A 10-year-old girl presented with 8 months of progressive weight loss, intermittent and sharp left upper quadrant abdominal pain, and increasing anxiety surrounding eating.
Physical examination showed a firm, irregular upper abdominal mass that was intermittently palpable. Laboratory testing showed iron deficiency anemia with a hemoglobin concentration of 113 g/L; inflammatory markers and nutritional screening results were within normal limits. An initial abdominal ultrasound examination was technically limited by overlying bowel gas and didn't identify a clear abnormality. Her weight declined from the 60th to the first centile on the UK World Health Organization growth chart during that period. Although she reported hunger, she frequently refused solid foods and relied predominantly on liquids.
Magnetic resonance enterography, performed to evaluate for inflammatory bowel disease, showed a 15 to 16 cm intragastric filling defect extending into the proximal duodenum, consistent with a trichobezoar. Surgical exploration confirmed a large trichobezoar occupying the stomach with extension through the pylorus. Following initial laparoscopic assessment, the mass was removed via laparotomy through a left upper transverse incision. The bezoar, composed of hair and vegetable fibers, was extracted in its entirety, and the gastrotomy was closed without complication.
The condition was later confirmed to be Rapunzel syndrome, a rare form of trichobezoar characterized by extension of a gastric hair mass beyond the pylorus into the small intestine. Rapunzel syndrome may result in obstruction or perforation if untreated. It is most often described in adolescents with underlying psychiatric disease. In this case, the patient had attention-deficit/hyperactivity disorder and sensory aversion to food textures, factors that contributed to diagnostic delay.
Postoperatively, oral feeding was gradually reintroduced under dietetic supervision, and behavioral therapy was initiated to address trichotillomania and trichophagia. At 3-month follow-up, her weight improved to the 24th centile, abdominal symptoms resolved, and there was no evidence of recurrence.
“Rapunzel syndrome should be considered in pediatric patients presenting with unexplained weight loss and abdominal symptoms, even in the absence of classic psychiatric features,” noted lead study author Richard M. Lurshay, MD, MBBS, of Paediatrics and Child Health at South West Acute Hospital in Ireland, and colleagues.
The researchers reported no conflicts of interest.
Source: Cureus
