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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 16  |  Issue : 2  |  Page : 103-104

Laparoscopic management of rapunzel syndrome


Department of General Surgery, Apollo BGS Hospitals, Mysore, Karnataka, India

Date of Submission05-Jan-2019
Date of Acceptance03-Apr-2019
Date of Web Publication19-Jun-2019

Correspondence Address:
Jayanth Bannur Nagaraja
Apollo BGS Hospitals, Adichunchunagiri Road, Kuvempunagar, Mysore - 570 023, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_96_18

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  Abstract 


Rapunzel syndrome is caused by ingestion of hairs, which leads to formation of trichobezoar, which can extend up to small intestine especially in girls. Utility of laparoscopic approach for this condition is not well established in this population. We are presenting a case of Rapunzel syndrome in a 14-year-old girl presenting as a cause of gastric outlet obstruction. Early diagnosis and extraction of trichobezoar by laparoscopic-assisted approach in children may be a better approach with respect to cosmesis, lesser morbidity, and early recovery.

Keywords: Gastrotomy, laparoscopy, pediatric, Rapunzel syndrome, trichobezoar, trichotillomania


How to cite this article:
Nagaraja JB, Saraf S, Shivathirthan N, Srinath S. Laparoscopic management of rapunzel syndrome. Apollo Med 2019;16:103-4

How to cite this URL:
Nagaraja JB, Saraf S, Shivathirthan N, Srinath S. Laparoscopic management of rapunzel syndrome. Apollo Med [serial online] 2019 [cited 2019 Aug 18];16:103-4. Available from: http://www.apollomedicine.org/text.asp?2019/16/2/103/260694




  Introduction Top


Bezoars are mass of foreign or intrinsic undigestable material found in gastrointestinal tract most commonly in stomach. If this foreign material is hairs, it is called as trichobezoars. A rare manifestation of trichobezoar is “RAPUNZEL SYNDROME”. It was described by Vaughan ED Jr. et al. in 1968 which is characterized by prolongation of giant trichobezoar into duodenum/jejunum. Ingested hair pieces are combined with mucus and food particles over long period leading to formation of trichobezoar.[1] It presents with nonspecific abdominal symptoms initially such as abdominal pain, nausea, vomiting, later can present as obstruction, weight loss, malnutrition, perforation, and peritonitis.[2] Early diagnosis and treatment of the such cases is of great importance.


  Case Report Top


A 14-year-old girl presented with a history of trichotillomania, weight loss in the last 3 years, poor appetite, nonbilious vomiting – multiple episodes, early satiety, and severe malnutrition. On general physical examination, she had mild pallor and cachexia. On per abdomen examination, large firm mass of 24 cm × 12 cm × 8 cm noted over epigastric region involving umbilical, right hypochondrium, and right lumbar region which was nontender and nonpulsatile. Ultrasonography done in view of abdominal pain few months back showed normal study. Hence, we went ahead with computed tomography (CT) scan whole abdomen which revealed heterogenous, mottled intraluminal mass with low attenuation of 24 cm × 10 cm × 6 cm [Figure 1]. Upper gastrointestinal endoscopy done which revealed giant trichobezoar admixed with food residues in stomach extending from fundus up to duodenum. Patient nutrition status was taken care of and she was optimized for surgery. She underwent laparoscopic gastrotomy and extraction of trichobezoar through Pfannenstiel incision. It revealed a giant trichobezoar of 24 cm × 13 cm × 7 cm extending up to duodenum mixed with foul-smelling undigested food particle of weight: 2.9 kg. [Figure 2] showing gross specimen of giant trichobezoar postoperatively.
Figure 1: Computed tomography scan abdomen showing heterogeneous, concentric, mottled intraluminal mass with low attenuation – trichobezoar

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Figure 2: Gross specimen of giant trichobezoar postoperative

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  Discussion Top


Rapunzel syndrome is rare type of trichobezoar which extends up to duodenum/jejunum. Incidence of trichobezoar is 0.4%.[3] History of trichotellomania is present in 50% of cases. More than 80% of trichobezoar are noted in <30 years of age group. Bezoars are most commonly located in stomach particularly after gastric surgery or anatomical changes. In the absence of anatomical changes or any past surgical history, psychotic disorders could be underlying etiology such as trichotillomania, Obssesive compulsion disorder, pica, depression, and anorexia. In our case, trichotillomania was the underlying cause for trichobezoar formation.

Early diagnosis is of great importance for this condition. Abdominal radiography should be done to reveal any distended stomach and small bowel dilatation. Chest radiograph should be done to rule out gastric or duodenal perforation in advance cases of trichobezoar. Ultrasound abdomen and CT scan can be performed to know nature, size, and position of mass. CT scan is the most accurate method. In [Figure 1], it is showing heterogenous, concentric, mottled intraluminal mass with low attenuation. Endoscopy is diagnostic technique of choice and has therapeutic potential in smaller bezoars. It can differentiate between trichobezoar and another foreign body that can be broken and removed endoscopically.

Successful management of bezoars requires removal of these undigestable substances and prevention of recurrence by psychological treatment. Endoscopic method fails primarily in large-sized bezoars. Complications such as ulcers, esophagitis, and perforations are common following this.[4] Other option which is most commonly performed is laparotomy followed by gastrostomy and extraction of trichobezoars. Advantages include simplicity, no contamination of peritoneal cavity, low complication rate, and successful results.[4]

A novel and minimally invasive option is laparoscopic approach, in which anterior gastrotomy and removal of trichobezoar is done. It is kept inside a sterile endobag to avoid contamination of the peritoneal cavity. Endo bag containing trichobezoar was extracted through a Pfannenstiel incision of around 8 cm which gave the patient better osmesis as compared to upper midline incision of laparotomy. It also enables less postoperative morbidity, shorter hospital stay, early recovery, and probably lesser postoperative complication in view of her pediatric age. After recovery, she was submitted for psychiatric counseling for trichotellomania and anxiety behavior aimed at to prevent recurrence. On follow-up after 1 year, she gained 15–20 kg of weight with healthy looks with no emotional disturbances and doing well with her academics and on psychiatric counseling.

Nirasawa et al. were the first to report on laparoscopic removal of trichobezoar.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, et al. Rapunzel syndrome reviewed and redefined. Dig Surg 2007;24:157-61.  Back to cited text no. 1
    
2.
Vaughan ED Jr., Sawyers JL, Scott HW Jr. The rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery 1968;63:339-43.  Back to cited text no. 2
    
3.
Pace AM, Fearne C. Trichbezoar in a 13 years old male: A case report and review of literature. Malta Med J 2003;15:39-40.  Back to cited text no. 3
    
4.
Kanetaka K, Azuma T, Ito S, Matsuo S, Yamaguchi S, Shirono K, et al. Two-channel method for retrieval of gastric trichobezoar: Report of a case. J Pediatr Surg 2003;38:e7.  Back to cited text no. 4
    
5.
Nirasawa Y, Mori T, Ito Y, Tanaka H, Seki N, Atomi Y, et al. Laparoscopic removal of a large gastric trichobezoar. J Pediatr Surg 1998;33:663-5.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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