Abstract：
BACKGROUND: Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 h of birth. However, this is not always possible in severely premature infants. Given the rarity of this patient population, we aimed to share our institutional experience and outcomes of severely premature infants with MMC.
METHODS: We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death.
RESULTS: Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1-80.8). Five patients were taken for surgery within the recommended 48 h of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient\'s defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria). In both cases of sepsis, patients developed signs and symptoms more than 72 h after birth. Notably, both instances of sepsis occurred unrelated to operative intervention as they occurred before permanent MMC closure. Two patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 h of MMC closure.
CONCLUSIONS: In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term.
METHODS: We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death.
RESULTS: Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1-80.8). Five patients were taken for surgery within the recommended 48 h of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient\'s defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria). In both cases of sepsis, patients developed signs and symptoms more than 72 h after birth. Notably, both instances of sepsis occurred unrelated to operative intervention as they occurred before permanent MMC closure. Two patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 h of MMC closure.
CONCLUSIONS: In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term.
摘要：
背景：脑膜膨出（MMC）是最常见的神经管缺损，但在早产儿中很少见。大多数中心主张在出生后24小时内关闭MMC。然而,这并不总是可能在严重的早产儿。鉴于这些患者的稀有性,我们的目的是分享我们的机构经验和严重早产儿MMC的结局.
方法：我们进行了回顾性研究，通过我们的多学科脊柱裂诊所(1995-2021年)和手术日志确定的早产儿(≤32周胎龄)的观察性回顾.对该样本进行描述性统计，包括MMC闭合时间和败血症等不良事件的发生率。脑脊液分流,脑膜炎,和死亡。
结果：8例患者（50%为男性）患有MMC，胎龄≤32周。该人群的平均胎龄为27.3周（SD3.5）。MMC闭合的中位时间为1.5天（IQR=1-80.8）。5例患者在出生后48小时内接受手术治疗；2例患者明显延迟闭合（107天和139天）；1例患者的上皮缺损未经手术干预。8例患者中有6例需要永久性脑脊液（CSF）分流（2例患者接受了脑室腹膜分流（VPS）治疗，3例接受内镜下第三脑室造瘘术（ETV）和脉络丛烧灼术（CPC）治疗，1例接受ETV治疗；出生后平均3年，从1天到16年不等)。两名患者需要一个以上的永久性CSF转移程序。两名患者发展为脓毒症(定义为满足至少2/4SIRS标准)。在这两种脓毒症病例中，患者在出生后72小时以上出现体征和症状。值得注意的是,这两种脓毒症的发生与手术干预无关,因为它们发生在永久性MMC闭合前.两名患者患有脑室内出血（均为III级）。在MMC关闭之前，没有患者发生脑膜炎（定义为阳性CSF培养物）。中位随访时间为9.7年。在这个时代，3名患者死亡：2名患者在2岁之前死亡，原因与手术干预无关。两名III级IVH患者中的一名在MMC闭合后24小时内死亡。
结论：在我们对患有MMC的早产儿的机构经验中，一些患者接受了延迟的MMC封堵术.脑膜炎的总发病率,脓毒症,患有MMC的早产儿的死亡率与足月出生的MMC患者相似。
方法：我们进行了回顾性研究，通过我们的多学科脊柱裂诊所(1995-2021年)和手术日志确定的早产儿(≤32周胎龄)的观察性回顾.对该样本进行描述性统计，包括MMC闭合时间和败血症等不良事件的发生率。脑脊液分流,脑膜炎,和死亡。
结果：8例患者（50%为男性）患有MMC，胎龄≤32周。该人群的平均胎龄为27.3周（SD3.5）。MMC闭合的中位时间为1.5天（IQR=1-80.8）。5例患者在出生后48小时内接受手术治疗；2例患者明显延迟闭合（107天和139天）；1例患者的上皮缺损未经手术干预。8例患者中有6例需要永久性脑脊液（CSF）分流（2例患者接受了脑室腹膜分流（VPS）治疗，3例接受内镜下第三脑室造瘘术（ETV）和脉络丛烧灼术（CPC）治疗，1例接受ETV治疗；出生后平均3年，从1天到16年不等)。两名患者需要一个以上的永久性CSF转移程序。两名患者发展为脓毒症(定义为满足至少2/4SIRS标准)。在这两种脓毒症病例中，患者在出生后72小时以上出现体征和症状。值得注意的是,这两种脓毒症的发生与手术干预无关,因为它们发生在永久性MMC闭合前.两名患者患有脑室内出血（均为III级）。在MMC关闭之前，没有患者发生脑膜炎（定义为阳性CSF培养物）。中位随访时间为9.7年。在这个时代，3名患者死亡：2名患者在2岁之前死亡，原因与手术干预无关。两名III级IVH患者中的一名在MMC闭合后24小时内死亡。
结论：在我们对患有MMC的早产儿的机构经验中，一些患者接受了延迟的MMC封堵术.脑膜炎的总发病率,脓毒症,患有MMC的早产儿的死亡率与足月出生的MMC患者相似。
