system (CNS) structures, in communication with cerebrospinal fluid (CSF) pathways, herniate through a defect in the cranium. Hydrocephalus occurs in 60–90% of patients with occipital encephaloceles. Assessment of the surgical management of hydrocephalus associated with occipital encephalocele and its effect on the clinical outcome. Between October 2015 and October 2019, a retrospective study was conducted on seventeen children with occipital encephaloceles who were operated upon. The presence of progressive hydrocephalus was determined by an abnormal increase in head circumference and an increase in the ventricular size on imaging studies. A ventriculoperitoneal (VP) shunt was applied in patients who had hydrocephalus. The clinical outcome was graded according to the developmental milestones of the children on outpatient follow-up visits. The mean age at surgery was 1.6 (range, 0–15) months. There were ten girls (58.8%) and seven boys (41.2%). Ten encephaloceles (58.8%) contained neural tissue. Ten patients (58.8%) had associated cranial anomalies. Eleven children (64.7%) had associated hydrocephalus: four of them (36.4%) diagnosed preoperatively, while seven children (63.6%) developed hydrocephalus postoperatively. Ten of them (90.9%) were managed by VP shunt. All children with hydrocephalus had some degree of developmental delay, including six (54.5%) with mild/moderate delay and five (45.5%) with severe delay. Half of the patients (50%) of the children with occipital encephalocele without hydrocephalus had normal neurological outcome during the follow-up period (p value= 0.034). Occipital encephalocele is often complicated by hydrocephalus. The presence of hydrocephalus resulted in a worse clinical outcome in children with occipital encephalocele, so it can help to guide prenatal and neonatal counseling.

中文翻译：

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与枕部脑膨出相关的脑积水：手术治疗和临床结果

脑膨出是囊性先天性畸形，其中中枢神经系统 (CNS) 结构与脑脊液 (CSF) 通路连通，通过颅骨中的缺陷突出。60-90% 的枕部脑膨出患者会出现脑积水。评估与枕部脑膨出相关的脑积水的手术治疗及其对临床结果的影响。2015 年 10 月至 2019 年 10 月期间，对 17 名接受手术的枕部脑膨出儿童进行了回顾性研究。进行性脑积水的存在是由头围异常增加和成像研究中脑室大小增加确定的。脑室腹腔 (VP) 分流术适用于脑积水患者。根据门诊随访时儿童的发育里程碑对临床结果进行分级。手术时的平均年龄为 1.6（范围，0-15）个月。有十个女孩（58.8%）和七个男孩（41.2%）。10 个脑膨出 (58.8%) 包含神经组织。10 名患者 (58.8%) 有相关的颅骨异常。11 名儿童 (64.7%) 合并脑积水：其中 4 名 (36.4%) 术前确诊，7 名儿童 (63.6%) 术后出现脑积水。其中 10 个 (90.9%) 由 VP 分流管理。所有脑积水儿童都有一定程度的发育迟缓，其中 6 名 (54.5%) 轻度/中度迟缓，5 名 (45.5%) 重度发育迟缓。在没有脑积水的枕部脑膨出儿童中，有一半 (50%) 的患者在随访期间神经功能正常（p 值 = 0.034）。枕部脑膨出常并发脑积水。脑积水的存在导致枕部脑膨出患儿的临床结果较差，因此有助于指导产前和新生儿咨询。