Atqiaee Khashayar, Samady Khanghah Ali, Mohajerzadeh Leily, Mardi Ali, Khayat Zahiri Farhad, Barin Sajjad
Department of Pediatric Surgery, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad.
Department of Surgery.
Ann Med Surg (Lond). 2023 Jun 28;85(8):4041-4044. doi: 10.1097/MS9.0000000000000395. eCollection 2023 Aug.
Lymphatic malformations (LM) refer to very rare hamartomatous benign lymphatic ectasias with an incidence of 1:250 000. They almost involve children more than adults, and among them, infants under one age are mostly affected. Macrocytic LM is found to be more than 2 cm in diameter or 2×2 cm in volume. The proper treatment for mesenteric LM is complete surgical excision unless there is vital structure involvement.
The authors report a mesenteric macrocytic LM in a 2-year-old girl complaining of vague abdominal discomfort and persistent vomiting in which ultrasonography revealed a cystic masse with seromucous components. She then underwent exploratory laparotomy. The operation and the follow-up duration were uneventful.
LMs are rare benign lesions of vascular origin with lymphatic differentiation, according to the latest International Society for the Study of Vascular Anomalies (ISSVA 2018). Under light microscopy, these malformations are characterized by their thin-walled endothelium and lymphatic tissue.These mobile lesions are incidentally found or appear with intestinal obstruction or acute abdomen scenarios.
Although benign, the LMs have the potential for invasion and recurrence. Thus, the examiner physician must keep such intra-abdominal lesions in mind.
淋巴管畸形(LM)是指非常罕见的错构瘤性良性淋巴管扩张,发病率为1:250000。它们几乎多见于儿童而非成人,其中,1岁以下婴儿受影响最为常见。巨囊性淋巴管畸形直径大于2cm或体积大于2×2cm。肠系膜淋巴管畸形的恰当治疗方法是完整手术切除,除非累及重要结构。
作者报告了一名2岁女孩的肠系膜巨囊性淋巴管畸形,该女孩主诉腹部隐痛和持续呕吐,超声检查显示一个含有浆液性成分的囊性肿块。随后她接受了剖腹探查术。手术及随访过程均顺利。
根据最新的国际血管畸形研究学会(2018年国际血管畸形研究学会),淋巴管畸形是罕见的起源于血管且具有淋巴分化的良性病变。在光学显微镜下,这些畸形的特征是其薄壁内皮和淋巴组织。这些可移动的病变多为偶然发现,或在肠梗阻或急腹症情况下出现。
尽管淋巴管畸形是良性的,但有侵袭和复发的可能。因此,检查医师必须牢记此类腹腔内病变。