Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature
Case Report
P: 113-118
March 2024

Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature

J Ankara Univ Fac Med 2024;77(1):113-118
1. Hacettepe University Faculty of Medicine, Departments of Pediatric Surgery, Ankara, Türkiye
No information available.
No information available
Received Date: 05.09.2023
Accepted Date: 03.11.2023
Publish Date: 05.04.2024


Rapunzel syndrome (RS) is a rare condition presenting with hair accumulation in the stomach that extends to small intestine. It may cause gastric outlet and intestinal obstruction. The most common clinical signs are abdominal pain, palpable mobile mass and vomiting. A 12-year-old girl with a history of trichotillomania, trichophagia, attention deficit and hyperactivity disorder presented with gastric outlet obstruction. A bezoar completely filling the stomach and reaching to third part of the duodenum was detected in abdominal ultrasound. Removal of the trichobezoar via gastrotomy was the treatment of choice. Herein we report a 12-year-old girl with RS to discuss the clinical features and treatment options in this rare presentation.

Keywords: Bezoar, trichobezoar, Rapunzel Syndrome, child


Bezoars are concretions that occur as a result of the accumulation of non-absorbable fibers such as hair and plant fibers in the gastrointestinal tract. The most common type of bezoar is the trichobezoar, which is mostly made of hair. Trichobezoar is a rare condition almost exclusively seen in young females (1). Rapunzel syndrome (RS) is an unusual and rare form of trichobezoar extending into the small intestine. The name “Rapunzel” syndrome comes from the Grimm Brothers’ fairytale and this syndrome was first described by Vaughan et al. in 1968 (2).

The most common clinical findings in trichobezoar cases are abdominal pain, palpable firm and mobile masses. However, more serious complications can also be seen in trichobezoars that extend to the small intestines, such as intestinal obstruction, perforation, intussusception, cholangitis and pancreatitis (3,4). A 12-year-old girl diagnosed with RS, which causes gastric outlet obstruction and has trichobezoars extending along the duodenum, is presented to discuss the difficulties experienced in the diagnosis and treatment of trichobezoars with acute gastric obstruction.

Case Presentation

A 12-year-old female, admitted to hospital with epigastric mass and non-bilious vomiting. Heart rate was 90/min, respiratory rate was 16/min, blood pressure was 120/80 mmHg and body temperature was 37.5 ºC. In her medical history that she has been following up for trichotillomania, trichophagia and attention deficit and hyperactivity disorder. She has been using methylphenidate (20 mg, Ritalin, USA) and risperidone (0.5 mg, Risperdal, Turkey) treatment. Laboratory findings were all within normal limits, including complete blood cell counts, serum electrolyte levels, liver and renal function tests. She complained of weight loss in last ten days. Abdominal examination revealed a non-tender palpable mass in the epigastric region. Abdominal ultrasonography (USG) revealed large foreign body completely filling the stomach. Abdominal computed tomography (CT) scan confirmed a large bezoar completely filling the stomach and extending to the third part of the duodenum (Figure 1). Upper gastrointestinal endoscopy revealed that her entire stomach was filled with trichobezoar and it extended from the gastric cardia to the pylorus. It was not possible to remove the bezoar with forceps and surgical exploration was planned in the same session. Laparotomy was performed with midline incision above umbilicus and the bezoar was removed by gastrotomy on the anterior aspect of the stomach (Figure 2).

Figure 1
Figure 2

She had an eventful postoperative recovery and fed orally on the 3rd postoperative. She was discharged after detailed psychiatric counselling on the 6th postoperative day. Two years later, she was admitted to our department with abdominal pain. She had stopped her psychiatric medical treatment for the last 6 months and started trichophagia. The USG showed no gastric outlet obstruction. She was consulted to pediatric psychiatry department again and has been following up with no symptoms.

A detailed PUBMED searched was performed by using the keywords “child, bezoar, obstruction, Rapunzel”. Table 1 summarizes these cases with RS with gastric outlet obstruction.

Table 1


The word “bezoar” comes from the Arabic word “bedzehr” or the Persian word “padzhar,” meaning “protecting against a poison” (3). The first reference to a bezoar in a human was in 1779 during an autopsy of a patient who died from gastric perforation and peritonitis (4). Bezoars are named as phytobezoar (fruit, vegetable fibres), lactobezoar (milk residues) and trichobezoar (hair, nylon) according to their content. Trichobezoars are due to the accumulation of indigestible materials such as hair, nylon or wool in the stomach associated with trichotillomania and trichophagia. Human hair is resistant to digestion and peristalsis. As the hair continues to be swallowed, it becomes a mass by accumulating in the stomach folds along with the mucus and nutrients. Although it is more common in girls, mainly because they have long hair, a boy eating his sister’s hair has also been reported (5).

