Division of Pediatric Neurosurgery, Children's of Alabama, Birmingham, AL, USA.
Childs Nerv Syst. 2024 Nov;40(11):3609-3614. doi: 10.1007/s00381-024-06524-3. Epub 2024 Jul 20.
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.
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.
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.
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.
脊髓脊膜膨出（MMC）是最常见的神经管缺陷，但在早产儿中很少见。大多数中心主张在出生后 24 小时内对 MMC 进行闭合。然而，对于极早产儿来说，这并不总是可行的。鉴于此类患者人群的罕见性，我们旨在分享我们机构对患有 MMC 的极早产儿的经验和结果。
我们通过多学科脊柱裂诊所（1995-2021 年）和手术记录，对通过我们多学科脊柱裂诊所（1995-2021 年）和手术记录识别出的极早产儿（<32 周胎龄）进行了回顾性、观察性研究。对该样本的相关数据进行了描述性统计，包括 MMC 闭合的时间以及诸如败血症、CSF 引流、脑膜炎和死亡等不良事件的发生率。
确定了 8 名患有 MMC 的患者（50%为男性），他们的胎龄均<32 周。该人群的平均胎龄为 27.3 周（SD 3.5）。MMC 闭合的中位时间为 1.5 天（IQR=1-80.8）。5 名患者在出生后 48 小时内接受了手术；2 名患者的手术时间明显延迟（107 和 139 天）；1 名患者的缺陷自行上皮化，无需手术干预。8 名患者中有 6 名需要永久性脑脊液（CSF）引流（2 名患者接受了脑室腹膜分流术（VPS）；3 名患者接受了内镜第三脑室造瘘术（ETV）联合脉络丛烧灼术（CPC）；1 名患者接受了 ETV；出生后平均随访 3 年，范围为 1 天至 16 年）。2 名患者需要不止一次永久性 CSF 引流手术。2 名患者发生败血症（定义为至少满足 2/4 SIRS 标准）。在这 2 例败血症中，患者在出生后超过 72 小时出现症状和体征。值得注意的是，这两例败血症均与手术干预无关，因为它们发生在永久性 MMC 闭合之前。2 名患者发生脑室出血（均为 3 级）。在 MMC 闭合之前，没有患者发生脑膜炎（定义为 CSF 培养阳性）。中位随访时间为 9.7 年。在此期间，有 3 名患者死亡：2 名患者在 2 岁之前死于与手术干预无关的原因。两名 III 级 IVH 患者中有 1 名在 MMC 闭合后 24 小时内死亡。
在我们对患有 MMC 的极早产儿的机构经验中，一些患者接受了延迟的 MMC 闭合。患有 MMC 的早产儿与足月出生的 MMC 患者相比，其脑膜炎、败血症和死亡率总体相似。
