A bezoar is a foreign body composed of non-digestible material located in any portion of the digestive tract, being more common its location in the stomach [1]. The word bezoar derives from the Persian Pâdzahr which means antidote [2].

Trichobezoar is the most common type of bezoar. There are five types of bezoars according to their composition; trichobezoar: composed of human hair; phytobezoar: composed of fruit and vegetable fibers such as non-digestible cellulose; lactobezoar: composed of poorly diluted milk; pharmacobezoar: composed by ingestion of medicines and finally foreign bodies or substances different from the previous ones such as wood chips (linnobezoars), resins (resinobezoar), starch (almilobezoar), among others [3].

Unlike the rest of the bezoars, trichobezoars are common in young women with psychiatric disorders such as trichotillomania or trichophagia. Rapunzel syndrome is a rare and more severe form of gastric trichobezoar that has an extension into the small intestine [4]. Rapunzel syndrome can be an uncommon cause of chronic abdominal pain with rare complications such as intestinal obstruction and perforation.

We present the clinical case and subsequent management of Rapunzel syndrome in a patient with no documented history of trichotillomania or trichophagia who debuted with gastric perforation.

A 27-year-old female patient, with a surgical history of two cesarean sections, presented to the emergency department of a tertiary level hospital in the metropolitan area of Monterrey, Nuevo Leon, Mexico; with a 2-year history of abdominal pain, nausea, intermittent vomiting, and early satiety. She denied other accompanying features such as changes in bowel habit, hematemesis, or melena and had no urinary tract or gynecologic symptoms.

On physical examination she had a heart rate of 120 beats per minute with a blood pressure of 90/60 mmHg, an axillary temperature of 37.2 °C, distended abdomen, auscultation showed the absence of peristaltic sounds, acute peritoneal signs such as abdominal rigidity, and generalized pain of intensity 9/10 evaluated with visual analog scale, the rest of the examination was without relevant data. The paraclinical examinations showed white blood cells of 32,000/mm, arterial gasometry showed an acidosis of 7.31 pH, metabolic type. Abdominal and chest X-rays were requested, which showed a J-shaped stomach, and intragastric mass as well as subdiaphragmatic free air (Figure 1). It was decided to admit her to the operating room by the General Surgery service, performing a laparotomy revealing a gastric perforation in the lesser curvature at the level of the body on the anterior gastric face (Figure 2) with the presence of a foreign body inside the stomach lumen compatible with trichobezoar, performing a wide gastrotomy extracting trichobezoar of 25 × 20 cm (Figure 3), which occupied the entire gastric chamber presenting extension to the second portion of the duodenum.

During her postoperative recovery, the patient evolved favorably, being discharged on the fifth postoperative day with diagnoses of trichotillomania, gastric perforation by gastroduodenal trichobezoar, generalized anxiety disorder, and depression, being in control and follow-up by the Psychiatry Service.

The oldest reports of this condition date back to the 12th century BC in India; however, the first officially documented trichobezoar was reported by Bardeman in 1779 as a mass of hair found in the autopsy of a patient who died of gastric perforation and peritonitis [5].

The presence of a trichobezoar is rare, most cases being in young women under 30 years of age with underlying psychiatric disease, such as trichotillomania, although it is not uncommon for them to deny an underlying pathology.

Human hair is resistant to digestion and intestinal peristalsis, so continuous and prolonged ingestion causes its impaction in the lumina. Other factors associated with the formation of bezoars include altered gastric motility, decreased acidity, bacterial colonies, lack of surface area for propulsion, and entrapment of the fibers in the folds of the gastric mucosa [6].

Trichobezoar mainly affects the stomach; however, on rare occasions it goes beyond the pylorus and manages to extend to the duodenum and jejunum, being called Rapunzel syndrome, named after the eponymous heroine of a German fairy tale written by the Brothers Grimm, first described by Vaughan et al. in 1968 [7].

The clinical picture in these patients usually presents until the trichobezoar grows considerably to obstruction, the most common symptoms include a palpable abdominal lump in the epigastrium, chronic abdominal pain, nausea, vomiting, constipation, alopecia areata, and even in some patients halitosis, weight loss, anorexia, hematemesis, and iron deficiency anemia can be found. Complications, although rare, include ulcer formation, perforation, peritonitis, intussusception, malnutrition, jaundice, and pancreatitis.

Radiological diagnosis can be made with simple abdominal radiography, gastrointestinal series with contrast, and abdominal computed tomography in which the trichobezoar can be evidenced as a filling defect in the stomach and small intestine, being possible to delimit its extension.

Upper endoscopy plays an important role in the diagnosis of trichobezoars and Rapunzel syndrome since it allows direct visualization, as well as a possible therapeutic intervention; however, it is rarely a definitive treatment due to the large size that gastric trichobezoars can have [8].

Surgical removal remains the cornerstone in the treatment of trichobezoars and Rapunzel syndrome, it is performed by gastrotomy or enterotomy as in the present case; however, recent studies describe the successful laparoscopic removal of trichobezoars in pediatric patients.

Trichobezoar is infrequent and Rapunzel syndrome is a very rare condition, yet it should be considered as a differential diagnosis in young women with psychiatric history who report palpable abdominal tumor, abdominal pain, nausea, vomiting, early satiety, or other gastrointestinal manifestations. Early diagnosis avoids complications that can become serious. Intentional questioning of a patient's history of trichotillomania or trichophagia is essential for diagnosis. Multidisciplinary management and the long-term psychiatric follow-up are important parts of treatment and prevention of recurrence.