Rapunzel syndrome (gastric trichobezoar), a rare presentation with generalised oedema: case report and review of the literature

Abstract

Rapunzel syndrome is a rare form of gastric trichobezoar. A 4-year-old girl presented with generalised oedema and an epigastric mass. Her family was of a relatively low socio-economic background. There was microcytic hypochromic anaemia, hypoalbuminaemia and an elevated α1-antitrypsin clearance. Abdominal ultrasound and non-contrast computed tomography demonstrated a heterogeneous mass related to the stomach. Upper gastro-intestinal tract endoscopy failed to remove it. Surgical laparotomy was undertaken through a single anterior gastrotomy incision and a large mass was extracted which was a cast of the duodenum and stomach and had a tail of approximately 60 cm in length which extended to the jejunum. Low socio-economic status, child neglect and pica are risk factors for trichobezoars. Surgical laparotomy is the optimal treatment of large bezoars.

Trichobezoar as a cause of pediatric acute small bowel obstruction

“…Given the rarity of TTM and trichophagia, it is estimated that only 1-2 people per million will be affected by trichobezoar. To our knowledge, there are only six documented cases of children younger than our patient having a trichobezoar 5,17,18,[21][22][23] and in only one of these was the site of impaction in the small intestine. 23 Rapunzel syndrome has been described as an uncommon form of this condition, but even more rare is the complete passage of a gastric trichobezoar through the pylorus into the small bowel causing a subsequent acute small bowel obstruction, as was found in our case.…”

“…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 .…”

“…Severe malnutrition (from vitamin and protein malabsorption) has been described, as have appendicitis, obstructive jaundice, and small bowel intussusception. [15][16][17] Anemia can arise from both iron malabsorption and chronic ulceration, while acute hematemesis is present in up to 6% of cases. 4 If left undiagnosed, acute bowel obstruction occurs in 26% of patients and peritonitis from visceral perforation will affect 18%.…”

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

“…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). PLE has been very rarely reported in the literature as a presenting symptom of RS (8,9).…”

Revisión sistemática del Síndrome de Rapunzel

“…En una revisión realizada en 2007 por Naik y cols., se evaluaron 28 casos del síndrome de Rapunzel (27 mujeres y un hombre), con un rango de edad de entre cuatro y 22 años (15). Nour y cols, en otra revisión (2017), encontraron cuatro casos del síndrome de Rapunzel en pacientes masculinos (16). En este estudio se encontró que la presencia de tricobezoar fue mayor en el sexo femenino, reportándose solo cinco casos en el masculino (17)(18)(19)(20)(21).…”