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[长发公主综合征:影像学诊断]

[Rapunzel syndrome: Radiological diagnosis].

作者信息

Lara-Zavala Yhessica Yhazmin, Álvarez-Ciaca Inés, Montiel-Jarquin Álvaro José, Bertado-Ramírez Nancy Rosalía, García-Galicia Arturo, Alonso-Torres Gisela

机构信息

Instituto Mexicano del Seguro Social, Centro Médico Nacional "General de División Manuel Ávila Camacho", Hospital de Especialidades de Puebla, Servicio de Radiología. Puebla, Puebla, México.

Instituto Mexicano del Seguro Social, Hospital General de Zona No. 20, Servicio de Radiología. Puebla, Puebla, México.

出版信息

Rev Med Inst Mex Seguro Soc. 2023 Jul 31;61(4):539-542. doi: 10.5281/zenodo.8200619.

DOI:10.5281/zenodo.8200619
PMID:37540757
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10484550/
Abstract

BACKGROUND

Rapunzel syndrome is a rare presentation of trichobezoar, secondary to the ingestion of hair known as trichophagia. This bezoar has been found mainly in women, it invades the stomach and extends to the small intestine. Clinically, patients present weight loss and chronic obstructive symptoms at the intestinal level. A case of Rapunzel syndrome is presented.

CLINICAL CASE

A 13-year-old female presented with a weight loss of 10kg in two months, chronic constipation, predominantly nocturnal vomiting, and abdominal pain of seven days' duration. Physical examination revealed decreased peristalsis and a palpable mass in the epigastrium. Laboratories taken on admission: normal blood count, kidney function tests, and liver function tests. The abdominal X-ray showed opacity in the fundus, body and gastric antrum, the abdominal ultrasound showed non-specific findings in the epigastrium, later an abdominal tomography was performed with a swallow of water-soluble contrast medium and showed occupation in the gastric lumen. She underwent exploratory laparotomy and the finding was a trichobezoar in the stomach with extension to the duodenum and part of the jejunum, which was removed without complications. The evolution of the patient was favorable.

CONCLUSIONS

For the diagnosis of Rapunzel Syndrome, the use of contrast imaging studies is necessary, and the treatment of choice is surgical.

摘要

背景

长发公主综合征是毛石病的一种罕见表现,继发于吞食毛发即食毛癖。这种毛石主要在女性中发现,它侵入胃并延伸至小肠。临床上,患者表现为体重减轻和肠道水平的慢性梗阻症状。本文报告一例长发公主综合征病例。

临床病例

一名13岁女性在两个月内体重减轻10kg,伴有慢性便秘、主要为夜间呕吐以及持续7天的腹痛。体格检查发现肠蠕动减弱,上腹部可触及肿块。入院时实验室检查:血常规、肾功能检查和肝功能检查均正常。腹部X线显示胃底、胃体和胃窦部不透光,腹部超声在上腹部显示非特异性结果,随后进行了口服水溶性造影剂的腹部断层扫描,显示胃腔内占位。她接受了剖腹探查术,发现胃内有一个毛石,延伸至十二指肠和部分空肠,手术切除且无并发症。患者恢复良好。

