Gastric trichobezoar and Rapunzel syndrome: case report of a very rare condition

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CASE REPORT

Hippokratia 2023, 27(1): 25-27

Kouskos E1, Polychroni D2, Rizou M1, Andronikou A1, Petrellis V3
1Surgical Department, General Hospital of Mytilene, Lesvos Island, Greece
2Department of Obstetrics and Gynecology, General Hospital of Mytilene, Lesvos Island, Greece
3Department of Radiology, General Hospital of Mytilene, Lesvos Island, Greece

Abstract

Background: Rapunzel syndrome is a rare trichobezoar variant extending from the gastric cavity into the small bowel.

Case description: We report the case of a 22-year-old woman who presented with epigastric pain, nausea, and loss of appetite within the preceding five weeks. She had a palpable mass in the epigastric area with mild localized tenderness. Her abdominal computed tomography scan showed a distended stomach and duodenum, with a heterogeneous solid material, suspicious for a bezoar. Upper gastrointestinal endoscopy revealed a large, densely packed trichobezoar occupying the gastric cavity and extending through the pylorus. Endoscopic removal of the bezoar was unsuccessful. The patient underwent a 6cm-long gastrotomy, and the 150cm-long bezoar, extending from the stomach to the jejunum, was uneventfully removed. The patient was referred postoperatively to a dietitian and psychiatrist for management of her trichotillomania and trichophagia.

Conclusion: Trichobezoars are commonly found in young females with a history of trichotillomania and trichophagia and are associated with psychiatric disorders. HIPPOKRATIA 2023, 27 (1):25-27.

Keywords: Trichobezoar, Rapunzel syndrome, trichotillomania, trichophagia

Corresponding author: Kouskos Efstratios, Surgical Department, General Hospital of Mytilene, Lesvos Island, Greece, e-mail: skouskos@hotmail.com 

Introduction

Bezoars are concretions of undigested material found in the stomach. They are differentiated depending on the type of retained foreign bodies1. This material may include plant fibers (phytobezoar), hair (trichobezoar), medications (pharmacobezoar), paper, milk protein, etc1,2. Trichobezoars are formed by ingesting and accumulating hair- or hair-like fibers in the gastrointestinal tract, especially in the stomach1,3.

Rapunzel syndrome is a rare form of trichobezoar. This entity occurs as gastric bezoars get bigger, beyond the small bowel, producing long, tail-like hair extensions1.

Bezoars usually cause no specific symptoms and, as a result, may imitate other diseases such as tumors1. By the time they reach a large size, they can be related to severe gastrointestinal complications such as perforation, peritonitis, pancreatitis, constipation, appendicitis, jaundice, steatorrhea, protein-losing enteropathy, and intussusception4.

Trichotillomania is a psychiatric condition characterized by compulsive behaviors like self-induced and repetitive hair-pulling, in combination with obsessive thoughts, mood disorders, and anxiety1. Almost half of trichotillomania cases have skin-picking disorder as a comorbid condition5. It affects up to 4 % of the population, mostly children and adolescents2.

In this article, we report a rare case of Rapunzel syndrome in a young female patient with a very long trichobezoar that was successfully treated surgically. 

Case presentation

A 22-year-old woman with clinical signs of malnutrition presented to the emergency department of our institution with dull abdominal pain and distention in the epigastric area. She also complained of nausea, occasional vomiting, and loss of appetite for at least the preceding five weeks. She reported normal bowel movements and denied fever or diarrhea. Her past medical history was unremarkable, and her vital signs were stable.

Physical examination revealed a soft abdomen and a well-defined, relatively mobile, large epigastric mass extending from the left upper quarter to the umbilicus. Bowel sounds were audible. There were no other signs indicating peritonitis. There was no blood on digital rectal examination.

Laboratory results revealed a reduced hemoglobin level of 9.8 g/dl and an elevated white cell count with 82.3 % neutrophils. C-reactive protein was elevated at 12.9 mg/l. Urea, electrolytes, liver function tests, and coagulation profile were within normal range. The abdominal computed tomography (CT) scan demonstrated a distended stomach and duodenum with a large, well-circumscribed mass with internal air loculi occupying almost the entire stomach and extending into the duodenum (Figure 1). Neither gastric obstruction nor perforation was evident. Images were highly suggestive of gastric bezoar. Given the characteristic radiological findings, the patient underwent upper gastrointestinal endoscopy under conscious sedation. The procedure confirmed the diagnosis, revealing Rapunzel syndrome. The mass was found under the esophagogastric junction extending through the pylorus to the duodenum (Figure 2). The hairball, covered by mucus, had a glistening shiny surface. Due to the large size, repeated attempts of endoscopic removal of the tangled bezoar were unsuccessful.

