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脑干切断术。

Brainstem disconnection.

机构信息

Department of Radiology, University of California Davis, Medical Center, UC Davis Children's Hospital, Sacramento, CA 95817, USA.

出版信息

Pediatr Radiol. 2009 Dec;39(12):1357-60. doi: 10.1007/s00247-009-1378-3. Epub 2009 Aug 11.

DOI:10.1007/s00247-009-1378-3
PMID:19669743
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2776168/
Abstract

Brainstem disconnection is a very rare neonatal abnormality, with only seven cases reported. We report a unique case of a neonate who presented at delivery with hypertonia, dysmorphic facial features, and respiratory distress, as well as numerous musculoskeletal and genitourinary abnormalities. MRI of the brain showed disconnection between the pons and medulla with cerebellar hypoplasia and absent cerebellar peduncles. It aided in the description of the neurological and vascular anomalies associated with this diagnosis.

摘要

脑干切断是一种非常罕见的新生儿异常,仅有 7 例报告。我们报告了一例独特的新生儿病例,该新生儿在分娩时表现为张力亢进、面部畸形和呼吸窘迫,以及许多肌肉骨骼和泌尿生殖系统异常。脑 MRI 显示桥脑和延髓之间的连接中断,伴有小脑发育不良和小脑脚缺失。它有助于描述与该诊断相关的神经和血管异常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2700/2776168/5b0731192f62/247_2009_1378_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2700/2776168/43e59c1d5460/247_2009_1378_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2700/2776168/5b0731192f62/247_2009_1378_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2700/2776168/43e59c1d5460/247_2009_1378_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2700/2776168/5b0731192f62/247_2009_1378_Fig2_HTML.jpg

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Magnetic resonance imaging of the brainstem in children, part 1: imaging techniques, embryology, anatomy and review of congenital conditions.儿童脑桥磁共振成像,第 1 部分:成像技术、胚胎学、解剖学及先天性疾病综述。
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本文引用的文献

1
Brainstem disconnection: case report and review of the literature.
Neuropediatrics. 2007 Aug;38(4):210-2. doi: 10.1055/s-2007-985907.
2
A developmental classification of malformations of the brainstem.脑干畸形的发育分类
Ann Neurol. 2007 Dec;62(6):625-39. doi: 10.1002/ana.21239.
3
Midbrain disconnection: an aetiology of severe central neonatal hypotonia.
Eur J Paediatr Neurol. 2005;9(6):419-22. doi: 10.1016/j.ejpn.2005.06.003. Epub 2005 Aug 2.
4
孤立性新生儿双侧声带麻痹:揭示单侧延髓病变:病例报告。
BMC Pediatr. 2018 Nov 9;18(1):351. doi: 10.1186/s12887-018-1329-y.
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Update on neuroimaging phenotypes of mid-hindbrain malformations.中后脑畸形的神经影像学表型更新
Neuroradiology. 2015 Feb;57(2):113-38. doi: 10.1007/s00234-014-1431-2. Epub 2014 Oct 23.
Pontomedullary disconnection: fetal and neonatal considerations.
脑桥延髓离断:胎儿及新生儿相关考量
Pediatr Radiol. 2005 Aug;35(8):812-4. doi: 10.1007/s00247-005-1455-1. Epub 2005 Apr 6.
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Human malformations of the midbrain and hindbrain: review and proposed classification scheme.人类中脑和后脑畸形:综述与分类方案建议
Mol Genet Metab. 2003 Sep-Oct;80(1-2):36-53. doi: 10.1016/j.ymgme.2003.08.010.
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Agenesis of the mesencephalon and metencephalon with cerebellar hypoplasia: putative mutation in the EN2 gene--report of 2 cases in early infancy.
Pediatr Dev Pathol. 2002 Jan-Feb;5(1):54-68. doi: 10.1007/s10024-001-0103-5.
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Neonatal pontomedullary disconnection with aplasia or destruction of the lower brain stem: a case of pontoneocerebellar hypoplasia?新生儿脑桥延髓离断伴低位脑干发育不全或破坏:脑桥小脑发育不全病例?
AJNR Am J Neuroradiol. 1994 Sep;15(8):1483-5.
8
Pontoneocerebellar hypoplasia: report of a case in a newborn and review of the literature.脑桥小脑发育不全:一例新生儿病例报告及文献复习
Clin Neuropathol. 1990 Jan-Feb;9(1):33-8.