背景:脊髓膜膨出(MMC)是最严重的脊柱裂形式,神经管发育的先天性缺陷。极低出生体重患者的缺陷闭合存在独特的挑战和风险;较低的出生体重与多器官系统问题有关。稳态是困难的,局部组织不发达。就作者所知,本病例是出生后MMC修复新生儿的最低体重(490g).
方法:一名早产男性,产前诊断为腰骶部MMC,并伴有Chiari畸形II型,出生23周1天,母亲29岁,孕次6平偶4。患者在生命第5天进行医学稳定并进行MMC闭合。修复时他的体重是490克,他没有任何手术并发症。16个月大的时候,他接受了内镜下第三脑室造瘘术和脉络丛烧灼术;自30个月大的最后一次随访以来,他不需要任何进一步的脑积水治疗。
结论:据作者所知,该病例是文献中报道的子宫外出生体重最低的MMC封堵术.早产和大小的挑战需要适当的术前稳定,小心止血和体温调节,细致的手术技术。
BACKGROUND: Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors\' knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair.
METHODS: A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age.
CONCLUSIONS: To the authors\' knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.