Association for Assistance of Disabled Children, Recife, Pernambuco, Brazil.
Barão de Lucena Hospital, Recife, Pernambuco, Brazil.
JAMA Neurol. 2019 Feb 1;76(2):203-210. doi: 10.1001/jamaneurol.2018.3553.
Hydrocephalus is a treatable but potentially fatal complication that has not been previously described in congenital Zika syndrome (CZS).
To describe the clinical features and imaging findings in 24 patients with congenital Zika syndrome (CZS) who developed hydrocephalus.
DESIGN, SETTING, AND PARTICIPANTS: This case series included patients with hydrocephalus who were born in October and November 2015 and followed up until mid-2017 in the 2 largest national referral centers for CZS in Brazil. The participants included consecutively enrolled children with a clinical and laboratorial diagnosis of CZS who developed clinical and/or image findings suggestive of hydrocephalus and who were confirmed to experience increased intracranial hypertension during ventriculoperitoneal shunt procedures.
To retrospectively describe clinical and image findings in these 24 patients.
This multicenter cohort included 308 patients with CZS; 24 consecutive children were enrolled in this study. These children were aged between 3 to 18 months, and 13 of 24 (54%) were female. All patients presented with at least 1 positive test result for anti-Zika antibodies in cerebrospinal fluid or serum and had classic signs of CZS. At the time of hydrocephalus diagnosis, only 14 of 24 patients (58%) had symptoms and signs suggestive of hydrocephalus (mainly worsening seizures, vomiting, irritability, and/or sudden increase of head circumference percentile). Two of 24 patients (8%) had no symptoms suggestive of hydrocephalus but were found to have reduced brain volume on repeated imaging. Cerebellar or brainstem hypoplasia on baseline imaging were found in 18 of 23 patients (78%). At the second computed tomographic scan, all patients showed a marked increase of ventricular volume, compatible with communicating hydrocephalus, and reduction of brain tissue that was visibly worse than on baseline imaging for the 23 patients with repeated scans.
We present evidence that hydrocephalus is a complication of CZS in at least a proportion of patients. The clinical spectrum of this condition continues to evolve, but given that presenting signs and symptoms of hydrocephalus can be challenging to recognize in CZS, we provisionally recommend that high suspicion and appropriate monitoring for hydrocephalus should be part of the standard care of patients with CZS.
脑积水是一种可治疗但可能致命的并发症，以前并未在先天性寨卡综合征（CZS）中描述过。
描述 24 例先天性寨卡综合征（CZS）患者并发脑积水的临床特征和影像学表现。
设计、地点和参与者： 本病例系列研究纳入了 2015 年 10 月和 11 月在巴西 2 家最大的 CZS 国家转诊中心出生并随访至 2017 年年中的脑积水患者。参与者包括连续纳入的具有临床和实验室诊断的 CZS 患者，这些患者出现了临床和/或影像学表现提示脑积水，并在脑室-腹腔分流术期间证实颅内压升高。
回顾性描述这 24 例患者的临床和影像学发现。
该多中心队列包括 308 例 CZS 患者；连续纳入了 24 例患儿。这些患儿年龄在 3 至 18 个月之间，24 例患儿中有 13 例（54%）为女性。所有患者的脑脊液或血清中均至少有 1 项抗寨卡病毒抗体检测结果阳性，且均具有典型的 CZS 表现。在脑积水诊断时，仅有 24 例患者中的 14 例（58%）存在提示脑积水的症状和体征（主要为癫痫发作恶化、呕吐、烦躁不安和/或头围百分位突然增加）。24 例患儿中有 2 例（8%）无提示脑积水的症状，但在重复影像学检查中发现脑容量减少。23 例进行了重复影像学检查的患者中，基线影像学检查显示 18 例（78%）存在小脑或脑干发育不良。在第二次计算机断层扫描时，所有患者的脑室容积均明显增大，符合交通性脑积水，与基线影像学相比，23 例进行了重复扫描的患者的脑组织减少更明显。
我们提供的证据表明，脑积水是 CZS 患者的一种并发症，至少在一部分患者中是这样。该疾病的临床表现仍在不断演变，但鉴于 CZS 患者的脑积水表现和症状可能难以识别，我们暂建议对 CZS 患者应高度怀疑并进行适当的脑积水监测，这应成为标准治疗的一部分。
