Hiridis Savvas, Hadgigeorgiou Renos, Karakitsos Dimitrios, Karabinis Andreas
Intensive Care Unit, General Hospital of Athens, Athens, Greece.
J Med Case Rep. 2008 Dec 17;2:388. doi: 10.1186/1752-1947-2-388.
Sclerosing mesenteritis is a rare disease resembling a mesenteric tumour. We present here a case of sclerosing mesenteritis that affected both the large and the small intestine of the patient. Therapeutic and diagnostic issues are discussed.
A 62-year-old man with a history of non-Hodgkin lymphoma presented with fatigue, a palpable tender abdominal mass and clinical signs of progressing intestinal obstruction. The preoperative evaluation failed to prove recurrence of the lymphoma or any other definite diagnosis. A laparotomy was performed through a midline incision. The mesentery resembled a tumour-like thickened and fibrotic mass. Abundant, rigid intestinal loop adhesions were observed. Diffuse fibrotic infiltration of the ileum and of the sigmoid colon, which obviously affected the intestinal vascular supply, were identified. A right colectomy and partial sigmoidectomy were performed. Pathological evaluation revealed extensive myofibroblastic reaction of the mesentery with accompanying loci of fat necrosis and areas of inflammation. A diffuse fibrotic infiltration that focally showed a ground-glass appearance was observed. The post-operative course was complicated by respiratory insufficiency and infections and the patient died 2 months after the operation.
Sclerosing mesenteritis that affects both the small and the large intestine is extremely rare. The disease is characterized by myofibroblastic reaction, fat necrosis and diffuse fibrosis of the mesentery. Pathological confirmation may be required for definite diagnosis. If the disease is characterized by severe and diffuse fibrosis, then the application of surgical therapy may be problematic.
硬化性肠系膜炎是一种罕见疾病,类似肠系膜肿瘤。我们在此报告一例硬化性肠系膜炎病例,该病例累及患者的大肠和小肠。并对治疗和诊断问题进行了讨论。
一名62岁有非霍奇金淋巴瘤病史的男性患者,出现疲劳、可触及的腹部压痛肿块以及进行性肠梗阻的临床体征。术前评估未能证实淋巴瘤复发或任何其他明确诊断。通过中线切口进行剖腹手术。肠系膜类似肿瘤样增厚和纤维化肿块。观察到大量坚硬的肠袢粘连。发现回肠和乙状结肠弥漫性纤维化浸润,明显影响了肠道血管供应。进行了右半结肠切除术和部分乙状结肠切除术。病理评估显示肠系膜广泛的肌成纤维细胞反应,伴有脂肪坏死灶和炎症区域。观察到弥漫性纤维化浸润,局部呈磨玻璃样外观。术后病程因呼吸功能不全和感染而复杂化,患者术后2个月死亡。
累及小肠和大肠的硬化性肠系膜炎极为罕见。该疾病的特征是肌成纤维细胞反应、脂肪坏死和肠系膜弥漫性纤维化。明确诊断可能需要病理证实。如果疾病以严重弥漫性纤维化为特征,那么手术治疗的应用可能存在问题。