1Department of Pediatric Surgery, Division of Pediatric Neurosurgery.
2Clinical Research Unit.
Neurosurg Focus. 2019 Oct 1;47(4):E2. doi: 10.3171/2019.7.FOCUS19462.
The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC).
A retrospective cohort study was conducted to analyze factors associated with MMC that influence the morbidity and mortality of the disease. Data were collected from medical records of children who underwent the primary repair of MMC at the Fernandes Figueira Institute-Oswaldo Cruz Foundation (IFF-Fiocruz) between January 1995 and January 2015, with a minimum follow-up of 1 year. The following variables were analyzed: demographic characteristics (gestational age, sex, and birth weight); clinical features (head circumference at birth, anatomical and functional levels of MMC, hydrocephalus, symptomatic Chiari malformation type II, neurogenic bladder, and urinary tract infection [UTI]); and surgical details such as timing of repair of MMC, age at first shunt placement, shunt surgery modality (elective or emergency), concurrent surgery (correction of MMC and shunt insertion in the same surgical procedure), incidence and cause of shunt dysfunction, use of external ventricular drain, transfontanelle puncture, surgical wound complications prior to shunting, and endoscopic treatment of hydrocephalus.
A total of 231 patients with MMC were included in the analysis. Patients were followed for periods ranging from 1 to 20 years, with a mean of 6.9 years. The frequency of shunt placement was observed mainly among patients with MMC at the highest spinal levels (p < 0.01). The main causes of morbidity and mortality in patients with MMC were shunt failures, diagnosed in 91 of 193 cases (47.2%) of hydrocephalus, and repeated UTIs, in 129 of 231 cases (55.8%) of MMC; these were the main causes of hospitalization and death. Head circumference ≥ 38 cm at birth was found to be a significant risk factor for shunt revision (p < 0.001; 95% CI 1.092-1.354). Also, the lumbar functional level of MMC was associated with less revision than upper levels (p < 0.014; 95% CI 0.143-0.805). There was a significant association between recurrent UTI and thoracic functional level.
Macrocephaly at birth and higher levels of the defect have an impact on worse outcome and, therefore, are a challenge to the daily practice of pediatric neurosurgery.
本研究旨在分析影响脊髓脊膜膨出（MMC）患者发病率和死亡率的因素。
本研究采用回顾性队列研究分析与 MMC 相关的影响疾病发病率和死亡率的因素。数据收集自 1995 年 1 月至 2015 年 1 月在菲格腊亚研究所-奥斯瓦尔多·克鲁兹基金会（IFF-Fiocruz）接受 MMC 初次修复的患儿的病历，所有患儿的随访时间均至少 1 年。分析的变量包括：人口统计学特征（胎龄、性别和出生体重）；临床特征（出生时头围、MMC 的解剖和功能水平、脑积水、Chiari Ⅱ型脊髓空洞症、神经源性膀胱和尿路感染[UTI]）；以及手术细节，如 MMC 修复时间、初次分流管放置年龄、分流管手术方式（择期或紧急）、同期手术（MMC 矫正和分流管插入同一手术）、分流管功能障碍的发生率和原因、使用外部脑室引流管、经颅穿刺、分流术前手术切口并发症和脑积水的内镜治疗。
共纳入 231 例 MMC 患儿进行分析。患儿的随访时间为 1 至 20 年，平均 6.9 年。主要分流管放置发生在最高脊髓水平的 MMC 患者中（p < 0.01）。MMC 患儿的发病率和死亡率的主要原因是分流管故障，193 例脑积水患儿中有 91 例（47.2%），反复发生 UTI，231 例 MMC 患儿中有 129 例（55.8%）；这些是住院和死亡的主要原因。出生时头围≥38 cm 是分流管修订的显著危险因素（p < 0.001；95%CI 1.092-1.354）。此外，MMC 的腰功能水平与上水平相比，需要更少的修订（p < 0.014；95%CI 0.143-0.805）。反复 UTI 与胸功能水平显著相关。
出生时头围较大和病变水平较高会影响预后，因此对儿科神经外科的日常实践提出挑战。
