Rapunzel Syndrome Diagnosed on CT Scan in Adolescent Girl with Neuropsychiatric Disorder
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Journal of Islamabad Medical & Dental College (JIMDC); 2015:4(3):125-127 Case Report Rapunzel Syndrome Diagnosed on CT Scan in Adolescent Girl with Neuropsychiatric Disorder Saba Binte Kashmir1, Shazia Yusuf2, Raja Amjad Mahmood3 1 Postgraduate resident Department of Radiology-CDA Hospital, Islamabad 2 Head, Department of Radiology-CDA Hospital, Islamabad 3 Director Children Hospital, PIMS, Islamabad Abstract Case Presentation Rapunzel syndrome is a rare type of trichobezoar with A 13-year-old girl was referred to our radiology imaging an extension of the hair ball into the small bowel. It is department, with history of epigastric pain and vomiting seen in adolescent girls with neuropsychiatric disorders. with vomitus containing hair for almost one year. She was We present a 13 years old girl with rapunzel syndrome treated in different primary health care centres as a case of presenting with history of epigastric pain and vomiting gastroenteritis and acid peptic disease. On examination an containing hair for last 1 year. Rapunzel syndrome is a oblong mobile well defined mass was palpable at rare form of trichobezoar, with a tail extending into the epigastrium. On questioning retrospectively her parents gave small intestine. It has a varied presentation and is seen in positive history of tricophagia. We performed CT abdomen females with psychiatric disturbance. and pelvis with contrast of this patient. CT scan showed Key words: Neuropsychiatric disorders, Repunzel distended stomach with well-defined air containing filling syndrome, Trichobezoar. defects extending from stomach into duodenum and jejunal loops (Figure 1). There was also evidence of multiple spontaneous intussusceptions involving small bowel loops Introduction in the left upper quadrant (Figure 2). Prompted by these Bezoar is defined as hard indigestible mass of material such diagnostic findings and positive history of trichophagia as hair, plant fibers or seeds formed in the alimentary canal patient was referred for surgical and psychiatric of animals and occasionally humans. It was formerly management. considered to be a useful medicine with magical properties and apparently still used for this purpose in some countries. According to the substance forming the ball, may be termed Discussion trichobezoar (hair ball), trichophytobezoar (hair and Trichobezoars are commonly seen in young females, often vegetable fiber mixed) or phytobezoar (Vegetable food ball). with an underlying psychiatric problem. Clinical Other rare substances forming bezoars has also been presentation may be confusing. Patients are usually described in literature.1 Trichobezoar commonly occur in asymptomatic at beginning. A history of recurrent hospital patients with psychiatric disturbances who chew and visits with abdominal pain and vomiting is found in most swallow their own hair called trichophagia. Repunzel cases. History of trichophagia is not always present either syndrome is a rare form of trichobezoar in the stomach and due to embarrassment or the unintentional nature of the its tail extending into the small bowel.2 The lifetime problem. However prolonged intake of indigestible material prevalence is estimated to be between 0.6%-4% of the can lead to gastric outlet obstruction which may present overall population.3 The standard imaging modality of similarly to small bowel obstruction. choice to make a pre surgical diagnosis is CT scan of the First case of trichobezoar was reported by Baudamant in abdomen.4 1779.1 The postulated reason for formation in the stomach is that human hair especially long hair is resistant to digestion Corresponding Author: as well as peristalsis. So it tends to stay in stomach and over Dr Shazia Yusuf a period of time may form a large “hair ball” if hair eating (trichophagia) is continued. Most patients with trichobezoar E mail: [email protected] 5 Received: Nov 16th 2015; Accepted: Dec 2nd 2015 have a co-morbidity of trichotillomania. Trichotillomania also known as trichotillosis is an irresistible desire to pull 125 Journal of Islamabad Medical & Dental College (JIMDC); 2015:4(3):125-127 Figure 2. Multiple spontaneous intussusceptions in left Figure 1. Gastric bezoar extending into duodenum and upper quadrant. jejunal loops shows a well-defined intraluminal ovoid heterogenous mass 14,15 out one’s own hair.3 It was first described by Hallopeau in with interspersed gas. 18891. Repunzel syndrome is a rare form of trichobezoar located in the stomach and tail extending into the small Conclusion bowel. Repunzel syndrome is named after a fairy tale Trichobezoars should be considered as a differential written in 1812 by the Brothers Grimm about a young diagnosis in young females with history of trichophagia and maiden, Repunzel, with long tresses. No fixed criteria have trichotillomania. Diagnosis can be easily made with the use been described to define Repunzel syndrome. Some have of CT scan. Majority of these patients have an underlying defined it as a gastric trichobezoar with a tail extending upto psychiatric or social disorder, so a multidisciplinary 6 ileocecal junction. Some have defined it simply as a approach is essential to prevent recurrence of the problem. trichobezoar with a long tail which may extend to the jejunum, ileum or ileocecal junction and still others have defined it as a trichobezoar of any size which presents in the References 7 1. Faria AP, Silva IZ, Santos A, Avilla SGA, Silveria AE: The form of an intestinal obstruction. Trichobezoar can also be Rapunzel syndrome – a case report: trichobezoar as a cause found distally in the gastrointestinal tract without continuity of intestinal perforation (in Portuguese). J Pediatria (Rio J) with the stomach bezoar due to breakage and distal 2000;76:83–86. propulsion. Presentation ranges from nonspecific abdominal 2. Vaughan ED Jr, Sawyers JL, Scott HW Jr: The Rapunzel syndrome. An unusual complication of intestinal bezoar. or epigastric pain to a range of complications including Surgery 1968;63:339–343. ulceration, perforation of stomach or small intestine, gastric 3. Huynh M, Gavino AC, Magid M. "Trichotillomania". Semin outlet obstruction, intussusceptions, obstructive jaundice, Cutan Med Surg (June 2013);32 (2): 88–94. protein losing enteropathy, pancreatitis and death.8-13 The 1. 4.Belsky J1, Whitcomb V, Zimmerman E, Stankovic C. hair appears black due to denaturing of the hair protein by Rapunzel syndrome: diagnosis via radiograph and history. 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