From the Department of Nephrology, Vanderbilt University Medical Center, Nashville, USA.
From the Division of Pediatric Urology, Department of Surgery, Children's Hospital of Eastern Ontario, University of Ottawa, Ontario, Canada.
Exp Clin Transplant. 2021 Feb;19(2):95-103. doi: 10.6002/ect.2020.0292. Epub 2021 Jan 26.
Autosomal dominant polycystic kidney disease is the fourth most common single cause of end-stage renal disease worldwide with both renal and extrarenal manifestations, resulting in significant morbidity. Approaches to the management of this disease vary widely, with no broadly accepted practice guidelines. Herein, we reviewed the various surgical and interventional management options that are targeted toward treating the symptoms or addressing the resulting kidney failure. Novel treatment modalities such as celiac plexus blockade and renal denervation appear to be promising in pain relief; however, further studies are lacking. Renal cyst decortication seems to have a higher success rate in targeting cyst-related pain compared with aspiration only. In terms of requiring major surgical intervention, such as need and timing of native nephrectomy, there are several considerations when deciding on transplantation with or without a pretransplant native nephrectomy. Patients who are not candidates for native nephrectomy may consider transcatheter arterial embolization. Based on our review of the contemporary indications for genitourinary interventions in the management of autosomal dominant polycystic kidney disease, we propose an algorithm that depicts the decision-making process on assessing the indications and timing of native nephrectomy in patients with end-stage renal disease awaiting transplant.
常染色体显性多囊肾病是全球第四大常见的终末期肾病单一致病原因,具有肾内和肾外表现,导致发病率较高。该病的治疗方法差异很大,没有广泛接受的临床实践指南。在此,我们回顾了各种针对治疗症状或解决由此导致的肾衰竭的手术和介入治疗选择。针对缓解疼痛,新型治疗方法如腹腔神经丛阻滞和肾脏去神经似乎很有前景,但还需要进一步的研究。与单纯抽吸相比,肾脏囊肿去皮质似乎在针对囊肿相关疼痛方面具有更高的成功率。在需要进行主要手术干预(如是否需要进行以及何时进行原肾切除术)方面,在决定是否进行移植以及是否在移植前进行原肾切除术时,需要考虑几个因素。对于不适合进行原肾切除术的患者,可以考虑经导管动脉栓塞术。基于我们对常染色体显性多囊肾病治疗中泌尿生殖系统干预的当代适应证的回顾,我们提出了一个算法,描述了在等待移植的终末期肾病患者中评估原肾切除术的适应证和时机的决策过程。