A Rare Case of Rapunzel Syndrome with Multiple Small Bowel

Formosan Journal of Surgery (2016) 49,67e69

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CASE REPORT A rare case of Rapunzel syndrome with multiple small bowel intussusceptions and bowel obstruction Siu-Chung Ha a, Chee-Chee Koh a, Cheng-Hung Lee a,b,* a Department of Pediatric Surgery, Far Eastern Memorial Hospital, Pan-Ciao, New Taipei, Taiwan b Department of Surgery, Buddhist Dalin Tzu Chi General Hospital, Chiayi, Taiwan

Received 9 September 2015; received in revised form 27 September 2015; accepted 5 October 2015 Available online 15 February 2016

KEYWORDS Abstract Rapunzel syndrome is a rare condition that is characterized by a gastric trichobe- intussusception; zoar with a long tail extending from the stomach to the small bowel. Patients can be asymp- Rapunzel syndrome; tomatic for a long period, and symptoms develop later when the bezoar enlarges in size. The trichobezoar most common presentations include chronic abdominal pain, malabsorption, gastrointestinal tract obstruction, gastrointestinal bleeding, and intussusceptions. We report a case of a 5-year-old girl with Rapunzel syndrome causing multiple small bowel intussusceptions. We were unable to identify the main cause of this condition during laparoscopic reduction. Deﬁ- nite diagnosis was made after the development of gastrointestinal tract obstruction. Surgeons should consider the possibility of Rapunzel syndrome when diagnosing the main cause of intussusceptions. Copyright ª 2016, Taiwan Surgical Association. Published by Elsevier Taiwan LLC. All rights reserved.

1. Introduction

A small bowel intussusception is rare and can be caused by infection, polyp, lymphoma, vasculitis, Meckel’s diverticulum, intestinal duplication, cystic fibrosis, and intra- mural hematoma. Multiple simultaneous small bowel Conflicts of interest: The authors declare they have no conflicts intussusceptions are rarer.1,2 Rapunzel syndrome is an of interest. * Corresponding author. Department of General Surgery, Buddhist extremely rare intestinal condition resulting from a tri- Dalin Tzu Chi General Hospital, Number 2 Minsheng Road, Dalin chobezoar with a tail-like extension from the stomach to Township, Chiayi County 622, Taiwan. the small bowel. Rapunzel syndrome may present as E-mail address: [email protected] (C.-H. Lee). chronic abdominal pain, malabsorption, gastrointestinal http://dx.doi.org/10.1016/j.fjs.2015.10.002 1682-606X/Copyright ª 2016, Taiwan Surgical Association. Published by Elsevier Taiwan LLC. All rights reserved. 68 S.-C. Ha et al. tract obstruction, gastrointestinal bleeding, and intussusceptions. Few cases of Rapunzel syndrome in patients presenting with intussusceptions have been reported.3 We report a case of a 5-year-old girl with Rapunzel syndrome causing intussusceptions and bowel obstruction.

2. Case Report

A 5-year-old girl presented with severe abdominal pain and nausea that started 2 days before presentation. She experienced cramping pain in the left abdomen for three weeks without other gastrointestinal symptoms. On physical examination, a left palpable abdominal mass was noted. Abdominal echo revealed typical imaging ﬁndings but an unusual location for intussusceptions. Abdominal computerized tomography (CT) revealed multiple target signs (Figure 1) over the jejunum. Radiological reduction was unsuitable for the multiple jejunal intussusceptions; there- fore, laparoscopic exploration was performed. During operation, 8 segmental jejunal intussusceptions ranging from 2 cm to 8 cm in length were observed at a distance of 20e100 cm from the ligament of Treitz. All intussusceptions were laparoscopically reduced. A mass or diverticulum was not observed. The patient had a smooth recovery and was discharged on the 5th day after operation. Six weeks after the discharge, the patient returned with severe pain in the upper abdomen. In addition, she com- plained of vomiting soon after a meal and experienced less Figure 2 A bezoar located at the junction of the duodenum pain after vomiting. Abdominal CT revealed a long bezoar and jejunum with distended stomach (red arrows) is revealed impacted in the junction of the duodenum and jejunum through computerized tomography. with proximal gastrointestinal tract obstruction, which was not observed in the previous CT (Figure 2). Esophagogas- approximately 6 cm in length was removed through the troduodenoscopy was arranged for evaluating any other gastrostomy wound. The total length of both possible bezoars. During examination, a gastric bezoar bezoars including the long tail was approximately 30 cm consisting of long hairs was revealed; however, we were (Figure 3). The gastrointestinal tract was examined by unable to extract it endoscopically. Midline laparotomy palpation for other residual bezoars; neither a mass nor an with gastrotomy was then performed. After exploration, a gastric bezoar approximately 4 cm in length with a connection stalk to another larger duodenal bezoar

