Abstract：
OBJECTIVE: 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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
目的：长发公主综合征在儿童中并不常见，其临床特征仍不清楚。这项研究提出了迄今为止最大的单中心儿科病例系列，目的是记录Rapunzel综合征儿童的临床特征和治疗方法。
方法：对2019年至2023年的Rapunzel综合征儿童进行了回顾性研究。我们记录了年龄,性别,症状,牛黄的位置，并发症,和治疗选择。
结果：纳入10例Rapunzel综合征患者。平均年龄为9.1岁，他们都是女性。最常见的临床症状是上腹部肿块（90%），腹痛（80%），恶心和呕吐（50%）。6例（60%）发生并发症，包括小肠梗阻（20%），重度胃扩张（10%），肠穿孔（10%），胆道扩张(10%),急性胰腺炎与胆囊炎（10％）。术前超声检查提示5例（50％）低回声异物持续到空肠或回盲区。术前胃镜检查尝试4例（40%）取出异物，所有这些都失败了。所有患者均接受手术治疗,胃切口异物取出术9例,胃切口异物取出联合肠穿孔修补术1例。所有患者恢复良好。随访期间未观察到复发。
结论：超声诊断诊断Rapunzel综合征的准确性很高；然而，如果不补充患者的病史，可能会导致误诊。内窥镜显示出更高的治疗风险和降低的成功率。这种情况通常会出现严重的并发症，因此，使剖腹手术成为一种安全有效的干预选择。
方法：对2019年至2023年的Rapunzel综合征儿童进行了回顾性研究。我们记录了年龄,性别,症状,牛黄的位置，并发症,和治疗选择。
结果：纳入10例Rapunzel综合征患者。平均年龄为9.1岁，他们都是女性。最常见的临床症状是上腹部肿块（90%），腹痛（80%），恶心和呕吐（50%）。6例（60%）发生并发症，包括小肠梗阻（20%），重度胃扩张（10%），肠穿孔（10%），胆道扩张(10%),急性胰腺炎与胆囊炎（10％）。术前超声检查提示5例（50％）低回声异物持续到空肠或回盲区。术前胃镜检查尝试4例（40%）取出异物，所有这些都失败了。所有患者均接受手术治疗,胃切口异物取出术9例,胃切口异物取出联合肠穿孔修补术1例。所有患者恢复良好。随访期间未观察到复发。
结论：超声诊断诊断Rapunzel综合征的准确性很高；然而，如果不补充患者的病史，可能会导致误诊。内窥镜显示出更高的治疗风险和降低的成功率。这种情况通常会出现严重的并发症，因此，使剖腹手术成为一种安全有效的干预选择。
