Rapunzel syndrome: a rare form of trichobezoar in the stomach with some extension into the small intestine

An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar

“…Trichobezoar is a compact and undigested foreign body of swallowed hair usually retained in the gastrointestinal tract, classified as Rapunzel Syndrome (RS) when extending from the stomach, the most frequent location, into the small bowel, which constitutes <6% of all bezoars. This condition is commonly observed in adolescents, particularly in young females with trichotillomania and trichophagia (1,2), and in patients with gastric motility disturbance. Our patient suffered both from behavioral disorders and possible gut motility impairment, as she had undergone intestinal resection for jejunal atresia and intestinal malrotation during her first year of life.…”

“…Clinical manifestations of trichobezoar depend on their size: at first patients may be asymptomatic until it reaches a large size; as the trichobezoar enlarges, symptoms develop and may include abdominal pain, nausea, vomiting, early satiety, and anorexia which in association with malabsorption result in malnutrition and macro-an micro nutrient deficiencies. Rarely, gastric bleeding and gastrointestinal obstruction may be observed as complications of large trichobezoar (1,2,5,6). Bezoars are a rare cause of bowel obstruction occurring in <1% of patients with intestinal obstruction (6,7) and in these circumstances they can cause intestinal plasma protein loss through the following mechanisms: (1) secondary intestinal lymphangiectasia due to mechanical obstruction of intestinal lymphatic drainage and (2) mucosal inflammatory injury (3).…”

“…The disease can be caused by abnormalities of the intestinal lymphatic system or impaired integrity of the mucosa; the diagnosis is suspected from clinical manifestations, but it can be confirmed by increased fecal level of alfa-1-antitrypsin. PLE and oedema may be very rarely onset manifestation of trichobezoar extending from stomach into the small bowel, therefore classified as Rapunzel Syndrome (RS) (1,2). In fact, as the trichobezoar enlarges can cause intestinal plasma protein loss through both mechanical obstruction of intestinal lymphatic drainage and mucosal inflammatory injury (3).…”

Trichobezoar as a cause of pediatric acute small bowel obstruction

“…When trichophagia is repeated and long‐standing, the accumulated strands of hair are retained in the gastric rugae because there is insufficient friction for them to be moved forward by peristalsis . The smooth and slippery surface of the trichobezoar, aided by entrapped food particles and mucus, further impairs its passage through the pylorus . At least 10% of patients have a history of psychiatric disturbance, behavioral disorders, or mental retardation, and a significant proportion of cases in children were observed in association with the recent death of a family member, recent parental separation, abuse, or socioeconomic disadvantage …”

“…Bezoars are hard indigestible masses which are most commonly encountered in the stomach because the pylorus acts as a natural impediment to further passage . The term is derived from the Arabic word “badzehr” meaning antidote, since bezoars from animals were traditionally used in ancient times as cures for poison and even prized as precious stones . Several types of bezoar have been described including phytobezoar (plant material), trichobezoar (hair), lithobezoar (stones), pharmacobezoar (tablets), plasticobezoar (plastic material), and lactobezoar (milk proteins) .…”