Figure 3 A gastric bezoar with a connection stalk to another larger duodenal bezoar is removed through gastrotomy. Figure 1 Multiple target signs (red arrows) are revealed D Z duodenal bezoar; G Z gastric bezoar; T Z long tail of the through computerized tomography. bezoar extracted from the proximal jejunum. Rapunzel syndrome with multiple intussusceptions 69 intussusception was observed. The patient recovered the reduction. In addition, during the first operation, the smoothly. During history taking, her parents revealed that patient probably had a gastric bezoar. We could not di- until recently, she had pulled and eaten her own hair for agnose the patient’s condition earlier through initial CT 3 months. She was then discharged and followed up in the because of the difficulty in distinguishing between a gastric psychiatry department. bezoar and food content retention. Before performing laparotomy, we planned to perform both gastrostomy and 3. Discussion enterostomy. However, because both the gastric and duodenal bezoars had a strong connection, we could remove both bezoars only through gastrostomy. Bezoars include trichobezoar (hair), phytobezoar (vege- In conclusion, we report a case of a 5-year-old girl with table material), lactobezoar (milk products), pharmaco- Rapunzel syndrome who had multiple intussusceptions and bezoar (medicines, particularly with cellulose residues), bowel obstruction caused by a trichobezoar with a long and plastobezoar (plastic materials). Rapunzel syndrome is tail. Multiple intussusceptions at unusual locations are an unusual form of trichobezoar. It is defined as a tricho- extremely rare. Surgeons should consider the possibility of bezoar with a tail-like extension from the stomach to the Rapunzel syndrome when diagnosing the cause of small bowel.4 Clinical presentation includes the presence of intussusceptions. a mass in the abdomen, abdominal pain, nausea, vomiting, weakness, weight loss, constipation, diarrhea, and malnu- trition.5 The bezoar itself can have a mass effect on the gastrointestinal tract, causing intestinal obstruction or gastric ulceration. The tail of the trichobezoar can inter- References fere with the peristalsis of the intestine and lead to intussusceptions.5 1. Pandey A, Rawat JD, Wakhlu A, Kureel SN, Gopal SC. Simulta- Most cases of Rapunzel syndrome are reported in neous occurrence of jejuno-jejunal and ileo-ileal intussusception in a child: a rare occurrence. BMJ Case Rep. 2011. http: teenage females with long hair. Surgical exploration re- //dx.doi.org/10.1136/bcr.08.2010.3294. mains the main treatment option for removing the tricho- 2. Singh JK, Bawa M, Kanojia RP, Ghai B, Menon P, Rao KL. Idio- bezoar. Enterotomy is advised for removing bezoars that pathic simultaneous intussusceptions in a neonate. Pediatr Surg extend into the intestine. After removal, the remaining Int. 2009;25:445e447. http://dx.doi.org/10.1007/s00383-009- bowel should be distally examined for any perforation or 2358-2. synchronous bezoar.4,5 3. Prasanna BK, Sasikumar K, Gurunandan U, Sreenath GS, Kate V. In our case, the patient experienced two complications Rapunzel syndrome: a rare presentation with multiple small of Rapunzel syndrome. One was multiple intussusceptions intestinal intussusceptions. World J Gastrointest Surg. 2013;5: at an unusual location resulting from the tail of the tri- 282e284. http://dx.doi.org/10.4240/wjgs.v5.i10.282. chobezoar, and the other was gastrointestinal obstruction 4. Gupta R, Prabhakar G, Mathur P, Goyal R, Sharma C, Ali M. Rapunzel syndrome and its variants in pediatric patients: Our caused by the mass effect of the body of the trichobezoar. experience. Arch Int Surg. 2014;4:152e157. http: We were unable to identify the main cause during the //dx.doi.org/10.4103/2278-9596.146417. first operation because we did not consider the possibility 5. Anantha RV, Chadi SA, Merritt N. Trichobezoar causing intus- of Rapunzel syndrome. Moreover, using laparoscopic in- susception: Youngest case of Rapunzel syndrome in a boy in struments for palpating small bundles of hair within the North America. J Ped Surg Case Rep. 2013;1:e11ee13. http: bowel was difficult because the bowel had swelled after //dx.doi.org/10.1016/j.jpedsurg.2011.11.002.