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长发公主综合征:罕见的“断尾”故事。

Rapunzel Syndrome: Rare 'Tale' of a Broken 'Tail'.

作者信息

Chauhan Narvir S, Kumar Satish, Bhoil Rohit

机构信息

Department of Radiology, Dr. Rajendra Prasad Government Medical College, Kangra, India.

Department of Surgery, Dr. Rajendra Prasad Government Medical College, Kangra, India.

出版信息

Pol J Radiol. 2016 Apr 14;81:166-9. doi: 10.12659/PJR.896154. eCollection 2016.

Abstract

BACKGROUND

Rapunzel syndrome is a rare and unique form of trichobezoar, in which a cast of hairs is formed in the stomach with its 'tail' extending up to varying lengths into the small bowel. Almost all cases described in literature are of 'classic' and more common form of Rapunzel. Sometimes however, the tail of bezoar is broken into smaller fragments. There is not much literature available on this rarer subset of Rapunzel syndrome.

CASE REPORT

In this report we present the ultrasound (USG) and CT findings of a case of Rapunzel syndrome in which the tail had broken into five separate fragments. The patient presented clinically with intestinal obstruction due to the impaction of the distal- most fragment in the ileum.

CONCLUSIONS

Our case highlights the fact that although USG features may be suggestive, a careful evaluation of CT images is essential for a confidant preoperative diagnosis of Rapunzel syndrome. In cases of this syndrome with a broken tail, CT is essential for precise count and localization of the separated fragments to ensure their complete removal at the time of surgery. We also propose to name the fragments of the broken tail as 'bezoarlets'. This word aptly describes the tail fragments as it suggests their origin from the larger gastric bezoar and the suffix 'lets' conveys they are smaller in size.

摘要

背景

长发公主综合征是一种罕见且独特的毛石症,其表现为胃内形成一团毛发,其“尾端”向上延伸至小肠,长度不等。文献中描述的几乎所有病例都是“经典”且更常见的长发公主综合征形式。然而,有时毛石的尾端会断裂成较小的碎片。关于长发公主综合征这一较罕见亚型的文献并不多。

病例报告

在本报告中,我们展示了一例长发公主综合征的超声(USG)和CT检查结果,该病例中毛石的尾端已断裂成五个独立的碎片。患者临床上因最远端的碎片嵌顿在回肠而出现肠梗阻。

结论

我们的病例突出了这样一个事实,即尽管超声特征可能具有提示性,但仔细评估CT图像对于确诊长发公主综合征至关重要。在这种尾端断裂的综合征病例中,CT对于精确计数和定位分离的碎片以确保手术时将其完全清除至关重要。我们还提议将断裂尾端的碎片命名为“小毛石”。这个词恰当地描述了尾端碎片,因为它表明了它们源自较大的胃内毛石,后缀“小”表示它们尺寸较小。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/61db/4835156/c8db99c051fe/poljradiol-81-166-g001.jpg

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Rapunzel Syndrome: Rare 'Tale' of a Broken 'Tail'.长发公主综合征:罕见的“断尾”故事。
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