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腹部淋巴管畸形

Abdominal lymphatic malformations.

作者信息

Wohlgemuth W A, Brill R, Dendl L M, Stangl F, Stoevesandt D, Schreyer A G

机构信息

University Clinic and Policlinic of Radiology, Martin-Luther-Universität Halle-Wittenberg, Ernst-Grube-Straße 40, 06120, Halle (Saale), Germany.

Institute of Radiology, University Medical Center Regensburg, Regensburg, Germany.

出版信息

Radiologe. 2018 Nov;58(Suppl 1):29-33. doi: 10.1007/s00117-017-0337-5.

Abstract

BACKGROUND

Abdominal lymphatic malformations (LM) are relatively rare findings in the differential diagnosis of focal abdominal lesions; however, they represent a challenge especially in younger patients. The aim of this review article is to provide up-to-date information about the different kinds of LM manifestations. In addition, related syndromes and typical imaging features to facilitate the diagnosis are discussed.

RESULTS

The clinical presentation of abdominal LM is unspecific, whereby most are asymptomatic and comprise incidental findings of thin-walled cystic masses anywhere in the abdomen. The fluid in the cystic masses may be proteinaceous, contain blood, or be infected. Radiological imaging features overlap with other cystic diseases; hallmark in LM is a lack of a solid component and exclusive enhancement of the walls and septa.

CONCLUSION

In cystic abdominal masses in early childhood or young adults, abdominal LM must be taken into account by the radiologist. Newly defined entities in this spectrum of diseases are central conducting lymphatic anomaly (CCLA) and generalized lymphatic anomaly (GLA).

摘要

背景

腹部淋巴管畸形(LM)在局灶性腹部病变的鉴别诊断中相对少见;然而,它们尤其给年轻患者带来挑战。这篇综述文章的目的是提供有关不同类型LM表现的最新信息。此外,还讨论了相关综合征以及有助于诊断的典型影像学特征。

结果

腹部LM的临床表现无特异性,大多数无症状,表现为腹部任何部位偶然发现的薄壁囊性肿块。囊性肿块内的液体可能为蛋白性、含血或被感染。放射学成像特征与其他囊性疾病重叠;LM的特征是缺乏实性成分,仅壁和间隔强化。

结论

对于儿童早期或年轻成人的腹部囊性肿块,放射科医生必须考虑到腹部LM。这一疾病谱中的新定义实体为中央传导性淋巴管异常(CCLA)和全身性淋巴管异常(GLA)。

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