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Introduction ADPKD is the main genetic cause of inherited end-stage renal disease in the world. Nephrectomies are common procedures in these patients performed in roughly 20% of them. They can be executed before, after or at the same time as a kidney transplantation. Common indications are lack of space for a future kidney transplant, pain, recurrent cyst infections and bleeding. There is still a lot of debate on the need and the timing of a nephrectomy in these patients especially for those patients who are kidney transplant candidates. Objectives The aim of this study is to investigate the influence of kidney volumes on indications for a nephrectomy, and to compare the pre-, peri- and post-operative information in ADPKD patients who are kidney transplant candidates. This data is used to suggest an evidence based surgical policy and to evaluate our surgical approach for performing a nephrectomy in ADPKD patients. Methods A retrospective analysis of all ADPKD patients who are candidates for a kidney transplantation between 1-1-2008 and 31-12-2019 in the University hospital of Leuven was performed. The volume of the native kidneys was determined using the CT imaging program Syngo.via®. Post-operative complications were collected up to one year after kidney transplantation or nephrectomy. They were scored according to the Clavien-Dindo classification and the comprehensive complication index was calculated. All data was compared based on the timing of the patients procedures and on the indications of the nephrectomy. Results A total of 182 patients were included in this study who underwent 122 kidney transplantations and 66 nephrectomies. The majority of nephrectomies were performed prior to a renal transplantation. The most common indication for a nephrectomy were the lack of space for a future kidney transplantation. The volumes of the abdominal cavity and the kidneys were significantly higher in patients receiving a nephrectomy than those without the procedure. This difference was found for both the absolute and relative volumes. When stratified based on the indication, a significantly higher volume was found in those with lack of space as an indication. The same significant difference was found for anatomical landmarks between indication groups. We observed no negative effect on the kidney function due to the nephrectomy. The late term kidney function was also not influenced by the timing of the nephrectomy. 30% of patients experienced major complications after transplantation. No difference was found between the timing of the transplantation and the number and gravity of complications post-procedure. 25% of patients experienced major complications post-nephrectomy. No differences were found based on the timing of the nephrectomies. No association was found for minor and/or major complications post-transplantation or post-nephrectomy. Conclusion We identified a cut off for the execution of a nephrectomy based on the volumes of the kidneys. We also determined that a nephrectomy should be performed based on the severity of the patients symptoms and not on the timing of the kidney transplantation as is our current standard of practice. In our experience, a nephrectomy does not compromise the results of a kidney transplantation. It remains a complex procedure that requires perfect surgical and medical management.

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Introduction Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease and an important cause of end-stage renal disease. In about 20% of patients with ADPKD, native nephrectomy is performed before, after or simultaneous with a kidney transplantation. Patients with untreatable pain, recurrent infection or hematuria, suspected malignancy, pressure symptoms and lack of space to place a transplant kidney are candidates for a nephrectomy. In the literature, there is no consensus regarding the indication or timing to perform a nephrectomy. Furthermore, the optimal surgical procedure and whether to perform the nephrectomy unilateral or bilateral are not standardized. Objectives The aim of this thesis is to evaluate pre-, peri- and postoperative information of ADPKD patients who underwent native nephrectomy to establish a surgical policy for performing nephrectomy in ADPKD patients. Methods A retrospective analysis of all ADPKD patients who underwent native nephrectomy in the University Hospitals Leuven between 1 January 2008 and 1 January 2018 was performed. Postoperative complications were collected up to one year after nephrectomy and scored according to the Clavien-Dindo classification. Clinical data was compared based on type and timing of nephrectomy. Results There were 87 patients identified who underwent a total of 94 nephrectomies. Fifty-four (57.4%) nephrectomies were performed before kidney transplantation, 25 (26.6%) simultaneous and 15 (16.0%) afterwards. Of these patients, 58 (61.7%) underwent unilateral nephrectomy. The main indication was lack of space in the pretransplant and simultaneous group and infection in the posttransplant group. Patients in the bilateral group experienced a significant longer operative time compared to the unilateral group, but no differences were seen in the number of red blood cell units transfused, length of stay or in the number of admissions to the intensive care unit. The incidence of arterial hypertension decreased 14% one year after nephrectomy. The majority of complications occurred within the first three months after nephrectomy. No significant differences were seen in complication rate, timing of complications or mortality between the pretransplant and posttransplant group. Morbidity and mortality were comparable between the unilateral and bilateral group. Abdominal complications were more common in the pretransplant group, while patients in the posttransplant and unilateral group developed more complications related to the urinary tract. Multivariable analyses showed that simultaneous nephrectomy is a predictor for the development of more severe complications. Conclusion Twenty percent of ADPKD patients require nephrectomy. Pretransplant nephrectomy is most commonly performed because of lack of space for a kidney transplant. Nephrectomy patients showed a reduced need of arterial hypertension therapy. Unilateral and bilateral nephrectomy are both safe in terms of postoperative morbidity. The final decision for timing and type of nephrectomy should take into account multidisciplinary team discussion, residual kidney function and diuresis together with patient preference.