Rapunzel syndrome presenting as jejuno-jejunal intussusception.

Abstract

The term Rapunzel syndrome is used to describe a trichobezoar of the stomach with a long tail of hair extending into the duodenum and small intestine. It is a rare clinical entity, and it is even rarer in these cases that jejuno-jejunal intussusception and resulting intestinal obstruction is a presenting feature. We report one such case, a young female who presented in the emergency department with abdominal pain and bilious vomiting of short duration. Contrast-enhanced CT of the abdomen enabled a diagnosis of a trichobezoar in the stomach extending into the small gut, along with jejuno-jejunal intussusception. On exploration, gastrotomy was performed to remove the gastric trichobezoar, and jejuno-jejunal intussusception was found on three segments in the proximal jejunum, which was resolved upon complete removal of the tail of the bezoar. This case report is unusual, since intussusception is a rare occurrence in Rapunzel syndrome, and this is the first time that it has been diagnosed preoperatively.