Rapunzel Syndrome: A Concealed Tale of the Misleading Tail

Area of Science:

• Gastroenterology and Hepatology
• Surgical Case Reports

Background:

• Rapunzel syndrome, characterized by a gastric 'tail' of trichobezoars extending into the small intestine, poses diagnostic challenges.
• Subtle clinical manifestations such as anorexia, constipation, anemia, hypoalbuminemia, and hypoproteinemia can be easily overlooked, delaying diagnosis and treatment.
• Early recognition is crucial for preventing severe complications associated with gastric trichobezoars.

Observation:

• A patient with Rapunzel syndrome initially presented with non-specific symptoms, leading to delayed diagnosis.
• The extensive trichobezoar ('Rapunzel tail') caused multiple episodes of intussusception within the gastrointestinal tract.
• An unusual complication of a sealed perforation, managed non-operatively due to being contained by intussusceptions, was observed.

Findings:

• The diagnosis of Rapunzel syndrome was challenging due to initially overlooked subtle clinical features.
• The Rapunzel tail was identified as the cause of multiple intussusceptions and a sealed perforation.
• A sealed perforation, contained by intussusceptions in a clinically stable patient, represents a novel finding in Rapunzel syndrome.

Implications:

• This case underscores the importance of considering Rapunzel syndrome even with vague gastrointestinal symptoms.
• The occurrence of a sealed perforation by intussusceptions highlights a previously undocumented complication and potential conservative management strategy.
• Increased awareness and diagnostic vigilance for Rapunzel syndrome are necessary to improve patient outcomes and prevent life-threatening events.