Ocal Eylem, Irwin Beverly, Cochrane Douglas, Singhal Ash, Steinbok Paul
Division of Pediatric Neurosurgery, Department of Surgery, British Columbia Children's Hospital, University of British Columbia, 4480 Oak Street, Vancouver, BC, Canada.
Childs Nerv Syst. 2012 Feb;28(2):265-71. doi: 10.1007/s00381-011-1585-8. Epub 2011 Sep 23.
Stridor, associated with vocal cord paralysis, in neonates with myelomeningocele (MMC) is a recognized symptom related to Chiari II malformation (CM). In most children, stridor appears after birth. Control of hydrocephalus, if present, and urgent decompression of the CM are recommended for treatment of these patients. Such management typically improves symptoms. Occasionally, stridor is present at birth and may be secondary, in part, to maldevelopment or prenatal ischemia of the brain stem, rather than treatable compression. There is minimal literature describing the outcome after Chiari decompression in this population. The purpose of this study was to review the outcomes of neonates with MMC and stridor at birth and compare it to MMC patients who develop stridor later. We hypothesized that unlike stridor which develops after birth, stridor at birth predicts a dismal outcome, despite aggressive surgical treatment.
Retrospective review of newborns with MMC and CM was performed in our institution from 1975 to 2010. Patients with stridor at birth and those who developed stridor later in infancy were identified. Outcomes were analyzed. Autopsy findings were reviewed when available.
Six patients with MMC who presented with stridor at birth were identified. Five of these patients had decompression of CM and treatment of hydrocephalus, if present, within the first 2 weeks of life. All patients died: three within 1 month and the oldest at 62 months. In the three patients with autopsies, vernix caseosa meningitis was present. Eight patients presented with stridor later in infancy. CM decompression was performed in seven of them. One patient out of the seven with late onset of stridor died at 13 months after CM surgery. The mortality rate after CM decompression was worse in patients with stridor at birth than those presenting later with stridor (chi-square p = 0.015).
In newborns with MMC, stridor at birth may predict dismal outcome despite CM decompression. Unlike the situation in neonates who develop stridor after birth, the outcome in those presenting with stridor at birth does not seem to be impacted by decompression of the CM. Nonoperative management may be an option to offer in this population. Additionally, vernix caseosa meningitis may contribute to the severe irreversible brain stem dysfunction in these newborns.
在患有脊髓脊膜膨出（MMC）的新生儿中，喘鸣与声带麻痹相关，是一种与Chiari II型畸形（CM）有关的公认症状。在大多数儿童中，喘鸣在出生后出现。对于这些患者的治疗，建议控制脑积水（如果存在）并紧急减压CM。这种治疗通常可改善症状。偶尔，喘鸣在出生时就存在，可能部分继发于脑干发育不良或产前缺血，而非可治疗的压迫。关于该人群Chiari减压术后结局的文献极少。本研究的目的是回顾出生时患有MMC和喘鸣的新生儿的结局，并将其与后来出现喘鸣的MMC患者进行比较。我们假设，与出生后出现的喘鸣不同，出生时的喘鸣预示着不良结局，尽管进行了积极的手术治疗。
对1975年至2010年在我们机构出生的患有MMC和CM的新生儿进行回顾性研究。确定出生时出现喘鸣的患者以及在婴儿期后期出现喘鸣的患者。分析结局。如有尸检结果，则进行回顾。
确定了6例出生时患有MMC且出现喘鸣的患者。其中5例患者在出生后的头2周内接受了CM减压及脑积水（如果存在）的治疗。所有患者均死亡：3例在1个月内死亡，年龄最大的在62个月时死亡。在3例进行尸检的患者中，发现有胎脂性脑膜炎。8例患者在婴儿期后期出现喘鸣。其中7例进行了CM减压。7例喘鸣出现较晚的患者中有1例在CM手术后13个月死亡。出生时出现喘鸣的患者CM减压后的死亡率高于喘鸣出现较晚的患者（卡方检验p = 0.015）。
在患有MMC的新生儿中，出生时的喘鸣可能预示着不良结局，尽管进行了CM减压。与出生后出现喘鸣的新生儿情况不同，出生时出现喘鸣的患者的结局似乎不受CM减压的影响。对于该人群，非手术治疗可能是一种选择。此外，胎脂性脑膜炎可能导致这些新生儿出现严重的不可逆脑干功能障碍。
