謝享宸、賀昊中

臺中榮民總醫院 外科部 移植外科,泌尿外科

Hsiang-Chen Hsieh, Hao-Chung Ho

Division of Transplantation Surgery and Urology, Department of Surgery, Taichung Veterans General Hospital, Taiwan

 

Introduction: Cloacal malformations (persistent cloaca) is a complex malformation in which the rectum, vagina and urinary tract open into a single common channel. In previous study, when post-reconstruction follow-up, renal impairment causes significant morbidity and mortality in patients with a cloacal malformation. In this report, we presented a case to describe living donor renal transplantation in a patient with cloacal anomalies who progressed to end stage renal failure.

Case Report: A 18-year-old patient, a case of cloacal anomalies who underwent anal reconstruction, with bicornuate uterus and intra-abdominal double vagina, and post hysterectomy and complete resection of vagina, progressed to end-stage renal failure with regular hemodialysis since aged 16, and recevied living donor renal transplantation at aged 18, The kidney donor was her father. The operation was arranged after well discussion with patient and her family. Kidney transplantation was performed smoothly after vessels anastomosis and ureteroneocystostomy. The warm ischemic time and cold ischemic time was 1 minute and 126 minutes, respectively. The patient recovery smoothly in the post-operative period. The follow-up sonography of graft kidney revealed well perfusion and no hydronephrosis. The lab data reported decreased creatinine and stable sereum level of FK-506 when follow-up.

Discussion: Cloacal anomalies occur when failure of the urogenital septum to separate the cloacal membrane results in the urethra, vagina, rectum and anus opening into a single common channel. The reported incidence is 1:50,000 live births. The aetiology is still unknown. Cloacal malformations require surgical repair, but the procedure depends on the type and extent of the abnormality. The ultimate goal of treatment is to separate the urinary control, bowel control and vagina (sexual function). These surgeries are done in stages. In addition, several factors can impact the fertility of a woman with a cloacal anomaly, including Mullerian anomalies, previous surgical procedures or vaginal stenosis. In our case, due to bicornuate uterus and intra-abdominal double vagina with obstructed menstruation at puberty, that the risks of miscarriage and preterm birth are significantly increased, she received hysterectomy and complete resection of vagina.

Conclusion: Renal transplantation in patient with cloacal malformations is a rare case and maybe a challenging surgery because of the anomalies of urogenital structures.