PDF(Pubmed)
Abstract：
BACKGROUND Caudal regression syndrome (CRS) is a rare anomaly characterized by maldevelopment of the caudal half of the body and can involve the genitourinary system. This report presents the case of a 13-year-old girl diagnosed with CRS and previously unknown distal vaginal atresia, presenting with monthly pelvic pain. CASE REPORT A 13-year-old pre-menarcheal patient with CRS sought emergency care due to debilitating monthly pelvic pain persisting for 3 months. Pelvic examination revealed the absence of a vaginal opening, and a rectal exam showed a 5-cm large bulge anteriorly, along with a 2-cm fibrous septum in the distal portion of the vagina. Pelvic ultrasound and magnetic resonance imaging confirmed the presence of hematometrocolpus and hematosalpinx on the right adnexa, while the left ovary was not identified. Treatment commenced with fixed analgesia and combined continuous oral contraception. Due to the persistent pain and uncertainty regarding the anatomy of the internal reproductive organs, diagnostic laparoscopy with drainage of the hematocolpus was performed 2 weeks later. Six months later, after multidisciplinary discussion, definitive surgery (pull-through vaginoplasty) was carried out, allowing for emotional preparation for postoperative dilation. One year after the definitive surgery, the patient remains asymptomatic, experiencing regular withdrawal bleeding with no signs of obstruction. CONCLUSIONS Patients with musculoskeletal anomalies should undergo urogenital tract evaluation. Timely identification of distal vaginal atresia is pivotal for devising appropriate treatment and averting complications. During the acute phase, laparoscopic drainage can alleviate symptoms and clarify anatomy, without compromising the success of subsequent definitive surgery.
摘要：
背景技术尾部回归综合征(CRS)是一种罕见的异常,其特征在于身体尾部一半的发育不良,并且可涉及泌尿生殖系统。本报告介绍了一名13岁女孩被诊断患有CRS和先前未知的远端阴道闭锁的情况。每月出现盆腔疼痛。病例报告一名13岁的初潮前CRS患者因每月骨盆疼痛持续3个月而寻求急诊治疗。盆腔检查显示没有阴道开口，直肠检查显示前部有5厘米大的隆起，阴道远端有一个2厘米的纤维隔膜。盆腔超声和磁共振成像证实右侧附件上存在血球和输卵管积血，而未发现左卵巢。从固定镇痛和联合连续口服避孕药开始治疗。由于内部生殖器官解剖结构的持续疼痛和不确定性，2周后进行诊断性腹腔镜检查并进行血肿引流.六个月后,经过多学科的讨论，进行了明确的手术（牵拉式阴道成形术），为术后扩张做好情绪准备。彻底手术一年后,病人仍然无症状，有规律的停药出血，没有阻塞的迹象。结论肌肉骨骼异常患者应进行泌尿生殖道评估。及时识别远端阴道闭锁对于设计适当的治疗方法和避免并发症至关重要。在急性期,腹腔镜引流术可以缓解症状，明确解剖，而不会影响后续确定性手术的成功。
