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腹茧症,一种导致肠梗阻的罕见疾病实体的病例报告。

Abdominal cocoon syndrome, a case report of a rare disease entity causing intestinal obstruction.

作者信息

Mohammed Farah, Abdulkarim Mohamed, Ibn Yasir Ammar, Taleballah Osman, Shani Dafalla, Salih Nadir

机构信息

MBBS, National Ribat University, Sudan.

Alzaiem Alazhari University - Department of Surgery, Sudan.

出版信息

Int J Surg Case Rep. 2021 Oct;87:106401. doi: 10.1016/j.ijscr.2021.106401. Epub 2021 Sep 13.

Abstract

INTRODUCTION AND IMPORTANCE

Abdominal cocoon syndrome is a rare condition characterized by small bowel encapsulation by a fibrous membrane or a cocoon-like sac. It is an uncommon cause of intestinal obstruction. Less than 300 cases have been reported from all over the world. This is the first case of such a disease entity to be reported from Sudan.

CASE PRESENTATION

A young female patient, presented with features of intestinal obstruction that was managed conservatively. Failure of the conservative management has warranted a laparotomy.

CLINICAL FINDINGS AND INVESTIGATIONS

Her features were suggestive of intestinal obstruction that was confirmed radiologically.

INTERVENTIONS AND OUTCOME

Laparotomy revealed a membrane-like fibrous material and extensive multiple loops adhesions, findings consistent with primary sclerosing encapsulating peritonitis (PSEP), also known as abdominal cocoon's disease. The membrane was excised and adhesiolysis was done. Intestinal obstruction was relieved after surgery and the patient showed good outcome.

CONCLUSIONS

Abdominal cocoon syndrome is a rare cause of intestinal obstruction.

RELEVANCE AND IMPACT

The takeaway lesson from this case would be that the PSEP should be sought in any patient with no clear cause for obstruction can be identified. A contrast-enhanced CT scan is the diagnostic modality of choice. Finally, we think that the disease is underreported from Africa and more efforts should be carried out to increase patients' access to healthcare especially in rural areas with no access to hospitals in order to bring more cases to light. This case report has been reported in line with the SCARE Criteria (Agha et al., 2020 [17]).

摘要

引言与重要性

腹茧症是一种罕见疾病,其特征为小肠被纤维膜或茧样囊包裹。它是肠梗阻的一种不常见病因。全世界报道的病例不足300例。这是苏丹首次报道的此类疾病病例。

病例介绍

一名年轻女性患者,表现出肠梗阻症状,最初接受了保守治疗。保守治疗失败后进行了剖腹手术。

临床发现与检查

其症状提示肠梗阻,经影像学检查得以确诊。

干预措施与结果

剖腹手术发现了膜样纤维物质和广泛的多处肠袢粘连,这些发现与原发性硬化性包裹性腹膜炎(PSEP)相符,PSEP也被称为腹茧症。切除了该膜并进行了粘连松解术。术后肠梗阻得到缓解,患者预后良好。

结论

腹茧症是肠梗阻的罕见病因。

相关性与影响

该病例的经验教训是,对于任何无法明确梗阻原因的患者,都应考虑原发性硬化性包裹性腹膜炎。增强CT扫描是首选的诊断方式。最后,我们认为非洲地区对该疾病的报道不足,应做出更多努力,以增加患者获得医疗保健的机会,特别是在无法就医的农村地区,以便发现更多病例。本病例报告是按照SCARE标准(Agha等人,2020 [17])撰写的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0aac/8449068/d34c6e42b4ab/gr1.jpg

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