The term
bezoar is derived from the Persian word
panzehar; or the Arabic word
badzehar, which means masses in the stomach of sacrificed animals used as antidote or antipoison.[
1] It is defined as a rare condition secondary to unusual accumulation of substances of different form forming solid masses or concretions inside the digestive tube, mostly in the stomach and sometimes in the small intestine.
Trichobezoars are composed of hair, fur or fibers. This pathology is observed almost exclusively in young girls (90%) aged less than 30 years, complaining of trichotillomania and trichophagia.[
1] Despite no reported underlying psychiatric troubles in some cases in the literature, some environmental and psychological factors such as a fragile personality, relational nonadaptation, parents, and depressive states may predispose individuals to this condition.[
1,
3] Trichobezoar may also occur in patients with digestive antecedents, as in pyloroantrectomy or esophagoplasty.[
3]
Rapunzel syndrome was described for the first time by Vaughan
et al. in 1968.[
2] It is a rare form of gastric trichobezoar with duodenal and jejunal extension. (The term Rapunzel-Raiponce in Germany is the name of a heroine with very long hair in the storybook of Grimm brothers.)[
3] Like in our patient, clinical picture has no specification; trichobezoar may stay asymptomatic for a long time or may manifest as epigastric discomfort (80%), abdominal pain (70%), nausea and vomiting (65%), asthenia with weight loss (38%) or intestinal transit troubles like diarrhea or constipation (33%).[
4,
5] Sometimes, the bezoar manifests itself with gastrointestinal complications such as upper gastrointestinal hemorrhage due to ulcerations, mechanical gastric or small intestinal occlusion, gastric or small intestinal perforation with peritonitis or subphrenic abscess, digestive fistula, cholestasis or acute pancreatitis due to obstruction of the ampulla of Vater by the prolongation of the trichobezoar as in Rapunzel syndrome.[
6]
In 85% of patients, clinical examination reveals a well-defined abdominal mass that is smooth, firm and mobile in the epigastric area.[
5] In our patient, the mass found by examination was due to dilatation of duodenojejunal flexure. Alopecia may also be noted in these patients. Endoscopy remains the examination of choice in the diagnosis of intragastric trichobezoar as it allows visualizing the hair threads, though it wasn’t the case in our patient. A normal oeso-gastroduodenal endoscopy does not exclude diagnosis of jejunal trichobezoar, as reported in many cases of Rapunzel syndrome.[
6] The barium follow-through examination shows an intraluminal gastric gap, which is mobile; with a convex border; and in case of a Rapunzel syndrome, there is a duodenal or jejunal extension corresponding to the trichobezoar prolongation.[
3] In some cases, abdominal x-ray can suggest the diagnosis by showing heterogeneous density, and abdominal ultrasound would confirm an intraluminal mass with a hyper-echoic arc-like surface and a marked acoustic shadow suggestive of a bezoar.[
4,
5] These examinations suggested the diagnosis in our patient, and performing CT scan did not seem necessary to us. In other reported cases, CT scan represented the examination of choice. It can delineate a well-defined oval intraluminal mass with air bubbles retained within the interstice or a homogenous mottled appearance in the region of the stomach or intestine.[
5] MRI allows making the diagnosis as the mass has variable signals in T1 and T2 and does not take up the contrast after a gadolinium injection.[
7] However, these two techniques are expensive and are not essential for the diagnosis of trichobezoar.
Small bowel bezoars are treated surgically. It is mandatory to explore the whole gastrointestinal tract in order to avoid synchronous bezoar and recurrence of intestinal obstruction due to retained bezoar.[
8]
The jejunal trichobezoar excision must be done by enterotomy. In our case, resection of the fissured jejunum was necessary.[
6,
8] Various therapeutic modalities have been proposed to treat trichobezoar and Rapunzel syndrome. Treatment options have been modified with the advent of laparoscopy.[
9]
Extraction of mass by endoscopy often fails and may lead to severe complications such as pneumomediastinum due to esophageal fissure. Dissolution of trichobezoar by Papain syrup is ineffective and is not proposed except for phytobezoars. The fragmentation of the ineradicable mass by YAG laser described in 1986[
10] constitutes a future perspective.[
3,
8,
10]
Psychological care of trichotillomania and trichophagia is difficult. The patient, as in our report, usually has a refusal attitude, which complicates the diagnosis and management. In cases of associated psychopathological problems such as compulsion, hyperkinetic and depressive syndromes, resort to behavioral treatment becomes necessary.[
11]