Rapunzel syndrome: the unsuspected culprit

Abstract

Trichobezoar is a rare intriguing disorder in which swallowed hairs accumulates in the stomach. Being indigestible and slippery, it could not be propulsed and becomes entrapped within the stomach. Large amounts can thus accumulate over the years forming a hair ball. Rapunzel syndrome is a variant where hair accumulation reaches the small gut and beyond in some cases. Although the syndrome has been known for many years, only 24 cases have been reported in the literature and the discovery of a new case is always surprising. In this report, we present two cases discovered within a period of three months. One of them was pregnant and had small bowel intussusception and perforation, a very rare combination. We hereby add two more cases to the literature. To our knowledge, this is the first report on two cases of Rapunzel syndrome, the diagnosis of which demands a high index of suspicion.

Figure 1

A hair ball occupying the whole stomach (A) and extending down into the jejunum (B).

Figure 2

Full extent of the hair ball extending into the jejunum.

Figure 3

Ulcer in the body of stomach (arrow) caused by hair ball.

Figure 4

Two perforations at the mesenteric border of the proximal jejunum (arrows).