Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 6  |  Page : 813-815  

Rapunzel syndrome: Surgical problem in a neuropsychiatric disorder


1 Department of Surgery, Smt. Shardaben general Hospital, Saraspur, Ahmedabad, Gujarat, India
2 Department of General Medicine, GCS Medical College and Hospital, Ahmedabad, Gujarat, India

Date of Web Publication19-Nov-2015

Correspondence Address:
Gauravkumar Patel
26, Shukan-5 Residency, Behind Gujarat High Court, Sola, Ahmedabad -380 060, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.169944

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  Abstract 

The Rapunzel syndrome is an extreme form of trichobezoar found in patients with a history of psychiatric disorders, like trichotillomania (habit of hair pulling) and trichophagia (morbid habit of chewing the hair). The signs and symptoms are nonspecific and include vomiting, early satiety, abdominal pain, abdominal mass, and weight loss. Diagnosis can be established with a thorough history combined with radiography and endoscopy with high index of suspicion. Treatment options include combination of laparoscopy and/or laparotomy with long-term psychiatric treatment and monitoring. We report the case of an 17-year-old female, who presented with an abdominal pain, fullness after meals, nausea, vomiting and significant weight loss due to a trichobezoar with a long, 130 cm tail into the small bowel. The patient had an uneventful postoperative outcome after laparotomy and removal of trichobezoar.

Keywords: Bezoar-complications, Rapunzel syndrome, surgery and bezoars and psychiatric intervention, trichobezoar, trichophagia


How to cite this article:
Patel G, Surela R, Patel R. Rapunzel syndrome: Surgical problem in a neuropsychiatric disorder. Med J DY Patil Univ 2015;8:813-5

How to cite this URL:
Patel G, Surela R, Patel R. Rapunzel syndrome: Surgical problem in a neuropsychiatric disorder. Med J DY Patil Univ [serial online] 2015 [cited 2020 Oct 23];8:813-5. Available from: https://www.mjdrdypu.org/text.asp?2015/8/6/813/169944


  Introduction Top


Bezoars can be divided according to the primary constituent, namely, trichobezoars (hair), phytobezoars (plant material) or miscellaneous (pharmacobezoars, lactobezoars, fungal agglomeration and foreign bodies). When a long tail of hair extends into the small bowel and colon from the trichobezoar in stomach in the stomach, it is known as Rapunzel syndrome, a rare presentation of a trichobezoar.


  Case Report Top


A 17-year-old female patient presented with chief complaints of occasional epigastric and left lumber pain along with nausea, early satiety, fullness of abdomen after meals, weight loss for 1-2 months and recent onset of vomiting after meals. Patient was a known case of hypothyroidism and on regular medical treatment in the form of thyroxin. On physical examination, vitals were normal, an approximately 5 cm × 4 cm lump was palpated in epigastric region that moves well with respiration. Rest of the examination was normal. Computed tomography findings suggestive of jejunal intussusception with thickened wall of the stomach. Upper gastrointestinal endoscopy shows large trichobezoar extending into the duodenum [Figure 1]. Next day laparotomy was planned, trichobezoar was found in the stomach with 2-3 fragments that were removed with gastrotomy. A tail was found in distal jejunum extending into the colon up to the hepatic flexure, which was removed after enterotomy made proximal to the ileocecal junction [Figure 2] and [Figure 3]. Gastric biopsy was taken, and histopathological examination showed hyperplastic mucosa of the stomach. Patient had eventless recovery, and postoperative psychiatric reference was done. Patient had gained 5 kg weight after 6 months follow-up.
Figure 1: Upper gastrointestinal endoscopy shows large hairball extending into the duodenum

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Figure 2: Intra-operative photograph shows enterotomy with tail of trichobezoar coming out of it

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Figure 3: Full extent of trichobezoar which has 130 cm long tail extending into small and large bowel

