"Princess Disease" - Rapunzel Syndrome

This is the 3347th article of Da Yi Xiao Hu

Diseases caused by abdominal pain in girls

16-year-old Xixi (pseudonym) has suffered from repeated abdominal pain, bloating, nausea and vomiting in the past week. She went to the hospital for a check-up. A plain abdominal film showed intestinal gas. A gastroscopy revealed congestion and edema of the gastric mucosa in many places, and huge hair stones composed of hair and chyme in the stomach that gradually became thinner like horsetails. A colonoscopy also found a large number of hairs scattered in the intestinal cavity.

Eventually, doctors diagnosed Sissi with pica (a special habit that gradually develops in infants and children during the eating process), and that Sissi's gastric hair stones were a rare and special type of "Rapunzel syndrome."

Why Rapunzel Syndrome Got Its Name

Rapunzel syndrome is also known as tricholithiasis. Most patients have mental illness or pica and like to chew and swallow hair and silk, which causes high intestinal obstruction or pyloric obstruction. This syndrome is named after the protagonist of the same name in the Grimm fairy tale. We all know that in the fairy tale, Rapunzel, a long-haired girl, used her beautiful hair to help the prince climb up her tower. This disease is extremely rare. As early as 1812, foreign experts Jacob and Grimm first reported a case of a girl patient, and there have also been reports in my country.

The disease is caused by the fact that after long fibers such as hair threads are swallowed into the stomach, they cannot be digested by the body, but are entangled into a ball under the mechanical force of gastric peristalsis, forming a hair stone. As the gastric contents move into the small intestine, the tail end of the hair stone can enter the jejunum through the pylorus. Because the fiber-like substances are tightly entangled, the hair stone is difficult to move forward, and gradually increases in the stomach and upper jejunum, causing high intestinal obstruction or pyloric obstruction. The hair stone often compresses the mesenteric side of the small intestinal wall, causing necrosis or even perforation. Occasionally, the tail end of the hair stone will extend to the ileum or large intestine.

What are the symptoms of Rapunzel syndrome?

Most patients with this disease are female, especially adolescents, and they present with upper abdominal pain, bloating, nausea, and vomiting. Due to the valve function of the trichophyton, the amount of vomiting is generally not large, and the vomitus may contain bile, but if the obstruction is in the pylorus, it does not contain bile. A few may be accompanied by diarrhea and gastrointestinal bleeding. Physical examination often shows a mass in the upper abdomen. If perforation is caused by gastrointestinal wall necrosis, there may be symptoms and signs of diffuse peritonitis.

How is Rapunzel syndrome treated?

This syndrome requires surgical treatment. Since the stones are relatively long, it is generally difficult to remove them and multiple incisions are required. If intestinal necrosis occurs, intestinal resection should be performed.

This project is funded by Shanghai Science Education Development Foundation (Project No.: B202117)

Author: Baoshan Branch of Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine

Dr. Xia Lemin, MD, Department of Hematology