Rapunzel syndrome is a rare form of trichobezoar that extends from the stomach into the small intestine. This article discusses a case of Rapunzel syndrome along with the diagnostic approach and surgical management. 

---

Rapunzel syndrome

Rapunzel syndrome is a rare complication of trichotillomania, an impulse-based psychiatric disorder characterised by the plucking of one’s own hair. The associated behaviour of trichophagia, the consumption of hair, leads to the formation of trichobezoars in the stomach.

Rapunzel syndrome is a rare complication of trichotillomania and trichophagia, and usually affects young women and adolescent girls. Psychiatric comorbidities may be present in patients exhibiting trichotillomania such as obsessive-compulsive disorder, anxiety disorders, depression and in some cases cognitive deficits. The definition of Rapunzel syndrome is essential in that the trichobezoar extends from the stomach into the intestine, but it can present with a variety of abdominal symptoms which can initially cause confusion when establishing a diagnosis.

The presence of abdominal guarding or signs of peritonitis is not often apparent unless there is gastric or intestinal perforation. Prodromal symptoms, which can be elicited during history-taking may include symptoms of other psychiatric disorders, and the evidence of trichotillomania and trichoscopy can be used to confirm the foundational diagnosis of trichotillomania in an acutely unwell patient.

---

Diagnostic approach

The diagnostic approach to Rapunzel syndrome begins with the same algorithm for approaching all acute abdomen cases. It is important to take a thorough history and perform a comprehensive physical examination of the abdomen. Special care should be taken to note the presence of guarding, rebound tenderness and symptoms of bowel perforation.

Imaging is an invaluable tool in the assessment of Rapunzel syndrome. Ultrasound should be performed, but it is not specific or adequate in diagnosing Rapunzel syndrome. It does, however, raise suspicion for foreign bodies present in the gastrointestinal system, and may help in raising the alarm to signs of tissue necrosis.

Trichobezoars are hypoechoic and may be curvilinear in shape, but this is not a specific ultrasound finding. The gold standard for imaging is always an Abdominal CT scan, with or without contrast. Contrast Abdominal CT scans can be performed if there is no suspicion of gastric or bowel perforation. Abdominal CT scans will often show a hyperlucent mass, with clear indications that the mass is obstructing part of the gastric outflow tract, if not completely blocking it.

---

Surgical management

1. There are two main surgical approaches to extracting trichobezoar in Rapunzel syndrome.
2. The first is endoscopic and is reserved for smaller trichobezoars and the second is an exploratory laparotomy.
3. It must be emphasised that laparotomy is often better because it allows the surgeon to explore the bowel along with the stomach and attempt repairs of the bowel if bowel necrosis and perforation are present.
4. Endoscopic approaches can help to fragment the trichobezoar but completely removing it may not be possible.
5. A snare for polypectomies can be used to fragment the trichobezoar, but an important limitation is the amount of argon used and the poor visibility resulting from the argon gas in the stomach.
6. Open laparotomy remains the most effective approach to extracting the trichobezoar.
7. It is always essential for the surgeon to examine the extent of the trichobezoar and examine whether the bowel is perforated.
8. If there is no bowel perforation or necrosis, then the trichobezoar can be extracted safely through the stomach and the stomach can be closed.
9. However, in the event of bowel perforation, it is essential that the surgeon repair the bowel immediately.

---

Case report

The case

A 24-year-old female patient was referred from the psychiatry department for epigastric pain, epigastric tenderness, intermittent emesis and fever. Her medical history was significant for Obsessive Compulsive Disorder(OCD) and Substance Use Disorder(SUD). At the time of the evaluation, she was placed in the psychiatry department for observation following complaints of increased unmanageable obsessive thoughts related to self-harm. She had complained of vague abdominal pain for a few months, but this pain had increased in the preceding two weeks.

Diagnosis

Upon physical examination, epigastric tenderness was established but there is no guarding, and no signs of peritonitis were observed. An ultrasound was performed which showed a diffusely hyperechoic mass in the stomach but was nonspecific. An abdominal CT was performed which showed a mass in the stomach, extending to the first part of the duodenum. The mass was described as a heterogenous mass with varying density.

Treatment

The patient then admitted to “eating bits of her hair” in times of crisis, a habit she had developed in her late adolescence. Unfortunately, this trichophagia was not discovered by the psychiatrists or the therapists at the treating hospital. Blood tests revealed no specific findings, except for a slightly raised WBC count of 12X109/L. Liver function tests were normal.

The patient underwent an exploratory laparotomy. Intra-operatively, the bowel was intact, with no signs of intestinal perforation. The stomach was incised, and it revealed a trichobezoar which extended into the first third of the curve of the duodenum. The excised trichobezoar was 10cm long, 10cm wide and around 5cm thick. After the excision, the stomach was closed. The patient had no postoperative complications and resumed in-patient recovery and rehabilitation at the department of psychiatry.

---

Conclusion

Trichobezoars are resistant and hard, making their destruction in vivo, almost impossible. It is often better to have an open approach to extracting the trichobezoar and to take measures to ensure that postoperative complications are reduced.

The follow-up management of Rapunzel syndrome is essential for patients. It is important that the underlying psychiatric causes of trichotillomania and trichophagia are addressed by psychiatrists and therapists. There are reported cases of recurring Rapunzel syndrome in patients, but these are rare.

Current therapeutic management of trichotillomania and trichophagia revolves around the use of Fluoxetine, among other drugs, but only fluoxetine combined with behaviour therapy and harm reduction therapy is the most effective, with other therapeutic measures showing little to no efficiency at reducing trichophagia.

Disclaimer- The views and opinions expressed in this article are those of the author and do not necessarily reflect the official policy or position of M3 India.

About the author of this article: This article has been written by Dr Sujay Shanker (General surgeon from Jamshedpur) and is co-authored by Dr Aparajeya Shanker