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原发性硬化性包裹性腹膜炎:一例报告。

Primary sclerosing encapsulating peritonitis: a case report.

机构信息

Medical Faculty, University of Ljubljana, Ljubljana, Slovenia.

University Medical Center Ljubljana, Ljubljana, Slovenia.

出版信息

J Med Case Rep. 2023 Jul 14;17(1):318. doi: 10.1186/s13256-023-04020-x.

Abstract

BACKGROUND

Sclerosing encapsulating peritonitis is a rare condition with a typical macroscopic appearance, with fibrocollagenous membrane enclosing loops of the small intestine, causing intestinal obstruction. Unexplained recurrent abdominal pain, obstruction, and a large array of other possible clinical signs and symptoms make sclerosing encapsulating peritonitis a diagnostic challenge.

CASE PRESENTATION

A 48-year-old man of Persian ethnicity was admitted multiple times to the emergency surgery department due to recurrent sudden abdominal pain and chronic obstruction without significant findings in medical history or clinical evaluation. Computed tomography was positive for proximal jejunal dilatation and duodenojejunal flexure stenosis due to internal mesenteric hernia. Exploratory laparoscopy, followed by laparotomy, confirmed thick membrane-like fibrous tissue with complete small intestinal loop envelopment. Extensive membrane excision and adhesiolysis was performed, but no mesenteric herniation was found. Early postoperative paralytic ileus with introduction of low-dose steroid therapy, based on histopathological and immunological results, confirming type III sclerosing encapsulating peritonitis, was completely resolved.

CONCLUSION

Sclerosing encapsulating peritonitis is a rare and difficult-to-diagnose condition, further divided into primary and secondary sclerosing encapsulating peritonitis, on the basis of underlying etiology, dictating treatment modality and prognosis. Intraoperative diagnosis and surgical treatment are mandatory, besides a wide variety of abdominal computed tomography scans, inconclusive results, and clinical presentations. There are so far no known specific markers for the diagnosis of sclerosing encapsulating peritonitis.

摘要

背景

硬化性包裹性腹膜炎是一种罕见的疾病,具有典型的宏观外观,纤维胶原膜包裹小肠环,导致肠梗阻。原因不明的反复发作性腹痛、梗阻以及一系列其他可能的临床体征和症状,使得硬化性包裹性腹膜炎的诊断具有挑战性。

病例介绍

一名 48 岁的波斯裔男子因反复发作的突发性腹痛和慢性梗阻多次入住急诊外科病房,但在病史或临床评估中未发现明显异常。计算机断层扫描显示近端空肠扩张和十二指肠空肠曲狭窄,原因是肠系膜内疝。腹腔镜探查术,随后行剖腹术,证实为厚膜状纤维组织,完全包裹小肠环。进行了广泛的膜切除和粘连松解,但未发现肠系膜疝。术后早期出现麻痹性肠梗阻,根据组织病理学和免疫学结果,给予低剂量类固醇治疗,明确诊断为 III 型硬化性包裹性腹膜炎,完全缓解。

结论

硬化性包裹性腹膜炎是一种罕见且难以诊断的疾病,根据潜在病因进一步分为原发性和继发性硬化性包裹性腹膜炎,决定了治疗方式和预后。除了各种腹部计算机断层扫描、不确定的结果和临床表现外,术中诊断和手术治疗也是必需的。目前尚无用于硬化性包裹性腹膜炎诊断的特定标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b78/10347712/5deb57cdbdcd/13256_2023_4020_Fig1_HTML.jpg

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