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MDLinxRapunzel Syndrome: A Rare Condition You Could Share with Your Cat

Exploring Rapunzel Syndrome: Gastrointestinal Implications and Psychiatric Correlations in Young Patients

Rapunzel syndrome, a rare gastrointestinal disorder characterized by the formation of a trichobezoar extending into the small intestine, predominantly affects young females with specific psychiatric comorbidities. This complex condition necessitates a multidisciplinary approach involving surgical and psychiatric interventions to manage and prevent recurrence effectively. Understanding the nuances of its diagnosis and treatment can aid physicians in early detection and comprehensive care.

Key Points:

  • Prevalence and Demographics: Rapunzel syndrome is extremely rare, with approximately 50 cases documented. It primarily affects young women, often those with underlying psychiatric disorders such as obsessive-compulsive disorder (OCD).
  • Definition and Pathophysiology: The syndrome is defined by the presence of a trichobezoar that not only forms in the stomach but also extends into the small intestine, causing potential blockages.
  • Psychiatric Link: Patients with this condition frequently exhibit behaviors associated with trichotillomania (hair pulling) and trichophagia (hair eating), highlighting a significant psychiatric component.
  • Diagnostic Techniques: Diagnosis is predominantly achieved through imaging techniques like CT and ultrasound, with upper GI endoscopy serving as the gold standard by visualizing and possibly removing hair masses.
  • Surgical and Psychiatric Treatment: Treatment generally involves the surgical removal of the hair mass and comprehensive psychiatric care, including medications like SSRIs and therapies such as cognitive-behavioral therapy (CBT).
  • Complications: Without timely intervention, the syndrome can lead to severe gastrointestinal complications, including gastric necrosis, perforation, and obstruction.
  • Preventive Strategies: Ongoing psychiatric evaluation and treatment are crucial in preventing recurrence, with a focus on managing the underlying compulsive behaviors.
  • Prognosis and Management: With dedicated psychiatric care and appropriate surgical intervention, patients have a favorable long-term prognosis.

Although discussed in the medical literature for over a century, trichotillomania was not officially included as a mental health disorder in the American psychiatric association’s diagnostic and statistical manual of mental disorders (DSM) until the DSM-III-R (1987) when it was classified as an impulse control disorder not elsewhere classified.

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