Rapunzel syndrome is rare in children and delayed diagnosis and treatment may lead to various complications.The purpose of this paper was to explore the clinical characteristics and diagnostic and treatment countermeasures of Rapunzel syndrome in children.

From January 2019 to May 2021, retrospective review was conducted for clinical manifestations, treatment courses and outcomes of five hospitalized cases of children with Rapunzel syndrome.The search terms of "Rapunzel/Princess Rapunzel, trichobezoar" were employed for searching the databases of Wanfang, Wipu, CNKI and "Rapunzel syndrome, trichobezoar". The relevant literatures of PubMed and Embase databases were summarized after excluding literature reviews, repeated cases, adult cases and cases for which detailed information was non-available.

All five children were female, four with long hair and one with short hair and alopecia.Clinical manifestations included gastrointestinal obstruction such as vomiting, abdominal pain and abdominal masses, etc.All five cases were confirmed to have gastrointestinal foreign bodies by gastroscopy and/or CT pre-operation and the diagnosis of Rapunzel's syndrome was finally confirmed by a history of hair eating or plucking.Complications were electrolyte disturbance and multiple small bowel perforations (n=1), malnutrition and weight loss (n=1) and intestinal ischemic necrosis (n=1). The remainders had no significant complications or comorbidities.Hair masses were removed by small incisional dissection.The average operative duration was 2h and the average hospital stay 10 days.All of them recovered well and were discharged successfully.After discharge, consultations with mental health department continued.None of them had recurrence during the mean follow-up period of 1.7 years (3 months to 3 years). Through a literature retrieval, a total of 28 literature reports in Chinese and English were collected, with 52 cases with a male to female ratio of 3∶49 and 94.2% of females.The mean age was 11.73 years and there were 28 cases with a history of hair extraction (28/52, 53.8%). Fifty-two children were operated and laparotomy (39/52, 75%) predominated with gastroenterostomy for foreign body removal.The outcome was excellent in 51 of them, except for one child with preoperative gastric perforation who died postoperatively due to severe malnutrition and persistent gastrointestinal bleeding.

Rapunzel syndrome in children often causes non-specific manifestations such as malnutrition and can lead to complications such as intestinal obstruction and occult bowel perforation, and delayed treatment may be life-threatening.CT and gastroscopy may provide an early diagnosis and surgery remains a major treatment.Long-term psychiatric follow-ups and psychotherapy prevents a recurrence.

Intestinal Obstruction; Bezoars; Gastroenterostomy; Intestinal Perforation; Surgical Procedures, Operative; Child