Stritzke Amelie I, Dunham Christopher P, Smyth John A, Steinbok Paul
Division of Neonatology, Children’s and Women’s Health Center of British Columbia, Vancouver, British Columbia, Canada.
J Neurosurg Pediatr. 2011 Oct;8(4):372-6. doi: 10.3171/2011.7.PEDS11208.
The authors describe the case of a late preterm infant girl who presented prenatally with a low lumbar neural tube defect and features of Chiari malformation type II (CM-II). At birth, she exhibited stridor and underwent surgical repair of a lumbosacral myelomeningocele on Day 2 of life. The prognosis was deemed to be poor, and hence a "Chiari decompression" procedure was not undertaken. The patient was subsequently extubated and died on Day 10. Postmortem findings included a rarely described but characteristic granulomatous meningitic reaction to vernix caseosa, which presumably entered the subarachnoid space and spinal cord syrinx antenatally via the open neural tube defect. The significance of congenital stridor in the context of CM-II and in particular the role of vernix caseosa granulomatous meningitis are examined. The antenatal repair of myelomeningoceles, as championed by some, may prevent this ominous meningitic complication.
作者描述了一名晚期早产儿女婴的病例，该女婴在产前被诊断为低位腰椎神经管缺陷并伴有Ⅱ型 Chiari 畸形（CM-II）的特征。出生时，她出现喘鸣，并在出生后第 2 天接受了腰骶部脊髓脊膜膨出的手术修复。预后被认为很差，因此未进行“Chiari 减压”手术。患者随后拔管，并于第 10 天死亡。尸检结果包括一种罕见但特征性的对胎脂的肉芽肿性脑膜炎反应，推测胎脂是通过开放的神经管缺陷在产前进入蛛网膜下腔和脊髓空洞的。本文探讨了 CM-II 背景下先天性喘鸣的意义，特别是胎脂肉芽肿性脑膜炎的作用。一些人倡导的脊髓脊膜膨出产前修复术可能会预防这种不祥的脑膜炎并发症。