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  Discussion Top


Accumulation of organic or nonbiological substances inside the gastrointestinal system is defined as bezoars among which trichobezoar (hair balls) are very rare and virtually exclusively found in premenarchal young female having neuropsychiatric disorders. [1],[2] However, when gastric trichobezoar extending into small and large bowel with a tail, it is called as Rapunzel syndrome which was first described by Vaughan et al. in 1968 and few case reports published in literature afterward. [2],[3] Few cases have been reported in pediatric age group and male patients too. [4],[5] Some authors describe Rapunzel syndrome as the trichobezoar with extension into the pylorus and others with extension up to jejunum and beyond, so there is no uniform consensus about its definition. [6] Psychiatric conditions like trichotillomania and trichophagia usually precede trichobezoar and Rapunzel syndrome later on. [6] Clinically patient may have a history of nonspecific abdominal pain, lump in the abdomen, anemia, vomiting, early satiety, and abdominal distension. [7] As the hair is an indigestible substance, Rapunzel syndrome usually present as a gastric outlet syndrome, sub-acute intestinal obstruction, ulceration, gastrointestinal bleeding, and perforation. [3] It may cause pancreatitis, appendicitis, intussusception. [4],[8],[9] Diagnosis of Rapunzel syndrome needed high index of suspicion and established by upper gastrointestinal endoscopy, ultrasonography and computed tomography scan after common etiologies have been excluded. [7] Currently surgical management of this condition is the treatment of choice, which includes open laparotomy, laparoscopy-assisted technique, and endoscopic intervention. [10] Javed and Agarwal 2013 described minimal invasive approach to remove large hairballs from stomach without risk of peritoneal contamination and short operative time which makes it an attractive alternative to laparotomy. Psychiatric consultation and long-term follow-up is of extreme value after patient has been cured by surgical intervention because they were reported cases of recurrence of Rapunzel syndrome in the literature. [11] Psychiatric intervention includes quetiapine, hair-extension removal, family involvement, anti-depressant treatment and behavioral therapy, which can prevent hair consumption. [12]

 
  References Top

1.
Islek A, Sayar E, Yilmaz A, Boneval C, Artan R. A rare outcome of iron deficiency and pica: Rapunzel syndrome in a 5-year-old child iron deficiency and pica. Turk J Gastroenterol 2014;25: 100-2.  Back to cited text no. 1
    
2.
Belsky J, Whitcomb V, Zimmerman E, Stankovic C. Rapunzel syndrome: Diagnosis via radiograph and history. Pediatr Emerg Care 2014;30:352-3.  Back to cited text no. 2
    
3.
Tegene T, Foda Y, Hussain O, Oloniyo K, Ha NT, Manikonda G. Rapunzel syndrome: A rare postpartum case. Case Rep Gastrointest Med 2013;2013:857374.  Back to cited text no. 3
    
4.
Kohler JE, Millie M, Neuger E. Trichobezoar causing pancreatitis: First reported case of Rapunzel syndrome in a boy in North America. J Pediatr Surg 2012;47:e17-9.  Back to cited text no. 4
    
5.
Middleton E, Macksey LF, Phillips JD. Rapunzel syndrome in a pediatric patient: A case report. AANA J 2012;80:115-9.  Back to cited text no. 5
    
6.
Veloso N, Silva JD, Gonçalves L, Medeiros I, Godinho R, Viveiros C. Trichotillomania and trichophagia: The causes of Rapunzel syndrome. Rev Esp Enferm Dig 2013;105:103-4.  Back to cited text no. 6
    
7.
Prasanna BK, Sasikumar K, Gurunandan U, Sreenath GS, Kate V. Rapunzel syndrome: A rare presentation with multiple small intestinal intussusceptions. World J Gastrointest Surg 2013;5:282-4.  Back to cited text no. 7
    
8.
Dogra S, Yadav YK, Sharma U, Gupta K. Rapunzel syndrome causing appendicitis in an 8-year-old Girl. Int J Trichology 2012;4:278-9.  Back to cited text no. 8
    
9.
Singh S, Wakhlu A, Pandey A, Gupta A, Ahmed I, Chandra N. Complicated Rapunzel syndrome mimicking intussusception. BMJ Case Rep 2011;2011.  Back to cited text no. 9
    
10.
Javed A, Agarwal AK. A modified minimally invasive technique for the surgical management of large trichobezoars. J Minim Access Surg 2013;9:42-4.  Back to cited text no. 10
    
11.
Tiwary SK, Kumar S, Khanna R, Khanna AK. Recurrent Rapunzel syndrome. Singapore Med J 2011;52:e128-30.  Back to cited text no. 11
    
12.
Jones GC, Coutinho K, Anjaria D, Hussain N, Dholakia R. Treatment of recurrent Rapunzel syndrome and trichotillomania: Case report and literature review. Psychosomatics 2010;51: 443-6.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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