Liang Cui-Ping, Yang Min, Chen Pei-Yu, Geng Lan-Lan, Li Ding-You, Gong Si-Tang
Department of Gastroenterology, Guangzhou Women and Children's Medical Center, 9 Jinsui Road, Guangzhou, 510623, China.
Department of Pediatrics, Division of Gastroenterology, University of Missouri-Kansas City, Children's Mercy Hospital, Kansas City, MO, USA.
BMC Pediatr. 2017 Aug 1;17(1):179. doi: 10.1186/s12887-017-0931-8.
Sclerosing mesenteritis is a rare fibroinflammatory disorder of unknown etiology that primarily affects the mesentery of the small intestine during late adult life. Only about twenty pediatric cases have been reported to date, but none has been reported in Chinese children.
A 5-year-old Chinese male presented with a 4-week history of recurrent bloating, abdominal pain, anorexia and vomiting. On admission, physical examination showed a severely distended abdomen. Biochemical investigations showed a slightly increased C-reactive protein, and normal serum levels of electrolytes and erythrocyte sedimentation rate. An abdominal film showed small intestine obstruction and massive ascites. An exploratory laparotomy revealed widespread inflammatory fibrotic adhesions between the bowel and the abdominal wall, thickening of the small bowel and massive ascites. During a prolonged hospital course, a 2nd surgery (4 months after 1st exploratory laparotomy) was performed in order to close the ileostomy and revealed that the bowel was still severely edematous, with very tight adhesions between the bowel and the abdominal wall. Histopathological examination of excised mesentery and nodules showed chronic inflammatory cell infiltration, fat necrosis and fibrosis. A diagnosis of sclerosing mesenteritis was finally established. Prednisolone at 2 mg/kg was started and he experienced rapid clinical improvement in 4 weeks.
Sclerosing mesenteritis is extremely rare in children and often misdiagnosed due to its nonspecific clinical manifestation. It is important to be aware of sclerosing mesenteritis when evaluating a child with intractable abdominal pain, bloating, intestinal obstruction and massive ascites.
硬化性肠系膜脂膜炎是一种病因不明的罕见纤维炎症性疾病,主要在成年后期影响小肠系膜。迄今为止,仅报道了约20例儿科病例,但尚无中国儿童病例的报道。
一名5岁中国男性,有4周反复腹胀、腹痛、厌食和呕吐的病史。入院时,体格检查显示腹部严重膨隆。生化检查显示C反应蛋白略有升高,血清电解质和红细胞沉降率正常。腹部平片显示小肠梗阻和大量腹水。剖腹探查发现肠管与腹壁之间广泛存在炎性纤维粘连、小肠增厚和大量腹水。在漫长的住院过程中,进行了第二次手术(首次剖腹探查术后4个月)以关闭回肠造口术,结果显示肠管仍严重水肿,肠管与腹壁之间粘连紧密。对切除的系膜和结节进行组织病理学检查,显示慢性炎性细胞浸润、脂肪坏死和纤维化。最终确诊为硬化性肠系膜脂膜炎。开始使用2mg/kg的泼尼松龙治疗,4周后他的临床症状迅速改善。
硬化性肠系膜脂膜炎在儿童中极为罕见,因其临床表现不具特异性,常被误诊。在评估患有顽固性腹痛、腹胀、肠梗阻和大量腹水的儿童时,认识到硬化性肠系膜脂膜炎很重要。