|Year : 2012 | Volume
| Issue : 4 | Page : 275-277
Rapunzel Syndrome Case Report: A 13-year-old Girl
Hakan Ozdemir, Zehra U Ozdemir, Ibrahim T Sahiner, Metin Senol
Department of General Surgery, Ministry of Health, Nevsehir State Hospital, Nevsehir, Turkey
|Date of Web Publication||26-Apr-2013|
Department of General Surgery, Ministry of Health Nevsehir State Hospital, Nevsehir
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Bezoars are masses, which are commonly encountered in patients after stomach surgery or in those with psychiatric problems, formed by the accumulation of intraluminal nondigestible substances that can lead to obstruction of the stomach and the small intestine. The anatomical changes in the gastrointestinal tract are known to cause bezoar formation. In the absence of an anatomical change, psychiatric disorders such as trichotillomania may lead to the formation of trichobezoars in the stomach. The so-called Rapunzel syndrome is the extension of the bezoars down to the duodenum and the jejunum, which is a rare condition. In this paper, a 13-year-old patient with trichotillomania is reported, who was admitted to our clinic with nausea, vomiting, and fatigue complaints, in whom a giant trichobezoar was identified, which completely filled the stomach and duodenum, without causing obstruction.
Keywords: Rapunzel syndrome, trichobezoar, trichotillomania
|How to cite this article:|
Ozdemir H, Ozdemir ZU, Sahiner IT, Senol M. Rapunzel Syndrome Case Report: A 13-year-old Girl. Int J Trichol 2012;4:275-7
| Introduction|| |
Bezoars are masses formed by indigestible food or foreign substances in the gastrointestinal tract. These masses may occur due to leafy vegetables (fitobezoar), medications such as antacids (farmobezoar) or hair and fat (trichobezoar). 
The bezoars are most commonly located in the stomach in the gastrointestinal tract, particularly after gastric surgery. In cases without past surgery, the underlying etiology for the bezoar is usually a psychotic disorder such as trichotillomania. 
In 1968, Vaughan, et al. described Rapunzel Syndrome, which is characterized by the prolongation of a gastric trichobezoar in the duodenum and/or jejunum.  The ingested hair pieces are combined with mucus and food particles over a long period, causing the formation of a trichobezoar. The extension of this mass of hair in the small intestine can result in intestinal obstructions and perforations.
Trichobezoars can cause nonspecific symptoms in the early stages. Later, they can cause symptoms such as chronic abdominal pain, stomach ulcer, perforation, stomach bleeding, intussusception, and obstruction.  In cases of trichobezoar observed in young women, the coexistence of psychiatric disorders such as trichotillomania and trichophagy should be kept in mind. Trichobezoars may also be associated with other psychiatric disorders such as compulsive obsessive disorders, pica, depression, and anorexia nervosa. 
| Case Report|| |
A 13-year-old girl was admitted with complaints of abdominal pain, nausea, and vomiting. She experienced abdominal pain for approximately 5 months and had complaints of nausea and vomiting for the previous one month. Physical examination revealed epigastric tenderness and a hard mass with smooth margins. No pathologic findings were detected in the patient's laboratory findings, except for iron deficiency anemia.
The gastroscopy, which was carried out under anesthesia, revealed a trichobezoar formed by a hair mass, which filled the entire stomach. During the interview with the patient after the procedure, it was found out that the patient ate her own hair for about 10 months. The Psychiatry Department was consulted. Trichotillomania with anxiety disorder was diagnosed. A 50 mg/day dose of Sertraline was prescribed and the operation was postponed for 3 weeks. The patient was operated on after 3 weeks. At the operation, a 23 × 8 cm bezoar was removed as a whole through a gastrostomy incision of approximately 10 cm at the anterior surface of the gastric corpus [Figure 1] and [Figure 2]. There were no postoperative complications. The patient was discharged from the hospital with the advice of the Psychiatry Department.
| Discussion|| |
The trichobezoars, which are formed by indigestible hair or bristles, due to their keratinous structures, can be located throughout the entire intestinal tract, and generally in the stomach.  The trichobezoars are often encountered in young women and are frequently associated with an underlying psychiatric disorder. Usually they cannot be detected in the early stages. They are detected as hard masses in the epigastric region, bringing malignancy to mind. They can cause nonspecific symptoms such as epigastric pain, heartburn, and weight loss, and also can lead to ulceration, perforation, obstruction, and pancreatitis. 
Previous gastric surgery, gastroparesis, diabetes mellitus, reduction of gastric acid secretion, and prolonged gastric emptying are the factors that increase susceptibility to the formation of bezoars. Sometimes excess intake of indigestible substances alone can also cause the formation of a bezoar. 
The treatment of bezoars consists of the removal of these indigestible masses. Although noninvasive techniques such as medical treatment and enzymatic dissolution have been attempted, the reported success rates were low. , Endoscopic methods failed because of the difficulties in a majority of the cases of removal of large bezoars. ,, During the endoscopic removal, complications were seen such as pressure ulcers, esophagitis, and esophageal perforation. ,
Although there are successful results in the treatment of bezoars with the laparoscopic approach, the long duration of the operation compared to laparotomy is not desirable. The advantages of the laparoscopic approach are better cosmetic results, less post-operative complications and a short hospital stay.  Laparotomy, on the other hand, is an important treatment option due to its advantages such as shorter duration of operation, simplicity of the procedure, low complication rate, possibility of exploration of satellite lesions in the gastrointestinal tract, and successful results. Laparotomy should be the treatment of choice for large gastrointestinal tract bezoars, whereas laparoscopic and endoscopic procedures should be preferred for smaller ones.
Unlike the cases in the literature, in the present case, the psychiatric assessment and treatment were done preoperatively. In the preoperative period, the patient had serious anxiety and suicidal thoughts. Upon the evaluation of the clinical situation with the psychiatrist, it was concluded that the surgical procedure may worsen the psychiatric table of the patient. For this reason, it was decided that the treatment would begin with psychological and medical support, and the surgical planning would be done after the clinical condition became stable. The surgical treatment of the bezoar, which filled the entire stomach and was protruding into the duodenum, was postponed. The trichotillomania, which is a chronic condition, does not require emergency surgery, as long as there are no clinical complications. For this reason, it is believed to be appropriate to perform surgical procedures after stabilizing the psychological condition of the patient, in pathologies such as trichotillomania, associated with chronic psychiatric disorders. In psychiatric disorders such as trichotillomania, the attempt to solve the individual's problems will help to increase the patient compliance and the patient acceptance of treatment recommendations. In this case, improvement was observed in the preoperative period after the psychiatric treatment and the postoperative period was uneventful.
In conclusion, the trichobezoar cases secondary to trichotillomania, accompanied by an underlying psychiatric disorder, should be treated with the appropriate surgical method after the initiation of treatment for the psychiatric disorder.
| Acknowledgment|| |
On behalf of my co-authors, I hereby declare that, this study neither will be submitted nor send for possible submission to elsewhere until your final decision is given. We declare that there is no competing or conflict of interest of the researchers.
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[Figure 1], [Figure 2]