The Rapunzel Syndrome

Bet you’ve never heard of this. Neither had I. The Rapunzel Syndrome is an extremely rare condition with less than two dozen cases being reported. It is part of the larger group of conditions we call pica syndromes.

Pica is the repetitive eating of non-food items. Some of these are cultural (clay eating) while others are associated with various psychiatric disorders such as schizophrenia and severe Obsessive-Compulsive Disorder (OCD). These people consume things such as paper, paint chips, dirt, chalk, coins, rubber bands, paper clips and other metallic objects, hair, clay, almost anything you can think of. Clay eating is a type of geophagia–geo means earth and phagia means eating. It is not a rare occurrence, particularly in the south, since it is thought to be healthy to do so. It can lead to constipation and even bowel rupture, but most commonly causes anemia since the clay inhibits the absorption of iron from the GI tract. Paint chips were a common cause of lead poisoning in children until laws changed, requiring indoor paint to be lead free. Still some older buildings have leaded paint, maybe painted over a few times but still there, so that when the paint peels the leaded layer can be exposed and consumed.

Wikipedia Pica Article

Some of these ingested materials can form a ball known as a bezoar, the same thing your cat coughs up. This can be anything from a hard ball of clay to a tightly wound wad of hair. It can cause intestinal obstruction and intestinal erosions with bleeding and perforation. Both of these can be life-threatening and often require surgical treatment.

When hair is eaten, known as trichophagia (tricho means hair; phagia means to eat), it can form a hair ball (trichobezoar) in the stomach. This can cause the obstruction and erosions mentioned above, usually near the bottom of the stomach in the area known as the gastric outlet–where the stomach empties into the small bowel. Rarely the bezoar will form a long tail that extends downstream into the small bowel. This long tail of hair is reminiscent of Rapunzel’s hair in the Grimm Brothers famous fairy tale.

This tail can cause obstruction or erosion of the small bowel and can lead to death if not treated surgically in a timely manner. A recent article published Forensic Science International reports on the death of a child from this syndrome. Just under four years old, she appears to be the victim of neglectful abuse and is considered the youngest victim of this rare syndrome ever reported.

Medicine is filled with bizarre syndromes and this is definitely one of them.

Wikipedia Rapunzel Syndrome Article