Rapunzel syndrome in children: a retrospective review of ten cases combined with literature review in a tertiary referral center
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In brief
In brief
This retrospective study of 10 pediatric Rapunzel syndrome cases (median age 9.1 years, all female) found that 60% presented with serious complications including bowel obstruction and perforation. Ultrasound showed high diagnostic accuracy, but endoscopic removal failed in all attempts, making laparotomy the preferred treatment approach with excellent outcomes and no recurrence.
- Rapunzel syndrome predominantly affects female children (median age 9.1 years) and presents with abdominal mass, pain, and vomiting.
- Complications occur in 60% of cases, including bowel obstruction, perforation, and pancreatitis requiring prompt surgical intervention.
- Ultrasound has high diagnostic accuracy when combined with clinical history; endoscopic removal has low success rates in pediatric cases.
- Laparotomy with gastric incision is the safe, effective treatment of choice given high complication rates and endoscopic failure.
- All patients in this series recovered well post-surgery with no recurrence, supporting early surgical management over conservative approaches.
Written by the GCMD Library team from the article.
Abstract
Purpose
Rapunzel syndrome is an uncommon condition in children, and its clinical features remain unclear. This study presents the largest single-center series of pediatric cases to date, with the objective of documenting the clinical characteristics and treatment approaches for children with Rapunzel syndrome.
Methods
A retrospective study was conducted in children with Rapunzel syndrome from 2019 to 2023. We recorded age, gender, symptoms, locations of bezoar, complications, and treatment options.
Results
Ten patients with Rapunzel syndrome were included. The median age was 9.1 years, with all of whom were female. The most common clinical symptoms were upper abdominal mass (90%), abdominal pain (80%), and nausea and vomiting (50%). Complications occurred in six cases (60%), including small bowel obstruction (20%), severe gastric dilatation (10%), intestinal perforation (10%), choledochodilation (10%), acute pancreatitis with cholecystitis (10%). Preoperative ultrasonography suggested low-echoic foreign bodies continuing to the jejunum or ileocecal region in five cases (50%). Preoperative gastroscopy attempted in four cases (40%) to remove the foreign bodies, all of which failed. All patients underwent surgical treatment, with nine cases undergoing gastric incision foreign body removal, and one case undergoing gastric incision foreign body removal combined with intestinal perforation repair. All patients recovered well. No recurrence was observed during follow-up.
Conclusion
The accuracy of ultrasound diagnosis in identifying Rapunzel syndrome is high; however, it may lead to misdiagnosis if not complemented with the patient’s medical history. Endoscopic presents a heightened treatment risk and a reduced success rate. The condition commonly presents with severe complications, thus making laparotomy a safe and effective option for intervention.