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小肠囊性肠系膜淋巴管瘤病程中出现的多年腹痛并伴有胃肠道梗阻。

Multiannual abdominal pain complicated by obstruction of the gastrointestinal tract in the course of cystic form of mesenteric lymhangioma of the small intestine.

作者信息

Majewski Mateusz, Lorenc Zbigniew, Krawczyk Wiktor

出版信息

Pol Przegl Chir. 2015 Apr;87(4):185-8. doi: 10.1515/pjs-2015-0042.

Abstract

Lymphangiomas constitute a group of very rare diseases and occur with a frequency of 1/250,000 to 1/20,000 of hospitalizations. Even though they are benign lesions, their complications may turn into a life-threatening condition. They usually occur in children (90%), they are either congenital or they appear before the child's second birthday. Occassionally they are found in adults. Lymphangiomas are usually localized around neck, but also near armpits and in the groin area. Less than 1% of lymphagiomas are detected in the retroperitoneal space and intestinal mesentery. Vascular lesions derived from lymphatic vessels can be divided into ordinary ones, usually known as capillary, cavernous, and cystic. The cystic tumor (lymphangioma mesenteri) is the least common. It occurs mostly in the retroperitoneal space, mesentery of the colon, or extremely rarely in the mesentery of the small intestine. Preoperative diagnosis is difficult due to non-specific clinical symptoms and noncharacteristic image in the diagnostic tests. Lymphangioma complications, such as intestinal obstruction or perforation, and persistent pain, are the cause of exploratory laparotomy. Final diagnosis requires microscopic examination of material collected during an operation. This article presented the case of a 40-year-old female, operated due to the obstruction of the gastrointestinal tract, in whose case the lymphangioma was recognised in postoperative histopathological examination of the tumor from the jejunal mesentery. Since patients with these tumors have good chances of complete recovery - if there are no serious complications - it appears that the optimal therapeutic procedure should be early surgery, which reduces the possibility of complications.

摘要

淋巴管瘤是一组非常罕见的疾病,其在住院患者中的发生率为1/250,000至1/20,000。尽管它们是良性病变,但其并发症可能会发展成危及生命的状况。它们通常发生于儿童(90%),可为先天性或在儿童2岁前出现。偶尔也可见于成人。淋巴管瘤通常位于颈部周围,但也可出现在腋窝附近和腹股沟区。不到1%的淋巴管瘤在腹膜后间隙和肠系膜中被发现。源自淋巴管的血管病变可分为普通型,通常称为毛细血管型、海绵状和囊性。囊性肿瘤(肠系膜淋巴管瘤)最为少见。它大多发生于腹膜后间隙、结肠系膜,或极罕见地发生于小肠系膜。由于临床症状不具特异性且诊断检查中的影像无特征性,术前诊断较为困难。淋巴管瘤的并发症,如肠梗阻或穿孔以及持续性疼痛,是进行剖腹探查术的原因。最终诊断需要对手术中采集的材料进行显微镜检查。本文介绍了一例40岁女性患者,因胃肠道梗阻接受手术,在其空肠系膜肿瘤的术后组织病理学检查中确诊为淋巴管瘤。由于这些肿瘤患者若没有严重并发症则有良好的完全康复机会,因此最佳治疗方法似乎应为早期手术,这可降低并发症的发生可能性。

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