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[弯刀综合征合并马蹄肺。一种罕见但典型的表现——病例报告及文献复习]

[Combination of scimitar syndrome and horseshoe lung. A rare but typical finding--case report and review of the literature].

作者信息

Hassberg D, Steil E, Sieverding L, Rosendahl W

机构信息

Abt. für pädiatrische Kardiologie, Kinderklinik der Eberhard-Karls-Universität, Tübingen.

出版信息

Klin Padiatr. 1992 Nov-Dec;204(6):434-43. doi: 10.1055/s-2007-1025385.

Abstract

The scimitar syndrome is a rare congenital cardiopulmonary malformation. In association with a horseshoe lung it is extremely uncommon. We describe a child with typical scimitar syndrome (anomalous right pulmonary venous return, hypoplasia of the right lung with dextro-position of the heart and aberrant systemic arterial supply of the right lung), associated with a horseshoe lung. No clinical symptoms are present, surgical management is not necessary. This is the 20th case in addition to the 19 reported cases with scimitar syndrome associated with horseshoe lung. Eight other additional cases of horseshoe lung without scimitar syndrome are mentioned here. In horseshoe lung the pulmonary parenchyma extends from the right lung base across the midline and fuses with the left lung. In only 3 cases continuous parenchymal tissue could be found histological. In 8 cases the lungs were separated by fissurelike structures, seen in CT or chest film, and in 5 cases fissures were demonstrated histological. From 6 patients no findings are available. The various symptoms, the diagnostic tools and the therapeutic procedure will be discussed.

摘要

弯刀综合征是一种罕见的先天性心肺畸形。与马蹄肺相关时极为罕见。我们描述了一名患有典型弯刀综合征(右肺静脉异常回流、右肺发育不全伴心脏右旋及右肺异常体循环动脉供血)并伴有马蹄肺的儿童。目前无临床症状,无需手术治疗。这是除已报道的19例弯刀综合征合并马蹄肺病例外的第20例。此处还提及另外8例无弯刀综合征的马蹄肺病例。在马蹄肺中,肺实质从右肺底部延伸至中线并与左肺融合。仅3例在组织学上发现连续的实质组织。8例在CT或胸片上可见肺被裂隙样结构分隔,5例在组织学上证实有裂隙。6例患者未提供相关发现。将对各种症状、诊断工具和治疗方法进行讨论。

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