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中枢神经系统先天性异常:一项来自尼日利亚的前瞻性神经外科观察性研究。

Central nervous system congenital anomalies: a prospective neurosurgical observational study from Nigeria.

作者信息

Adeleye Amos Olufemi, Olowookere Kayode Gabriel

机构信息

Division of Neurosurgery, Department of Surgery, College of Medicine, University of Ibadan and University College Hospital, Ibadan, Nigeria.

出版信息

Congenit Anom (Kyoto). 2009 Dec;49(4):258-61. doi: 10.1111/j.1741-4520.2009.00241.x.

DOI:10.1111/j.1741-4520.2009.00241.x
PMID:20021485
Abstract

This is a prospective observational study on the profile of central nervous system (CNS) anomalies in a neurosurgical unit in Ikeja, Nigeria. All cases of CNS anomalies seen by the authors in 1 year, January to December 2005, were analyzed. Descriptive variables include some demographics of the patients and their parents; profiles of CNS anomalies, cranial or spinal, neural tube defects (NTD) or others; presence of associated anomalies; and rate of surgical correction of these anomalies. Sixty-one cases were recorded, approximately 64% of our total pediatric neurosurgical output. Forty-one cases were males, 20 females, with a male to female ratio of approximately 2 to 1. The age at presentation ranged from 2 days to 60 months (mean 5.6 months) and the majority of the parents were young (the mean ages of the fathers and mothers were 35 years and 28 years, respectively), and low income earners (70%). The cases comprised cranial (61%) and spinal (39%) anomalies, or 54% of NTD versus others. The cranial lesions were mainly congenital primary hydrocephalus in 44% of cases, encephalocele in 28%, and microcephaly in 17%. The spinal lesions were spinal dysraphism in more than 90%; all in the lumbosacral region. Seventy percent of the NTD had associated cranial or other system anomalies, mainly secondary hydrocephalus in 65%. The surgical correction rate of all of these cases was 38%. NTD were significantly more likely to present early, be associated with other anomalies and undergo surgical correction in this series (P < 0.001). CNS congenital anomalies are under-reported in Nigeria and other developing countries. A call is made for the establishment of nationwide efforts to fully clarify the epidemiology of this silent epidemic.

摘要

这是一项针对尼日利亚伊凯贾一家神经外科单位中枢神经系统(CNS)异常情况的前瞻性观察研究。作者分析了2005年1月至12月这1年期间所见到的所有中枢神经系统异常病例。描述性变量包括患者及其父母的一些人口统计学特征;中枢神经系统异常的情况,包括颅脑或脊柱异常、神经管缺陷(NTD)或其他异常;是否存在相关异常;以及这些异常的手术矫正率。共记录了61例病例,约占我们儿科神经外科总病例数的64%。其中41例为男性,20例为女性,男女比例约为2比1。就诊年龄范围为2天至60个月(平均5.6个月),大多数父母较为年轻(父亲和母亲的平均年龄分别为35岁和28岁),且收入较低(70%)。病例包括颅脑异常(61%)和脊柱异常(39%),其中神经管缺陷占54%,其他异常占46%。颅脑病变主要为先天性原发性脑积水,占44%;脑膨出占28%;小头畸形占17%。脊柱病变中超过90%为脊柱裂;均位于腰骶部。70%的神经管缺陷伴有颅脑或其他系统异常,主要是继发性脑积水,占65%。所有这些病例的手术矫正率为38%。在本系列中,神经管缺陷更有可能早期出现、伴有其他异常并接受手术矫正(P < 0.oo1)。在尼日利亚和其他发展中国家,中枢神经系统先天性异常的报告不足。呼吁在全国范围内努力,以全面阐明这一隐匿性流行病的流行病学情况。

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