RS is a rare form of trichobezoar and trichobezoars extend into the duodenum and small intestine. Affected patients can remain asymptomatic for a long time. The bezoar continues to grow and symptoms appear in the late stages. The most common findings in children are epigastric mass, epigastric pain, nausea, vomiting, weight loss, hematemesis, diarrhea or constipation (6). Besides causing gastrointestinal obstruction, they can also cause stomach ulcers, obstructive jaundice and acute pancreatitis (3,4). Protein-losing enteropathy, iron deficiency anemia and megaloblastic anemia are other complications related malabsorption.

The bezoar sometimes breaks off from the large mass and causes obstruction in the small intestines and may cause emergency surgical operation (7). In addition to obstruction, they can cause intussusception in different segments of the intestine. USG may be the first choice in diagnosis because the bezoar may cause intussusceptions (10). The acute surgical treatment in RS mostly correlates with obstructions and complications. Trichobezoars that usually cause partial gastric outlet obstruction and complete obstruction is extremely rare (3,11). We found 21 cases reporting RS that cause complete or partial gastric outlet obstruction with small intestine extension (Table 1). The most common clinical symptom was abdominal pain, palpable mass and vomiting. Most of the cases present with non-bilious vomiting. Our patient was diagnosed after the non-bilious vomiting and clinical findings of gastric outlet obstruction. One should be aware of whether trichotillomania is in the history or alopecia is on physical examination when bezoar is clinically suspected. The history of these patients may include other psychological disorders that cause bezoar, such as pica, obsessive compulsive disorder, depression, anorexia nervosa and substance abuse. In addition to psychiatric problems, social causes such as family quarrels, school changes, loss of parents can also create a predisposition (8). Our patient had a history of trichotillomania, tricophagia and psychological reasons such as the separation of the mother and father. However, the history of trichotillomania is suggestive for diagnosis, all patients with bezoars should consulted to psychological evaluation.

Trichobezoars can be detected as a palpable mobile, well-circumscribed mass in physical examination. Erect abdominal X-ray may be useful for diagnosis and evaluation of complications. However, despite the large ileal perforation due to trichobezoar, a case without pneumoperitonium on erect abdominal X-ray was reported, which was associated with the trichobezoar completely covering the perforated area (9). Trichobezoars can be diagnosed a firm epigastric mass in 88% of cases and USG is often the first imaging study performed in the clinical setting of a lump in the epigastrium (10). USG could be used to support diagnosis but few other causes of upper abdominal masses are showed intense sonic shadow like trichobezoar in USG (11). CT is another radiological option after USG to show the size and configuration of the bezoar and to differentiate it from gastrointestinal tumors such as gastrointestinal stromal tumor extending into the gastric lumen, thus it helps diagnose and demonstrate mechanical bowel complications (12). Although CT and magnetic resonance imaging (MRI) examination are used to exclude other mass causes in the differential diagnosis, upper gastrointestinal system endoscopy is also considered the gold standard for diagnosis. In this case, it was shown that the bezoar extended to the small intestine in the USG and CT performed before the endoscopy. In our case, the stomach was completely filled with hair and the attempts for endoscopic removal was failed. Although most of the patients underwent endoscopy, only one of the patients had successful endoscopic removal (13). Patients with partial gastric obstruction were more eligible for endoscopic treatment.

If endoscopical removal is not possible, removal of bezoars with gastrotomy via laparotomy is recommended. The first successful surgical intervention was made by Schonborn in 1885 (14). The first attempt at laparoscopy was made by Nirasawa et al. (15). In addition to surgical treatment, various other modalities such as extracorporeal shock wave lithotripsy (ESWL), enzymatic therapy, metoclopramide and acetylcysteine have been reported as successful treatment options (15). In our case, surgical treatment was the only choice because of very large trichobezoar completely filling the stomach. Patients with small intestinal extension should also undergo detailed exploration of the entire duodenum and jejunum.

Recurrence of trichobezoar is not uncommon. But psychological treatment is one of the most important steps in these patient’s treatment and it is also regular part to prevent recurrence. The most important evidence of this is that the case who had gastrotomy because of bezoar nine years ago, was operated again for bezoar at the age of twenty-five, because she continued her psychiatric treatment irregularly (16). In some cases, as in our patient, although the diagnosis is known, advanced tests and treatments may be required in cases who do not use their medications regularly and do not comply to appropriate treatment. Therefore, preventive measures should be taken into consideration in patients who are incompliant to their psychiatric treatment.


RS is a rare form of trichobezoars that can cause gastric outlet obstruction and require emergency surgical treatment. When endoscopic methods are insufficient in large masses, detailed imaging methods should be used to evaluate mass size and extension before the surgery. Psychiatric evaluation should be performed in all cases to prevent recurrence.


Informed Consent: Informed consent was obtained from the patient’s family.

Authorship Contributions

Conflict of Interest: All authors declare no conflict of interest.

Financial Disclosure: No financial and non-financial benefits have been received or will be received form any party related directly or indirectly to the subject of this article.


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