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Table of Contents   
CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 1  |  Page : 69-71
 

Rapunzel syndrome: A concealed tale of the misleading tail


Department of Pediatric Surgery, IMS, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Submission20-Jun-2022
Date of Decision28-Jul-2022
Date of Acceptance30-Jul-2022
Date of Web Publication10-Jan-2023

Correspondence Address:
Ruchira Nandan
Department of Pediatric Surgery, IMS, Banaras Hindu University, Varanasi - 221 005, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_86_22

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   Abstract 


The diagnosis of Rapunzel syndrome can sometimes be challenging. The silent features such as poor appetite, constipation, anemia, hypoalbuminemia, and hypoproteinemia were all overlooked in our patient initially. The Rapunzel tail leads to multiple intussusceptions and an unexpected perforation sealed by intussusceptions. Perforation sealed by intussusceptions in a clinically stable patient of Rapunzel syndrome has never been reported.


Keywords: Multiple intussusceptions, perforation, Rapunzel syndrome, trichobezoar


How to cite this article:
Kumar D, Pandey V, Nandan R. Rapunzel syndrome: A concealed tale of the misleading tail. J Indian Assoc Pediatr Surg 2023;28:69-71

How to cite this URL:
Kumar D, Pandey V, Nandan R. Rapunzel syndrome: A concealed tale of the misleading tail. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Feb 2];28:69-71. Available from: https://www.jiaps.com/text.asp?2023/28/1/69/367391





   Introduction Top


Rapunzel syndrome is named after a German princess who let her hair down the prison tower for her rescuer to climb up the window.[1] It is a rare disease, not so commonly seen in males and children like our patient. The symptoms are vague initially. A high degree of suspicion is required to make a correct and early diagnosis. Most of the cases are associated with psychiatric disorders such as trichophagia and trichotillomania.[2] The tail of the trichobezoar extending into the small intestine can act as a lead point for intussusception as in our case. Perforation was sealed by intussusceptions in our patient which prevented the clinical deterioration of our patient and the child was stable.


   Case Report Top


A 4-year-old male child presented with the complaint of intermittent episodes of severe pain abdomen and vomiting for 4 days. He was consulting a pediatrician for poor appetite and constipation for 1 year. He had low weight for age (12 kg), anemia (hemoglobin-8 g/dL), hypoproteinemia (protein-4.8 g/dL), and hypoalbuminemia (albumin-2 g/dL). Barium enema was advised, which was normal, and the patient was referred for surgical consultation because of the new onset of abdominal pain. There was no history of fever, hematemesis, hematochezia, melena, and jaundice. On general physical examination, the child was malnourished. He had long hair with no patches of alopecia. His vitals were stable. Epigastrium was distended [Figure 1]a. The abdomen was soft and nontender. There was no tenderness, guarding, or rigidity. No lump was palpable. Erect X-ray abdomen and ultrasound abdomen were normal. Barium meal showed a filling defect in the stomach which was suggestive of trichobezoars [Figure 1]b. On repeated interrogations, the parents agreed that the child sometimes eats his hair. On exploratory laparotomy, the stomach was distended, and multiple intussusceptions were noted in the jejunum [Figure 2]a. Mass was palpable in the stomach extending to the duodenum and jejunum. Gastrotomy was performed to remove the trichobezoar [Figure 2]b and [Figure 2]c. Intussusceptions were reduced as the whole of trichobezoar was extracted. A sealed perforation beneath one of the intussusceptions was noted. It was around 15 cm distal to the duodenojejunal flexure on the mesenteric side [Figure 2]d. Resection and anastomosis of the jejunum were done. Gastrotomy was closed. The postoperative period was uneventful. The patient was started orally on the postoperative day 5. Psychiatry consultation was done, and fluoxetine was started. At a follow-up of 6 months, the child is doing well and has gained weight. Hemoglobin, serum albumin, and total protein have improved.
Figure 1: (a) Distended epigastrium, (b) barium meal suggestive of trichobezoar in the stomach (retained contrast of barium enema done previously also seen)

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Figure 2: (a) Multiple intussusceptions, (b) trichobezoar removed through gastrotomy, (c) whole of the trichobezoar with tail, (d) perforation

