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伴有脑及皮下脓肿的多发性脑和肺动静脉畸形:遗传性出血性毛细血管扩张症的一种可能变异型——病例报告

Multiple cerebral and pulmonary arteriovenous malformations in association with brain and subcutaneous abscesses: a possible variant of hereditary hemorrhagic telangiectasia--case report.

作者信息

Kawaguchi T, Fujita S, Yamada H, Nishida Y, Izawa I

机构信息

Department of Neurosurgery, Hyogo Brain and Heart Center, Himeji.

出版信息

Neurol Med Chir (Tokyo). 1990 Apr;30(4):272-6. doi: 10.2176/nmc.30.272.

Abstract

The authors present a case in which multiple cerebral and pulmonary arteriovenous malformations (AVMs), a brain abscess, and a recurrent subcutaneous abscess were found concurrently in a 52-year-old male. He was admitted to our hospital for evaluation of a subcutaneous abscess in the right nuchal area and a right occipital AVM that had been detected at another hospital. Of his eight siblings, three had died of cerebrovascular disease (one in childhood and two as young adults), one had died of unknown causes in childhood, one had been hemiplegic since infancy, one had recently undergone removal of a pulmonary AVM, one was killed in World War II at the age of 24 years, and the remaining sibling was healthy. He had had surgery for a right occipital brain abscess three years prior to this admission. A general examination revealed no abnormalities other than the painful right nuchal mass. Neurological evaluation disclosed left homonymous hemianopsia, which was probably a result of his previous surgery for the right occipital brain abscess. Cerebral angiography showed AVMs in the right parietal (2 x 2 cm), right occipital (1.5 x 1.5 cm), and right cerebellar areas (1 x 1 cm). Digital subtraction angiography of the lung revealed multiple bilateral AVMs. The cerebral and pulmonary AVMs were removed in a two-stage operation. Although this case did not correspond precisely to the triad of hereditary hemorrhagic telangiectasia (HHT), the authors consider it to be related to HHT, since that syndrome is often complicated by multiple cerebral and pulmonary AVMs.

摘要

作者报告了一例52岁男性同时发现多处脑和肺动静脉畸形(AVM)、脑脓肿及复发性皮下脓肿的病例。他因右侧颈部皮下脓肿及另一家医院检出的右枕叶AVM而入住我院。他的八个兄弟姐妹中,三人死于脑血管疾病(一人于儿童期,两人于青年期),一人于儿童期死因不明,一人自婴儿期起偏瘫,一人近期接受了肺AVM切除术,一人在二战时24岁死亡,其余兄弟姐妹健康。此次入院前三年,他曾因右枕叶脑脓肿接受手术。体格检查除右侧颈部疼痛性肿块外未见异常。神经学评估发现左侧同向性偏盲,可能是其既往右枕叶脑脓肿手术的结果。脑血管造影显示右侧顶叶(2×2cm)、右枕叶(1.5×1.5cm)及右小脑区域(1×1cm)存在AVM。肺部数字减影血管造影显示双侧多处AVM。通过两阶段手术切除了脑和肺的AVM。尽管该病例并不完全符合遗传性出血性毛细血管扩张症(HHT)三联征,但作者认为其与HHT有关,因为该综合征常并发多处脑和肺AVM。

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