Small-Bowel Obstruction Secondary to Ileal Trichobezoar in a Patient with Rapunzel Syndrome

Abstract

Bezoars are conglomerations of undigested foreign material retained in the gastrointestinal tract. Trichobezoar is a compact conglomeration of swallowed hair and constitutes less than 6% of all bezoars. Their most frequent location is in the stomach but they may extend through the pylorus into the small bowel. This condition is known as Rapunzel syndrome. Many patients may remain asymptomatic or present a mild form of the disease characterized by abdominal pain, early satiety, nausea, and vomiting. Complications may manifest as gastric outlet obstruction or bleeding, and intestinal obstruction. A 15-year-old female patient presented with clinical findings of intestinal obstruction. The patient suffered from depressive and anxiety disorders and trichotillomania, although trichophagy could not be assured. Alopecia circumscripta and irregular hair length on the scalp were identified. A computed tomography (CT) scan showed two images highly suggestive of trichobezoars, one in the stomach and the second one causing obstruction at the ileocecal valve. At laparotomy, both a mobile gastric trichobezoar with a tail extending to the duodenum and a trichobezoar causing obstruction at the ileocecal valve were removed. The postoperative course was uneventful. The passage of a detached trichobezoar fragment in a patient with Rapunzel syndrome may cause intestinal obstruction. CT is the preferred image modality for the evaluation of suspected trichobezoars in order to characterize their size and locations, the presence and level of obstruction, and complications such as ischemia or perforation. A case of small-bowel obstruction secondary to ileal trichobezoar in a patient with Rapunzel syndrome is herein reported.

Trichobezoar as a cause of pediatric acute small bowel obstruction

“…The majority of trichobezoars present with abdominal pain and a palpable mass and as previously discussed they are usually confined to the stomach . Occasionally there can be extension of a long “tail” of hair into the small intestine: This constitutes the so‐called Rapunzel syndrome, first described in 1968 and named after the German fairy‐tale character . There are a number of complications that can arise from trichobezoars depending on their size, location, and chronicity.…”

“…Severe malnutrition (from vitamin and protein malabsorption) has been described, as have appendicitis, obstructive jaundice, and small bowel intussusception . Anemia can arise from both iron malabsorption and chronic ulceration, while acute hematemesis is present in up to 6% of cases . If left undiagnosed, acute bowel obstruction occurs in 26% of patients and peritonitis from visceral perforation will affect 18% …”

“…Endoscopic retrieval has been described but is only successful in around 5% of cases of gastric trichobezoar, although it is slightly more effective for phytobezoar and lactobezoar . Gastric lavage and dissolution therapy using lipase, cola, and papain have also been attempted but the size and density of trichobezoars have limited the success of these methods …”

“…Several types of bezoar have been described including phytobezoar (plant material), trichobezoar (hair), lithobezoar (stones), pharmacobezoar (tablets), plasticobezoar (plastic material), and lactobezoar (milk proteins) . Phytobezoars are the most common type, while trichobezoars account for less than 6% of all cases of bezoars found in humans . Although they were first described in humans in 1779 affecting a 16‐year‐old boy, trichobezoars are a phenomenon which almost exclusively affects females in the second and third decades of life and are quite uncommon in the pediatric age‐group .…”

“…2 Phytobezoars are the most common type, while trichobezoars account for less than 6% of all cases of bezoars found in humans. 4 Although they were first described in humans in 1779 affecting a 16-year-old boy, trichobezoars are a phenomenon which almost exclusively affects females in the second and third decades of life and are quite uncommon in the pediatric age-group. 5,6 The development of trichobezoars is strongly associated with trichotillomania (hair pulling) and trichophagia (hair eating), but there are reported cases of patients ingesting hair which is not their own (for example, that of other family members and even toy dolls).…”