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足月儿脑瘫:158例法医学病例回顾的临床病理分析

Cerebral palsy in term infants: a clinicopathologic analysis of 158 medicolegal case reviews.

作者信息

Redline Raymond W

机构信息

Department of Pathology, University Hospitals Case Medical Center and Case Western Reserve School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA.

出版信息

Pediatr Dev Pathol. 2008 Nov-Dec;11(6):456-64. doi: 10.2350/08-05-0468.1.

Abstract

Our understanding of cerebral palsy (CP) in term infants is hindered by its low incidence and sporadic presentation. Many of these CP cases enter litigation, and a focused review of medicolegal consultations provides an opportunity to better understand the pathogenesis of these cases. In this study complete clinical and pathologic data from 158 cases of CP complicating singleton pregnancies after 36 weeks of gestation were prospectively collected over a 10-year period extending from 1998 to 2008. A hierarchical system was used to separate cases into the following 5 groups: (1) clinical/sentinel events (20%), (2) severe large fetoplacental vascular lesions (34%), (3) placental lesions indicative of chronic placental dysfunction (23%), and (4) placental lesions indicative of subacute/chronic adaptation to hypoxia (15%). The remaining 8% (group 5) of cases were idiopathic. Common to all subgroups was clinical and/or pathologic evidence of umbilical cord obstruction, which was observed in 63% of cases. The following clinical features significantly differed among subgroups. Group 1 had less maternal obesity and more cases involving multicystic encephalopathy. Group 2 had increased oligohydramnios, cerebral edema, nucleated red blood cell counts greater than 10 000/mm(3), hypoglycemia, pulmonary hypertension, and cardiac dysfunction. Group 3 had more preeclampsia and, together with group 2, more infants with a low ponderal index. Group 5 had a higher prevalence of positive family history of neurodevelopmental disorders. In conclusion, infant cases subject to litigation related to CP following term birth can be separated into distinct clinicopathologic subgroups with only a small number lacking either clinical/sentinel events or placental evidence of subacute or chronic in utero stress.

摘要

足月儿脑瘫(CP)的发病率较低且呈散发性,这给我们对其的认识带来了阻碍。许多脑瘫病例会进入诉讼程序,而对法医学咨询进行重点回顾,为更好地理解这些病例的发病机制提供了契机。在本研究中,前瞻性收集了1998年至2008年这10年间158例孕36周后单胎妊娠合并脑瘫病例的完整临床和病理数据。采用分层系统将病例分为以下5组:(1)临床/哨兵事件(20%),(2)严重的巨大胎儿 - 胎盘血管病变(34%),(3)提示慢性胎盘功能障碍的胎盘病变(23%),以及(4)提示对缺氧的亚急性/慢性适应的胎盘病变(15%)。其余8%(第5组)病例为特发性。所有亚组的共同特征是存在脐带梗阻的临床和/或病理证据,63%的病例观察到了这一情况。以下临床特征在各亚组间存在显著差异。第1组孕妇肥胖较少,多囊肿性脑病病例较多。第2组羊水过少、脑水肿、有核红细胞计数大于10000/mm³、低血糖、肺动脉高压和心脏功能障碍增多。第3组子痫前期较多,与第2组一起,低体重指数婴儿较多。第5组神经发育障碍家族史阳性的患病率较高。总之,足月出生后因脑瘫相关诉讼的婴儿病例可分为不同的临床病理亚组,只有少数病例既无临床/哨兵事件,也无子宫内亚急性或慢性应激的胎盘证据。

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