Rapunzel syndrome in children: a retrospective review of ten cases combined with literature review in a tertiary referral center.
Rapunzel syndrome
Trichobezoar
Trichotillomania
Journal
Pediatric surgery international
ISSN: 1437-9813
Titre abrégé: Pediatr Surg Int
Pays: Germany
ID NLM: 8609169
Informations de publication
Date de publication:
04 May 2024
04 May 2024
Historique:
accepted:
21
04
2024
medline:
5
5
2024
pubmed:
5
5
2024
entrez:
4
5
2024
Statut:
epublish
Résumé
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. 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. 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. 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.
Identifiants
pubmed: 38703237
doi: 10.1007/s00383-024-05705-0
pii: 10.1007/s00383-024-05705-0
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
121Informations de copyright
© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
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