Gastric perforation secondary to Rapunzel syndrome

Abstract

Rapunzel syndrome is rare and describes a trichobezoar that extends through the pylorus into the jejunum, ileum or even the colon. Due to the large intraluminal size and weight they can attain, acute presentations of obstruction or perforation may occur. We report a case of a 17-year-old girl who presented to the emergency department following a syncopal episode. On examination, a left upper quadrant mass was appreciated with no signs of peritonism. Contrast-enhanced CT demonstrated a giant trichobezoar with resulting gastric perforation and intra-abdominal free fluid. Laparotomy and gastrotomy were performed and the patient had an uneventful recovery with psychiatric review prior to discharge. Though uncommon, bezoars should be included in our differential diagnosis as they can present in various ways owing to their size and weight. This case illustrates the risk of gastric perforation with large gastric bezoars.

Background

The true incidence of trichotillomania and symptomatic trichobezoars is unknown as they are generally seen in patients with a psychiatric diagnosis.

Though uncommon, the importance of inclusion of trichobezoars in the differential when faced with young women with a mental health diagnosis and an acute abdomen cannot be understated.

Multidisciplinary input with psychiatry and dietician review is necessary for best patient outcome.

We report this case of a large trichobezoar complicated by gastric perforation to highlight the importance of a sound history and clinical examination in the approach to the acute abdomen.

Case presentation

A 17-year-old girl presented to our institution after having two syncopal episodes which resulted in facial bruising and a scalp haematoma. Initial management was aimed to rule out intracranial injury and surgical input sought after she was noted to have a left upper quadrant (LUQ) mass and tender epigastrium.

On further questioning she gave a 5-month history of intermittent abdominal pain which had worsened over the preceding 2 weeks. Importantly, a history of trichotillomania and trichophagia ongoing for 3 years due to a background of anxiety was obtained. She noted weight loss over this period, however denied any change in appetite and her bowels opened regularly. She was known to the community paediatric team for social interaction difficulties but had no other significant medical, family or surgical history. On examination she appeared cachectic, there was epigastric and LUQ tenderness, and appreciation of a mass arising from the LUQ extending towards the umbilicus. No signs of peritonism were demonstrated. On examination of the chest, there was decreased air entry to the left lower zone.

Observations revealed a pulse of 137 beats/min, blood pressure of 112/66 mm Hg and a temperature of 36.1°C. Laboratory investigations showed a haemoglobin 100 g/L, white cell count 35×10⁹/L, platelets 876, C reactive protein 306 and urea and electrolytes within normal limits.

Investigations

Contrast-enhanced CT showed a grossly distended stomach with a large volume intraluminal gastric mass (figure 1). There was pneumatosis of much of the gastric mucosa, anterior perforation of the stomach wall with resulting pneumoperitoneum and stomach contents in the superior abdominal cavity (figure 2).

Figure 1.

Coronal contrast-enhanced CT image showing large bezoar and pneumoperitoneum.

Figure 2.

Sagittal contrast-enhanced CT image showing large bezoar and pneumoperitoneum.

Treatment

The patient was resuscitated and intravenous antibiotics for abdominal sepsis commenced. Emergency laparotomy performed, a 2 cm anterior gastric perforation with multiple adhesions between the omentum and anterior abdominal wall was noted. There were multiple pockets of pus, fibrin and collections between the very distended stomach, spleen, diaphragm and left lobe of the liver. Gastrotomy extending from the gastric perforation was undertaken and the large trichobezoar which formed a cast of the entire stomach and duodenum was easily removed. The gastrotomy was closed with 3–0 PDS and covered with omentum, copious lavage of the abdomen was performed with 4 L warm saline and a large drain positioned in the left subphrenic region, a feeding jejunostomy was also established. Analysis of the surgical specimen confirmed an oval shaped mass of hair measuring 480×70×50 mm, consistent with a trichobezoar (figure 3).

Figure 3.

Trichobezoar specimen.

Postoperatively, the patient was admitted to the intensive care unit, reviewed by dietetics and diet commenced via feeding jejunostomy. After inpatient review by our psychiatry service and an uneventful postoperative course, she was discharged on postoperative day seven.

Outcome and follow-up

On follow-up at 30 days, there was no sign of postoperative complications and the patient was progressing well with dietary advice and engaging with outpatient psychology reviews.

Discussion

Bezoars are foreign and intrinsic material found in the gastrointestinal tract formed from the accumulation of indigestible material. Trichobezoars are hair balls that are mainly confined to the stomach with a higher incidence in women with a background of trichotillomania and trichophagia.^{1 2} Due to hair being resistant to digestive enzymes and ability to be unaffected by gastric peristalsis owing to its slippery nature, the fibres become trapped within the gastric folds and stick to each other, if enough is ingested a ‘hair ball’ or bezoar is formed. Vaughan et al described a rare manifestation of trichobezoars in 1968 in which the bezoar extended beyond the pylorus into the small or large bowel.³

Trichobezoar is uncommon, affecting predominantly women with a mental health diagnosis,.² Incidence is quoted as 0.4%–1% of the general population; however, given that trichobezoars are generally seen in persons with psychiatric disorders, it is thought that the true incidence is unknown.^4–6 Typical symptoms include halitosis, epigastric discomfort and pain, nausea, vomiting, early satiety and weight loss.^{7 8} Complications of trichobezoars include intestinal obstruction, bleeding, perforation, intussusception, pancreatitis and cholangitis.⁷ Several approaches to treatment have been described in the literature including endoscopy, laparoscopy and conventional laparotomy. While endoscopy aids in the diagnosis and differentiation of different intragastric bezoars, there is no role for an endoscopic approach to treatment of large trichobezoars due to the size, density and hardness of the material encountered.^{4 9} Conventional laparotomy has proven to be superior to the laparoscopic approach for the removal of large trichobezoars especially when the patient presents with a complication due to this,.⁷

Learning points.

• Though uncommon, trichobezoars should be part of the differential in young women presenting with non-specific abdominal problems.

• The emergency general surgeon should be prepared to deal with complications as a result of trichobezoars.

• Importantly acute and long-term psychiatric and psychological support is the main treatment option to prevent recurrence.