Rapunzel syndrome: radiographic and ultrasonographic findings

Clinical History

The patient presented to the emergency department with upper abdominal pain and a feeling of discomfort after meals. Medical history included psychological problems and frequent ingestion of foreign bodies (sometimes metallic).

Imaging Findings

The patient initially underwent an abdominal X-ray in a standing position, which identified metallic foreign bodies along with air bubbles in the periumbilical area. There was also a metallic body in the lesser pelvis and limited fluid-fluid levels. (Fig. 1) Barium fluoroscopy identified a filling defect inside the stomach which was filled with barium. Later fluoroscopic images revealed the presence of small filling defects inside the duodenum. (Fig. 2)
Abdominal ultrasonography after the filling of the stomach with water identified a mass of mixed echogenicity inside the stomach. Motility of the mass was evident in different examination positions of the patient. (Fig. 3)

Discussion

The term “Rapunzel syndrome” is used to describe a rare complication of trichobezoar formation. Namely, it refers to a large trichobezoar completely filling the stomach and extending to the duodenum and possibly the jejunum or even colon through the pylorus. Parts of this extension may occasionally break off and migrate to the small intestine causing obstruction. The term was coined in 1968 by Vaughan et al. [1, 2, 3] This condition almost exclusively affects young female patients with psychiatric disorders like trichotillomania or trichophagia. Trichobezoars form because of the accumulation of undigested human hair along with mucus and food. Although asymptomatic in its origin, a trichobezoar may cause complications like mucosal erosion, ulceration or perforation and intussusception once it increases in size. [2] Adult patients with prior gastric surgery are also at risk of suffering from a bezoar. [4]
Plain radiographs usually provide signs of intestinal obstruction but can rarely identify the mottled air pattern of a bezoar. [4] Barium fluoroscopic examinations are more useful in diagnosing trichobezoars which are not radio-opaque and present as smooth or lobulated filling defects in the barium-filled stomach or are characterized by the honeycomb appearance. [3] The bezoar’s free motility enables its differentiation from a neoplasm. Ultrasonography is useful in evaluating young patients with this condition. The large trichobezoar may be identified as a free-floating solid mass of heterogeneous echogenicity lying inside the stomach. [1] It is also reported that bezoars may appear on ultrasonography as masses with a hyperechoic arclike surface associated with marked acoustic shadow. [4] In a series published by Ripollés et al, CT was found to be the most accurate modality as it diagnosed the presence of a bezoar in all patients and correctly identified the presence of multiple bezoars more frequently than ultrasound. However, the latter was also reliable as it established the diagnosis in the majority of cases. [4] Bezoars appear on CT as rounded or ovoid intraluminal masses containing air bubbles. Small bezoars may be seen floating on the gastric content whereas large ones fill the entire stomach. In any case, bezoars should be differentiated from retained food in the stomach based on their rounded shape. [4] Finally, MRI can also identify trichobezoars as free-floating heterogeneous solid masses characterized by a mottled gas pattern, lying inside the stomach. [1]
Conventional laparotomy represents the treatment of choice for Rapunzel syndrome, although laparoscopy and endoscopy can also be used. Psychiatric consultation is essential to support the patients and prevent relapses. [2]

Differential Diagnosis List

Final Diagnosis

Surgically proven gastroduodenal bezoar

URL:	https://eurorad.org/case/12987
DOI:	10.1594/EURORAD/CASE.12987
ISSN:	1563-4086