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   Discussion Top


Trichobezoars are hair balls formed due to hair ingestion.[2] Hairs are resistant to digestion. They are not propelled by peristalsis because of their smooth surface and lie along the gastric folds. As they accumulate with other indigestible material over a period of time, they take the shape of stomach forming trichobezoar.[2] With continued hair ingestion, the trichobezoar extends into the small intestine and is termed Rapunzel syndrome.[1]

It is usually seen in young girls.[1] In our case, the patient was a 4-year male. Very few cases of trichobezoar have been described in a male child.[1] It may be difficult to diagnose because of nonspecific complaints in the early stages. Hence, diagnosis may be delayed leading to complications. The most common presentations are nausea, vomiting, pain abdomen, anorexia, constipation, diarrhea, weight loss, and malnutrition. Complications such as gastrointestinal obstruction, perforation, gastric ulcer, pancreatitis, obstructive jaundice, appendicitis, and gastrointestinal bleeding can occur.[2] The tail of the trichobezoar extending into the small intestine can act as a lead point for intussusception, as in our case.[3] Few cases of intussusception have been described, and fewer with multiple intussusceptions.[3],[4] In our case, a perforation was also present, which was sealed by intussusceptions.

The diagnosis of Rapunzel syndrome is made by ultrasonography, computed tomography scan, and endoscopy. Treatment may be endoscopic, laparoscopic, and laparotomy.[2] In patients who have developed symptoms acutely, surgical (laparoscopic/laparotomy) removal should be done. Laparoscopy is not preferred in large trichobezoars as it prolongs the operating time and increases the chances of the intra-abdominal spill and incision extension for trichobezoar retrieval. Inspection of the entire intestine for fragments and its retrieval from the distal intestine can challenging. The tail of trichobezoar may break and migrate distally causing intestinal obstruction later.

In an emergency, laparotomy is preferred as it may not be possible to remove the extension of trichobezoar in the small intestine which may be impacted and require an enterotomy. After trichobezoar retrieval, the intestine should be examined for any perforation or bezoar distally.

Endoscopic removal is less invasive and therefore seems an attractive option.[5] Small trichobezoars can be removed endoscopically. Removal of all fragments requires the repeated introduction of the endoscope. It has a disadvantage of low success rate and chances of airway obstruction. Even if the therapeutic role is debatable, it plays an important role in cases with diagnostic dilemmas.[2]

The treatment of RS does not end with retrieval of the trichobezoar. The patient must be kept in follow-up, and a psychiatric evaluation and follow-up are recommended to prevent a recurrence.[4] Behavioral therapy and pharmacotherapy (like selective serotonin reuptake inhibitors) may be needed.

To conclude, Rapunzel syndrome has nonspecific complaints in the early stages, which can delay diagnosis, leading to complications. The Rapunzel's tail is a rare cause of intussusception. Multiple intussusceptions are rarer. Perforation sealed by intussusceptions has never been reported in a case of Rapunzel syndrome. After trichobezoar retrieval, the intestine should be examined for any perforation or distal bezoar which may be missed.

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 initial s 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.
Kumar M, Maletha M, Bhuddi S, Kumari R. Rapunzel syndrome in a 3-year-old boy: A menace too early to present. J Indian Assoc Pediatr Surg 2020;25:112-4.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Gorter RR, Kneepkens CM, Mattens EC, Aronson DC, Heij HA. Management of Trichobezoar: Case report and literature review. Pediatr Surg Int 2010;26:457-63.  Back to cited text no. 2
    
3.
Bolívar-Rodríguez MA, Fierro-López R, Pamanes-Lozano A, Cazarez-Aguilar MA, Osuna-Wong BA, Ortiz-Bojórquez JC. Surgical outcome of jejunum-jejunum intussusception secondary to Rapunzel syndrome: A case report. J Med Case Rep 2018;12:362.  Back to cited text no. 3
    
4.
Anantha RV, Chadi SA, Merritt N. Trichobezoar causing intussusception: Youngest case of Rapunzel syndrome in a boy in North America. J Pediatr Surg Case Rep 2013;1:e11-3.  Back to cited text no. 4
    
5.
Esmaili MR, Abbasi HR, Baradaranfar MH. Respiratory arrest due to airway obstruction following endoscopic removal of Trichobezoar. J Pak Med Assoc 2011;61:700-1.  Back to cited text no. 5
    


    Figures

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