Department of Emergency Surgery, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China.
Pediatr Surg Int. 2024 May 4;40(1):121. doi: 10.1007/s00383-024-05705-0.
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.
拉潘综合征（Rapunzel syndrome）在儿童中较为少见，其临床特征尚不清楚。本研究报告了迄今为止最大的单中心系列儿科病例，旨在记录拉潘综合征患儿的临床特征和治疗方法。
对 2019 年至 2023 年期间患有拉潘综合征的儿童进行回顾性研究。我们记录了年龄、性别、症状、胃石位置、并发症和治疗选择。
纳入 10 例拉潘综合征患儿。中位年龄为 9.1 岁，均为女性。最常见的临床症状为上腹部肿块（90%）、腹痛（80%）和恶心呕吐（50%）。6 例（60%）出现并发症，包括小肠梗阻（20%）、严重胃扩张（10%）、肠穿孔（10%）、胆总管扩张（10%）、伴有胆囊炎的急性胰腺炎（10%）。5 例（50%）术前超声提示低回声异物延续至空肠或回肠末端。4 例（40%）尝试行胃镜取异物，均未成功。所有患者均行手术治疗，9 例行胃切开取异物术，1 例行胃切开取异物术联合肠穿孔修补术。所有患者均恢复良好，随访期间无复发。
超声诊断拉潘综合征的准确性较高；但如果不结合患者的病史，可能会导致误诊。内镜治疗风险较高，成功率较低。该疾病常伴有严重并发症，因此剖腹手术是一种安全有效的干预措施。
