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先天性智力障碍患者的长发公主综合征需急诊剖腹手术。病例报告。

Rapunzel Syndrome in Congenital Mental Retardation Patient Requiring Emergency Laparotomy. Case report.

作者信息

Alibraheem Abdelaziz, Danial Aghyad, Kazan Amira, Masri Esraa, Obari Mohammad Yusr, Basha Samer Rajab

机构信息

Faculty of Medicine, University of Aleppo, Aleppo, Syria.

MD, Department of Surgery, Faculty of Medicine, University of Aleppo, Syria.

出版信息

Ann Med Surg (Lond). 2020 Sep 1;58:99-101. doi: 10.1016/j.amsu.2020.08.026. eCollection 2020 Oct.

Abstract

INTRODUCTION

When hair accumulates inside the stomach, it causes what is called a Trichobezoar, which leads to a stomach blockage. When the accumulated hair extends into the small intestine, it causes a rare disturbance called Rapunzel syndrome (RS).

DISCUSSION

Affected patients infrequently remain asymptomatic for several years. Symptoms begin while the bezoar increases in size to the point of obstruction, these symptoms are nonspecific like vomiting, nausea, anorexia, asymptomatic abdominal mass and digestive bleeding.

PRESENTATION OF CASE

The authors report an unusual case of a 25 years old young woman who presented with acute abdominal pain and gastrointestinal symptoms. With an upper gastroscopy, the condition was first diagnosed as a Trichobezoar that occupied the stomach. A decision to perform a surgical procedure was taken, only to discover, during the procedure, that the mass was extended to the duodenum and jejunum, thus diagnosing the condition as Rapunzel syndrome. The patient was managed by surgical removal of the huge mass.

CONCLUSION

Trichobezoar considers as a differential diagnosis for any patient with mental retardation and has gastrointestinal symptoms.

摘要

引言

当毛发在胃内积聚时,会形成所谓的胃石,进而导致胃梗阻。当积聚的毛发延伸至小肠时,则会引发一种罕见的病症,即长发公主综合征(RS)。

讨论

受影响的患者很少会在数年里一直无症状。症状在胃石增大到阻塞程度时开始出现,这些症状不具特异性,如呕吐、恶心、厌食、无症状腹部肿块及消化道出血。

病例呈现

作者报告了一例不寻常的病例,一名25岁年轻女性出现急性腹痛和胃肠道症状。通过上消化道内镜检查,该病症最初被诊断为占据胃部的胃石。决定进行手术,结果在手术过程中发现肿块已延伸至十二指肠和空肠,从而将该病症诊断为长发公主综合征。患者通过手术切除巨大肿块得到治疗。

结论

胃石应被视为任何患有智力障碍且有胃肠道症状患者的鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a31/7490442/8e3f251a0cba/gr1.jpg

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