结论

对于长发公主综合征的诊断,使用造影成像检查是必要的,首选治疗方法是手术治疗。

相似文献

1
[Rapunzel syndrome: Radiological diagnosis].[长发公主综合征:影像学诊断]
Rev Med Inst Mex Seguro Soc. 2023 Jul 31;61(4):539-542. doi: 10.5281/zenodo.8200619.
2
Rapunzel syndrome: an infrequent cause of severe iron deficiency anemia and abdominal pain presenting to the pediatric emergency department.长发公主综合征:小儿急诊科中严重缺铁性贫血和腹痛的罕见病因。
BMC Pediatr. 2018 Apr 4;18(1):125. doi: 10.1186/s12887-018-1097-8.
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Rapunzel syndrome resulting in gastric perforation.导致胃穿孔的长发公主综合征。
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Giant gastric trichobezoar in a young female with Rapunzel syndrome: case report.一名患有长发公主综合征的年轻女性的巨大胃毛石:病例报告。
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Rapunzel syndrome: a rare form of trichobezoar in the stomach with some extension into the small intestine.长发公主综合征:一种罕见的胃内毛石症,部分延伸至小肠。
Acta Dermatovenerol Alp Pannonica Adriat. 2018 Sep;27(3):155-157.
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Rapunzel Syndrome. A rare cause of intestinal obstruction.雷普索尔综合征。一种罕见的肠梗阻病因。
Cir Cir. 2021;89(S2):90-93. doi: 10.24875/CIRU.20001407.
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The Rapunzel syndrome: a hard-to-swallow tale.雷普莎综合征:一个难以咽下的故事。
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[Intestinal obstruction due to a trichobezoar or Rapunzel syndrome: report of one case].[毛发粪石或长发公主综合征所致肠梗阻:1例报告]
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Gastric perforation secondary to Rapunzel syndrome.胃穿孔继发于拉潘综合征。
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Trichobezoar.毛粪石。
Tidsskr Nor Laegeforen. 2020 Nov 23;140(17). doi: 10.4045/tidsskr.20.0472. Print 2020 Nov 24.

引用本文的文献

1
Trichobezoar: An uncommon cause of abdominal pain.毛粪石:腹痛的罕见病因。
Med J Armed Forces India. 2024 Dec;80(Suppl 1):S412-S414. doi: 10.1016/j.mjafi.2023.08.016. Epub 2023 Oct 16.
2
Rapunzel Syndrome: Diagnostic Challenges and Multidisciplinary Treatment Strategies.长发公主综合征:诊断挑战与多学科治疗策略
Cureus. 2024 May 29;16(5):e61294. doi: 10.7759/cureus.61294. eCollection 2024 May.

本文引用的文献

1
Giant gastroduodenal trichobezoar: A case report.巨大胃十二指肠毛石症:一例报告。
World J Clin Cases. 2019 Nov 6;7(21):3649-3654. doi: 10.12998/wjcc.v7.i21.3649.
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Extremely unusual case of gastrointestinal trichobezoar.极其罕见的胃肠道毛粪石病例。
World J Clin Cases. 2015 May 16;3(5):466-9. doi: 10.12998/wjcc.v3.i5.466.
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A case of rapunzel syndrome.一例长发公主综合征病例。
Pediatr Gastroenterol Hepatol Nutr. 2013 Jun;16(2):127-30. doi: 10.5223/pghn.2013.16.2.127. Epub 2013 Jun 30.
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The rapunzel syndrome: an unusual trichobezoar presentation.长发公主综合征:一种罕见的毛粪石表现。
Case Rep Med. 2010;2010:841028. doi: 10.1155/2010/841028. Epub 2010 Mar 24.
5
Management of trichobezoar: case report and literature review.毛发石的管理:病例报告与文献综述
Pediatr Surg Int. 2010 May;26(5):457-63. doi: 10.1007/s00383-010-2570-0. Epub 2010 Mar 6.
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Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar.长发公主综合征:一例罕见毛粪石病例的全面综述
Clin Med Res. 2009 Sep;7(3):99-102. doi: 10.3121/cmr.2009.822. Epub 2009 Jul 22.
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The Rapunzel syndrome: is it an Asian problem? (case report and review of literature).《长发公主综合征:这是亚洲的问题吗?(病例报告和文献回顾)》
Eur J Gastroenterol Hepatol. 2009 Aug;21(8):937-40. doi: 10.1097/MEG.0b013e3283140ed2.
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Trichotillomania and trichobezoar: a clinical practice insight with report of illustrative case.拔毛癖与毛发石:临床实践见解及病例报告
J Am Osteopath Assoc. 2006 Nov;106(11):647-52.
9
Rapunzel syndrome: a diagnosis overlooked.长发公主综合征:一种被忽视的诊断。
Acta Paediatr. 2007 Jan;96(1):135-7. doi: 10.1111/j.1651-2227.2007.00012.x.
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[Rapunzel syndrome].
Gastroenterol Hepatol. 2005 Apr;28(4):259-61. doi: 10.1157/13073100.