Clement Céline, Snoekx Rob, Ceulemans Pieter, Wyn Inez, Matheï Jan
a Departments of General Surgery , Mariaziekenhuis , Overpelt , Belgium.
b Departments of Pathology , Mariaziekenhuis , Overpelt , Belgium.
Acta Chir Belg. 2018 Oct;118(5):331-335. doi: 10.1080/00015458.2017.1379802. Epub 2017 Sep 19.
Lymphangioma accounts for 5% of benign pediatric tumors and less than 1% is located in the abdomen. While mesenteric lymphatic malformation or mesenteric lymphangioma (ML), mostly appears asymptomatic in adults, an acute clinical presentation is seen more often in children. Symptoms can mimic obstruction or peritonitis and diagnosis can therefore be challenging. Imaging studies are performed to exclude other causes of obstruction or peritonitis. Diagnosis of ML is always made by histology. Surgery is the treatment of choice and recurrence after complete resection is rare.
We describe an acute abdomen in a four-year-old boy caused by a mesenteric lymphatic malformation. Imaging studies at the emergency department were inconclusive and final diagnosis was made by histological examination. Surgical intervention with resection of an ischemic ileal segment was necessary. Complete resection of the ML was not possible without compromising the functional digestive outcome due to diffusely spread lesions in the mesentery of the small bowel. Follow-up with ultrasound was performed.
Mesenteric lymphangioma can cause an acute abdomen in children. Complete resection is recommended but might not be feasible without compromising the functional digestive outcome. Alternative treatments should be studied further to treat diffuse and complicated ML.
淋巴管瘤占小儿良性肿瘤的5%，位于腹部的不到1%。虽然肠系膜淋巴管畸形或肠系膜淋巴管瘤（ML）在成人中大多无症状，但在儿童中更常出现急性临床表现。症状可类似梗阻或腹膜炎，因此诊断具有挑战性。需进行影像学检查以排除梗阻或腹膜炎的其他原因。ML的诊断始终依靠组织学检查。手术是首选治疗方法，完全切除后复发罕见。
我们描述了一名4岁男孩因肠系膜淋巴管畸形导致的急腹症。急诊科的影像学检查结果不明确，最终通过组织学检查确诊。由于小肠系膜病变广泛扩散，若不影响功能性消化结果，无法完全切除ML，因此需要进行手术干预，切除一段缺血性回肠。术后进行了超声随访。
肠系膜淋巴管瘤可导致儿童急腹症。建议完全切除，但不影响功能性消化结果的情况下可能无法实现。应进一步研究替代治疗方法以治疗弥漫性和复杂性ML。
