Rapunzel Syndrome: A Rare Case of Small Bowel Intussusception in a Child

Abstract

Trichobezoars are indigestible masses of ingested hair commonly found in the stomach, often presenting with symptoms related to gastric outlet obstruction and severity related to the mass's size and location. Gastrointestinal complications include ulceration, perforation, peritonitis, pancreatitis, obstructive jaundice, pneumatosis intestinalis, and intussusception. Management of trichobezoars differs from that of other forms of bezoars, which can often be addressed with chemical dissolution. Trichobezoars are highdensity structures that are also resistant to enzymatic and pharmacotherapy degradation, and as such, they require endoscopic, or more commonly, surgical removal. Here, we present the diagnosis and surgical management of a 12-year-old female with a large trichobezoar causing gastric outlet obstruction, with an associated Rapunzel syndrome manifesting as multiple small intestinal intussusceptions.

Sepsis secondary to Rapunzel syndrome

“…Rapunzel syndrome is an uncommon cause of trichobezoar, characterized by trichotillomania and trichophagia, subsequently resulting in gastrointestinal trichobezoar. 1 Herein, we report a case of Rapunzel syndrome in a 4-yearold girl with concomitant intussusception and septic shock.…”

“…3 The criteria to identify Rapunzel syndrome includes a trichobezoar with tail, which extends at least to the jejunum causing intestinal obstruction. 1 The diagnosis of trichobezoars is quite challenging due to non-specific abdominal symptoms such as anorexia, weight loss, vomiting, abdominal pain and gastrointestinal bleeding. 3,4 Plain film and ultrasound can detect 20-60% of bezoar cases, while abdominal computerised tomography scans are effective in 97% of cases.…”

“…However, upper gastrointestinal endoscopy remains the gold standard for diagnosing trichobezoars. 1 The complications related to gastrointestinal bezoars include ulceration, perforation, peritonitis, pancreatitis, obstructive jaundice, pneumatosis intestinalis and intussusception. 1 Our Management of trichobezoars includes upper endoscopy, laparotomy, open surgical operation and psychiatric treatments.…”

“…1 The complications related to gastrointestinal bezoars include ulceration, perforation, peritonitis, pancreatitis, obstructive jaundice, pneumatosis intestinalis and intussusception. 1 Our Management of trichobezoars includes upper endoscopy, laparotomy, open surgical operation and psychiatric treatments. 1,4 Diagnosing and managing trichotillomania is an integral part of its treatment.…”

“…1 Our Management of trichobezoars includes upper endoscopy, laparotomy, open surgical operation and psychiatric treatments. 1,4 Diagnosing and managing trichotillomania is an integral part of its treatment. A multidisciplinary team including a dermatologist, pediatrician and/or psychiatrist along with patient education is necessary to prevent the aforementioned complications at the earliest stage.…”