Figure 1: Axial computed tomography image demonstrating a large heterogenous intraluminal mass obstructing the entire lumen of the stomach.

Figure 2: Endoscopic images during the upper gastrointestinal endoscopy, which confirmed gastric trichobezoar extending from the esophagogastric junction to the duodenum.

Based on imaging, we decided to proceed with open laparotomy. Through a limited midline supraumbilical incision, we performed a 6cm-long anterior gastrotomy. A huge gastric trichobezoar and its tapering tail were completely removed without complications (Figure 3, Figure 4). The proximal part of the bezoar was found molded into the shape of the stomach and duodenum, while its total length was more than 150 cm. The postoperative course of the patient was uneventful. She was discharged in satisfactory condition on the fourth postoperative day and referred for outpatient psychiatric and dietitian consultation. Psychiatric evaluation diagnosed her with anxiety, trichotillomania, and trichophagia, with onset dating back to her final high school year. She was assigned to a psychiatric follow-up and support to avoid recurrences.

Figure 3: Intraoperative view showing trichobezoar removal through limited gastrotomy.

Figure 4: A stomach-shaped trichobezoar of about 150 cm in length extending to the small bowel.

Discussion

Rapunzel syndrome is a rare form of an occluding trichobezoar, where the gastric bezoar with a tail extends to the duodenum, ileum, or even the colon3,6,7. The large hair quantity assumes the stomach shape as a single mass4. Underlying mental disorders that are usually associated with the syndrome are anxiety and post-traumatic stress, leading to trichophagia and trichotillomania8. The syndrome was first reported by Vaughan ED in 19689. The term originates from Grimm Brothers’ fairy tale of a very young princess locked in an inaccessible tower and a young prince, trying to save her, climbs up her long tresses4.

The accurate incidence of trichobezoars is unknown3. It is much more common in female patients between the ages of 10 and 19 years old, attributed to the traditional long hair3,4. Young women with emotional disorders, behavioral disturbances, and mental retardation are prone to continuous habitual ingestion of hair in the gastrointestinal tract2,10,11.

The formation of a bezoar is extremely rare, and it occurs in only 1 % of patients suffering from trichophagia. Trichobezoar is related to the indigestible composition of hair, which becomes entangled in the gastric folds. Hairballs that are resistant to digestive enzymes and escape the peristaltic propulsion continue accumulating into bigger mass size9,12. Gastric acid denatures and oxidizes the hair, and gastric mucus covers the mass8. As confirmed from our case report, the bezoar has a shiny, dark look.

Differential diagnosis may prove difficult due to nonspecific symptoms and the rarity of the syndrome. The personal history of trichotillomania and trichophagia and dietary habits could be helpful clues in the diagnostic process. Symptoms usually start developing as the hair mass sizes up8. Most patients are diagnosed with weight and appetite loss, halitosis, abdominal pain, abdominal mass, and gastrointestinal obstruction9,10. A well-defined, firm, mobile epigastric mass is revealed at physical examination in 85 % of patients3,6. Blood tests may demonstrate anemia, electrolyte disorders, malabsorption of iron, and hypoproteinemia3.

Early diagnosis is crucial since neglected obstructing or eroding bezoar may lead to complications such as bleeding, ulceration, and perforation8,13. Abdominal CT scan reveals a heterogenous intragastric lesion consisting of entrapped air bubbles, food debris, and concentric rings2,11. However, upper gastrointestinal endoscopy remains the diagnostic gold standard method3,8. It provides direct visualization of the bezoar and can be used for diagnostic and therapeutic purposes13. In our study, a CT scan showed an oval intraluminal mass in the distended stomach and duodenum, and endoscopy revealed the large size of the bezoar.

The treatment of the bezoar focuses on the complete removal of the hair mass. The treatment approach depends on the bezoar’s size, density, and localization3,8. Small trichobezoars can be managed by endoscopic removal, endoscopic fragmentation, gastric lavage, enzymatic therapy, or a combination of these methods8,14. Larger bezoars and Rapunzel syndrome usually require laparotomy with longitudinal gastrotomy, as performed in our case13. Although a minimally invasive approach with satisfactory postoperative outcomes has been reported by a few authors, the open conventional method is still the preferred method13.

A recurrence of the syndrome has been reported. The lack of postoperative care and/or the untreated underlying trichotillomania may result in the disorder’s relapse1. Since this entity is extremely rare, long-term psychiatric/psychological therapy and dietitian consultation seem to be prerequisites for the prevention of recurrence8.

Conflict of Interest

None declared by authors. 

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