Rapunzel syndrome in children: a retrospective review of ten cases combined with literature review in a tertiary referral center.

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.

Accidental ingestion of multiple magnetic beads by children and their impact on the gastrointestinal tract: a single-center study.

OBJECTIVE: In this study, we aimed to enhance the treatment protocols and help understand the harm caused by the accidental ingestion of magnetic beads by children. METHODS: Data were collected from 72 children with multiple gastrointestinal perforations or gastrointestinal obstructions. The 72 pediatric patients were divided into a perforation and a non-perforation group. The data collected for the analysis included the gender, age, medical history, place of residence (rural or urban), and symptoms along with the educational background of the caregiver, the location and quantity of any foreign bodies discovered during the procedure, whether perforation was confirmed during the procedure, and the number of times magnetic beads had been accidentally ingested. RESULTS: The accuracy rate of preoperative gastrointestinal perforation diagnosis via ultrasound was 71%, while that of the upright abdominal X-ray method was only 46%. In terms of symptoms, the risk of perforation was 13.844 and 12.703 times greater in pediatric patients who experienced vomiting and abdominal pain with vomiting and abdominal distension, respectively, compared to patients in an asymptomatic state. There were no statistical differences between the perforation and the non-perforation groups in terms of age, gender, medical history, and the number of magnetic beads ingested (P > 0.05); however, there were statistical differences in terms of white blood cell count (P = 0.048) and c-reactive protein levels (P = 0.033). A total of 56% of cases underwent a laparotomy along with perforation repair and 19% underwent gastroscopy along with laparotomy. All pediatric patients recovered without complications following surgery. CONCLUSION: Abdominal ultrasonography and/or upright abdominal X-ray analyses should be carried out as soon as possible in case of suspicion of accidental ingestion of magnetic beads by children. In most cases, immediate surgical intervention is required. Given the serious consequences of ingesting this type of foreign body, it is essential to inform parents and/or caregivers about the importance of preventing young children from using such products.

Adenomyoma of the small intestine in children: a 12-year experience in a tertiary referral center.

BACKGROUND: Adenomyoma of the small intestine is rare in children and the clinical characteristics is not clear. The study was to document the clinical characteristics and treatment of children with adenomyoma of the small intestine. METHODS: A retrospective study was conducted in children with intestinal adenomyoma from 2010 to 2022. We recorded age, gender, symptoms, location, tumour size and treatment options. RESULTS: Thirteen patients with adenomyoma of the small bowel were included. The median age was 20 months with a male-to-female ratio of 10:3 and more than half of the patients were younger than 2 years old. The mean tumour size was 2.0 cm. The lesion was found accidentally in one patient, and the others presented with symptoms of intussusception. A pathological lead point was found on ultrasound in seven patients. All tumours were located in the ileum, ranging from 24 to 260 cm proximal to the ileocecal valve. The tumour was found in an antimesenteric site in eight patients. Three patients suffered intestinal necrosis, and segmental resection of the ileum was performed. Three patients without intestinal necrosis underwent tumour rection, while intestinal resection and anastomosis were performed in the remaining seven. All patients recovered well except one, who developed intussusception 7 days after surgery; that patient underwent surgery and recovered uneventfully. CONCLUSIONS: Adenomyoma of the small intestine has a male predominance in children and intussusception is a common presentation. The ultrasound feature is a mass of mixed echogenicity containing several small cystic areas. Surgery is the primary treatment option and the procedure should be chosen based on intraoperative findings.

Clinical Characteristics of Pediatric Patients With Septic Shock Caused by Acute Appendicitis: A Case Series.

OBJECTIVES: This study aimed to explore the clinical characteristics of septic shock in pediatric patients caused by acute appendicitis. METHODS: This case series included patients with septic shock caused by acute appendicitis in Beijing Children's Hospital between January 2015 and December 2020. RESULTS: Six patients with septic shock caused by acute appendicitis were enrolled. One patient was an infant with extremely low weight; 2 patients were obese. The diagnosis was delayed in 4 patients (the time from onset to diagnosis was 5 days in 3 children and 4 days in 1 child). All patients had abnormally raised inflammatory markers (C-reactive protein 119.17 ± 48.36 mg/L, procalcitonin 129.95 ± 86.09 ng/mL). Severe abdominal infection was found in all patients. There was appendix perforation in 4 patients and diffused peritonitis in 3 patients. Two patients had metabolic diseases (Wilson disease and decreased biotinase activity, respectively). Five patients had an appendectomy and 1 patient received conservative treatment. Five patients were discharged in stable condition, while 1 patient died. CONCLUSIONS: Children with delayed diagnosis, abnormal body weight, significant elevation in inflammatory markers, and underlying metabolic disease may be at greater risk of complicated appendicitis and septic shock. EVIDENCEBASED MEDICINE: Level of Evidence: IV.