Kajal Pradeep, Bhutani Namita, Tyagi Niharika, Arya Pratibha
Deptt. of Pediatric Surgery, PGIMS, Rohtak, Haryana, India.
Deptt. of Pathology, PGIMS, Rohtak, Haryana, India.
Int J Surg Case Rep. 2017;40:23-26. doi: 10.1016/j.ijscr.2017.08.060. Epub 2017 Sep 8.
Trichobezoars are concretions formed by accumulation of hair in stomach. Usually, trichobezoar is confined to the stomach, but rarely it may extend from the stomach to the small intestine and even colon. This is an unusual form called Rapunzel syndrome. Our experience with this rare entity of Rapunzel syndrome and interesting entity of trichobezoar is being presented with review of literature.
We, at our institute, encountered four cases of trichobezoar in last five years, out of which two were found to be of Rapunzel syndrome. All of these cases were managed successfully by open surgical intervention in view of the very large size of the mass in all the cases.
The clinical presentation is highly variable ranging from asymptomatic cases diagnosed incidently to serious gastrointestinal symptoms and complications. Cases of trichobezoar have been reported in literature very infrequently but Rapunzel syndrome is extremely rare and less than 50 cases have been reported in medical literature till date.
Trichobezoar leading to Rapunzel syndrome is an extremely rare entity. The clinical presentation is usually vague and non-specific. Treatment is mainly surgical because of delayed presentation in majority of the cases. Psychiatric illness is the usual association.
胃石是毛发在胃内积聚形成的团块。通常,胃石局限于胃内,但很少会从胃延伸至小肠甚至结肠。这是一种不寻常的形式,称为长发公主综合征。我们将介绍我们在这种罕见的长发公主综合征以及有趣的胃石病例方面的经验,并对文献进行综述。
我们所在的机构在过去五年中遇到了4例胃石病例,其中2例被发现为长发公主综合征。鉴于所有病例中的肿块都非常大,所有这些病例均通过开放手术干预成功治疗。
临床表现差异很大,从偶然诊断出的无症状病例到严重的胃肠道症状和并发症。文献中报道的胃石病例非常少见,但长发公主综合征极为罕见,迄今为止医学文献中报道的病例不到50例。
导致长发公主综合征的胃石是一种极其罕见的病症。临床表现通常模糊且不具特异性。由于大多数病例就诊延迟,治疗主要是手术治疗。精神疾病是常见的关联因素。