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蓝色橡皮疱痣综合征合并肠套叠:一例报告及文献复习

Blue rubber bleb nevus syndrome with the complication of intussusception: A case report and literature review.

作者信息

Hu Zeming, Lin Xuan, Zhong Jianing, He Qingfang, Peng Qin, Xiao Jianbo, Chen Bin, Zhang Jianhong

机构信息

Department of General Surgery, The First Affiliated Hospital of Gannan Medical University, Ganzhou, Jiangxi Province, China.

Key Laboratory of Prevention and Treatment of Cardiovascular and Cerebrovascular Diseases, Ministry of Education, Gannan Medical University, Ganzhou, Jiangxi Province, China.

出版信息

Medicine (Baltimore). 2020 Jul 10;99(28):e21199. doi: 10.1097/MD.0000000000021199.

Abstract

RATIONALE

Blue rubber bleb nevus syndrome (BRBNS) is an extremely rare disorder characterized by multifocal venous malformations involving various organs such as the skin and gastrointestinal tract. Severe complications of BRBNS, such as intussusception, volvulus, and intestinal infarction are rarer and require surgery. This report describes a 33-year-old male of BRBNS complicated with intussusception that was successfully diagnosed and treated with surgery.

PATIENT CONCERNS

A 33-year-old Chinese man presented with persistent, colicky pain accompanied by nausea, abdominal distension, and dizziness. The patient presented with sporadic bluish nodules on his skin involving his head, neck, thorax, abdomen, and planta pedis.

DIAGNOSES

BRBNS with the complication of intussusception.

INTERVENTIONS

An emergency laparotomy was performed, and postoperative management included blood transfusions and oral iron supplementation for 2 weeks.

OUTCOMES

The patient's postoperative course of hospitalization was uneventful. During the 4-month follow-up, the patient showed no signs of intussusception recurrence.

LESSONS

Patients diagnosed with BRBNS who present with acute abdominal pain and distension should raise suspicion for the presence of intussusception, which requires emergent surgical intervention.

摘要

理论依据

蓝色橡皮疱痣综合征(BRBNS)是一种极为罕见的疾病,其特征为累及皮肤和胃肠道等多个器官的多灶性静脉畸形。BRBNS的严重并发症,如肠套叠、肠扭转和肠梗死较为罕见,需要进行手术治疗。本报告描述了一名33岁患有BRBNS并并发肠套叠的男性患者,该患者经手术成功诊断并治疗。

患者情况

一名33岁的中国男性,出现持续性绞痛,伴有恶心、腹胀和头晕。患者头部、颈部、胸部、腹部和足底皮肤出现散在的蓝色结节。

诊断

BRBNS并发肠套叠。

干预措施

进行了急诊剖腹手术,术后管理包括输血和口服铁剂补充治疗2周。

结果

患者术后住院过程顺利。在4个月的随访中,患者未出现肠套叠复发迹象。

经验教训

诊断为BRBNS且出现急性腹痛和腹胀的患者,应怀疑存在肠套叠,这需要紧急手术干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/01b2/7360318/cedf985c3492/medi-99-e21199-g001.jpg

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