Rapunzel syndrome is the term for a trichobezoar (gastric 'hair ball') which has a tail-like extension into the small bowel through the pylorus causing gastric outlet obstruction.
Human hair (especially long hair) is resistant to digestion as well as peristalsis. So it tends to stay in the stomach and over a period of time may form a large ‘hair ball’. A trichobezoar may extend up to the pylorus, duodenum, or even jejunum. A part may break off into small bowel and cause small bowel obstruction.
For discussion of other gastrointestinal foreign bodies, please see: bezoars
The patient usually is an adolescent girl. The patient may have history of a psychiatric illness and occasionally a prior history of surgery may be present.
The symptoms of Rapunzel syndrome is caused either by gastric outlet obstruction or its complications and includes:
- anorexia, bloating, early satiety
- weight loss
- vomiting immediately following meals
- acute epigastric pain
- patchy hair loss seen in scalp hair
- obstructive jaundice
- mechanical small bowel obstruction
- small bowel perforation
- acute pancreatitis
- distended stomach shadow with an intragastric mottled gas pattern, outlined by fundal gas, which may resemble a food-filled stomach
- free gas shadow under diaphragm may be seen on erect radiograph if bowel perforation is present
Fluoroscopy: barium studies
- may show an intraluminal filling defect with mottled gas pattern without attachment to bowel wall
- over time the interstices of trichobezoar are filled with barium. This barium may remain for a considerable period of time and can be seen in delayed radiographs when the barium has exited the stomach and duodenum
- may be seen as an echogenic mass with intense acoustic shadow seen within stomach and pylorus region
- complex intraperitoneal free fluid if complicated by bowel perforation
- CT is the best imaging modality for showing the size and configuration of the trichobezoar and most accurately identifying its location
- may show an intragastric well-circumscribed inhomogenous mass consisting of ‘mottled gas pattern’ or ‘compressed concentric rings’ pattern due to the presence of entrapped air and food debris
- body of the mass in stomach while tail may extend to the duodenum or jejunum
- normal stomach wall can be traced completely separate from the lesion
- no contrast enhancement
- mucosal edema and wall thickening may be seen in duodenum and jejunum
- intraperitoneal fluid with free gas can be seen if perforation is present
Treatment and prognosis
Medical management is restricted to correction of anaemia and weakness. The treatment is essentially surgical. Laporotomy with extraction of bezoar is done with exploration of rest of the small bowel to look for detached bezoars. Small bowel segments showing extensive ulcerations and gangrene are resected.
Psychiatric evaluation is suggested for underlying illness.
Although the imaging features allow confident diagnosis of trichobezoars, depending on the investigative modalities, the differentials to be considered are:
- gastrointestinal tumour such as a GIST extending into the stomach lumen
- other type of bezoar (e.g. phytobezoar)
- 1. Ripollés T, García-Aguayo J, Martínez MJ et-al. Gastrointestinal bezoars: sonographic and CT characteristics. AJR Am J Roentgenol. 2001;177 (1): 65-9. doi:10.2214/ajr.177.1.1770065 - Pubmed citation
- 2. Gorter RR, Kneepkens CM, Mattens EC et-al. Management of trichobezoar: case report and literature review. Pediatr. Surg. Int. 2010;26 (5): 457-63. doi:10.1007/s00383-010-2570-0 - Free text at pubmed - Pubmed citation