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腹茧症:急腹症综合征的罕见病因。

Abdominal cocoon syndrome: A rare cause of acute abdomen syndrome.

作者信息

Çolak Şükrü, Bektaş Hasan

机构信息

Department of General Surgery, Istanbul Training and Research Hospital, İstanbul-Turkey.

出版信息

Ulus Travma Acil Cerrahi Derg. 2019 Nov;25(6):575-579. doi: 10.14744/tjtes.2019.48380.

Abstract

BACKGROUND

A rare cause of acute abdomen or intestinal obstruction, the abdominal cocoon syndrome is also described in the literature as sclerosing peritonitis or sclerosing encapsulating peritonitis. Abdominal cocoon is characterized by the total or partial wrapping of the abdominal organs by a fibrous membrane. Although it is usually observed in young women, the etiology is unknown. The diagnosis is usually made during laparotomy. In this case series, we aimed to present seven patients diagnosed with abdominal cocoon syndrome during operation.

METHODS

The records of patients who underwent laparotomy for abdominal pain and/or intestinal obstruction in our hospital and diagnosed as abdominal cocoon during operation between January 2012 and November 2018 were retrospectively reviewed. The demographic characteristics of the patients, etiologic factors, surgical procedures, operative findings and follow-up of the patients were recorded.

RESULTS

Four out of seven patients who were operated for abdominal cocoon were male and 3 of them were female. The median age of patients was 61 (57-63) years in male and 39.6 (28-49) years in female. Six of the patients were operated in emergency conditions with the diagnosis of an acute abdomen or ileus. One of the patients was operated with the diagnosis of an intra-abdominal mass in elective conditions. In five out of seven patients, all of the small intestines were wrapped with a fibrous collagen capsule, while two of the patient intestines were partially wrapped with a fibrous collagen capsule. Four of the patients had no underlying disease, while one of the patients had Familial Mediterranean Fever (FMF), one had Endometriosis and one had beta-blocker medication. One patient who had small bowel necrosis and septic peritonitis were observed during the operation and died post operative 6th days. Postoperative complications were not observed in the follow-up of other patients and reoperation was not required due to recurrence.

CONCLUSION

Abdominal cocoon is a condition that is usually diagnosed during operation in patients that were operated for reasons, such as the acute abdomen or intestinal obstruction. When the diagnose delayed, death can be seen due to small bowel necrosis and septic complications. High clinical suspicion and radiological imaging are important in the preoperative diagnosis. Treatment is required adhesiolysis and excision of the fibrous membranes.

摘要

背景

腹茧症是急性腹痛或肠梗阻的罕见病因,文献中也将其描述为硬化性腹膜炎或硬化性包裹性腹膜炎。腹茧症的特征是腹部器官被纤维膜全部或部分包裹。虽然通常在年轻女性中观察到,但病因不明。诊断通常在剖腹手术时做出。在本病例系列中,我们旨在介绍7例在手术中被诊断为腹茧症的患者。

方法

回顾性分析2012年1月至2018年11月期间在我院因腹痛和/或肠梗阻接受剖腹手术并在术中诊断为腹茧症的患者记录。记录患者的人口统计学特征、病因、手术方式、手术发现及患者的随访情况。

结果

接受腹茧症手术的7例患者中,4例为男性,3例为女性。男性患者的中位年龄为61(57 - 63)岁,女性为39.6(28 - 49)岁。6例患者因急性腹痛或肠梗阻诊断而在急诊情况下接受手术。1例患者在择期情况下因腹腔内肿块诊断而接受手术。7例患者中有5例所有小肠均被纤维胶原囊包裹,而2例患者的肠管被纤维胶原囊部分包裹。4例患者无基础疾病,1例患者有家族性地中海热(FMF),1例有子宫内膜异位症,1例服用β受体阻滞剂。1例患者在手术中出现小肠坏死和感染性腹膜炎,术后第6天死亡。其他患者在随访中未观察到术后并发症,也未因复发而需要再次手术。

结论

腹茧症通常是在因急性腹痛或肠梗阻等原因接受手术的患者手术过程中被诊断出来的疾病。如果诊断延迟,可能会因小肠坏死和感染性并发症而导致死亡。术前诊断中高度的临床怀疑和影像学检查很重要。治疗需要进行粘连松解和纤维膜切除。

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