尾侧退化综合征伴孤立肾:1例报告及文献复习

A. Abubakar, B. Abubakar
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引用次数: 0

摘要

尾侧退行综合征(CRS)是一种罕见的先天性异常,常并发神经源性膀胱。上述患者的先天性肾脏和尿路异常对治疗提出了额外的挑战。本研究的目的是报告一例罕见的CRS合并神经源性膀胱和右侧孤立性肾积水。患者是一名3岁女婴,有严重的下尿路症状和先天性背部和下肢畸形。检查发现椎体和下肢异常。计算机断层扫描显示腰肩尾骨发育不全伴右侧孤立性肾积水。患者就诊时因尿路感染接受治疗,膀胱术证实为高压神经源性膀胱。她在最初的持续膀胱引流和随后的奥昔布宁清洁间歇导尿中表现良好。膀胱功能障碍的即时管理可以防止CRS和神经源性膀胱患者发生潜在的永久性孤立性肾功能障碍和继发性膀胱功能障碍。
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Caudal regression syndrome with a solitary kidney: A case report and review of the literature
Caudal regression syndrome (CRS) is a rare, congenital anomaly that is often complicated by a neurogenic bladder. Congenital anomalies of the kidney and urinary tract in aforesaid patient present an added challenge in the management. The aim and objective of this study is to report a rare case of CRS with neurogenic bladder and right solitary hydronephrotic kidney. The patient was a 3-year-old female infant with burdensome lower urinary tract symptoms and congenital back and lower limbs deformities. Her examination unveiled vertebra and lower limbs anomalies. The computed tomography scan showed lumbosacrococcygeal agenesis with a right solitary hydronephrotic kidney. She was treated for urinary tract infection at presentation, after which cystometry confirmed high-pressure neurogenic bladder. She did well to the initial continuous bladder drainage and the ensuing oxybutynin with clean intermittent catheterization. Instantaneous management of the bladder dysfunction prevents potentially permanent solitary renal dysfunction and the secondary bladder malfunction in a patient with CRS and neurogenic bladder.
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