Surgical Management of Autosomal Dominant Polycystic Kidney Disease: Principles and Current Practice.

Autosomal dominant polycystic kidney disease is the third most common cause of renal failure with no definitive treatment available that can directly target the development and growth of the cysts. Endeavours are being made to retard the growth of the cysts and preservation of renal function through medical treatment. However, 50% of the autosomal dominant polycystic kidney disease-affected persons develop complications and end-stage renal disease by the age of fifty-five and need surgical intervention for the management of complications, creation of dialysis access and renal transplantation. This review highlights the principles and current practice pertinent to the surgical management of autosomal dominant polycystic kidney disease. Keywords: polycystic kidney disease; nephrectomy; transplantation.

[Stanford Type A Acute Aortic Dissection Associated with Polycystic Kidney Disease].

Whereas cerebral aneurysm is a well-known consequence of autosomal dominant polycystic kidney disease (ADPKD), acute aortic dissection has been rarely reported. A patient was a 44-year-old male with a diagnosis of ADPKD, who had previously undergone transcatheter arterial embolization for a renal cyst hemorrhage. He presented with sudden onset of back pain, which got worse at emergency service. Contrast-enhanced computed tomography (CT) revealed Stanford type A acute aortic dissection. The patient subsequently underwent partial aortic arch replacement with a vascular graft under circulatory arrest. His postoperative course was complicated by pneumonia and required ventilation support for a week. Peak creatinine level was 3.28 mg/dl, but hemodialysis was not required. Patients with ADPKD should be considered a high-risk cohort of aortic dissection.

Unroofing of Lower Pole Native Kidney Cysts in Patients with Autosomal Dominant Polycystic Kidneys at the Time of Kidney Transplantation.

To report our experience with unroofing of ipsilateral lower pole kidney cysts in five patients with adult-type polycystic kidneys [ADPKD] when free implantation of kidney allograft interfered with lower pole native kidney cysts. In all of these patients, the native kidneys extended to the ipsilateral pelvis and bilateral ADPKD caused enlargement of the abdomen on gross examination. Unroofing of lower pole kidney cysts was performed during the same session of allograft transplantation. The decision to unroof lower pole cysts of the ipsilateral kidney was made after observing interference of lower pole cysts with free implantation of the allograft. In patient A, bilateral native nephrectomy was performed 6 weeks after kidney transplantation after consultation with the patient, when there was evidence of the good function of the allograft and the recipient was on a low dose of immunosuppressive medications. In other patients, no need for native nephrectomy observed. This experience suggests the possibility that when large ipsilateral kidney cysts interfere with safe implantation of the allograft, there is an option of performing cyst unroofing at the same session and proceeding with allograft implantation. In many patients, there would be no need for native nephrectomy and of deemed necessary, it will be performed later, when there is evidence of the good function of the allograft and the patient is on good kidney function with a low dose of immunosuppressive medications and a less risk profile for the operation. To our best knowledge, there is no prior such report in the literature.

Kidney Transplantation After Renal Transcatheter Arterial Embolization for Cyst Infection in a Hemodialysis Patient With Autosomal Dominant Polycystic Kidney Disease: A Case Report.

A 31-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) required antibiotic therapy for repeated renal cyst infections. The patient was scheduled for a living donor renal transplant with her mother as the donor. Two months before surgery, the patient was admitted to the hospital due to a severe renal cyst infection that improved with antibiotic treatment and percutaneous drainage, but the scheduled surgery was postponed. Transcatheter arterial embolization (TAE) was performed to control repeated renal cyst infections. Seven months after TAE, the patient underwent living donor renal transplantation. The postoperative course was uneventful, and the patient was discharged from the hospital on immunosuppressive medication 26 days after surgery with no evidence of recurrent infection or deterioration of renal function. Thirty months after transplantation, there has been no recurrence of infection.

A Case Report of Successful Kidney Transplantation in a Patient With Autosomal Dominant Polycystic Kidney Disease Who Underwent Thoracic Endovascular Aortic Repair for Type B Aortic Dissection.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is associated with several cardiovascular disorders, including aortic dissection, which preferentially occurs at the thoracic or abdominal level. Because there are few case reports describing surgical repair for aortic dissection followed by renal transplantation in patients with ADPKD, kidney transplantation performed after repair for aortic dissection remains challenging. CASE PRESENTATION: A 34-year-old Japanese man with end-stage renal disease secondary to ADPKD underwent thoracic endovascular aortic repair for complicated acute type B aortic dissection 12 months earlier. A contrast computed tomography scan before transplantation revealed an aortic dissection involving the descending aorta proximal to the common iliac arteries and confirmed multiple large bilateral renal cysts. After simultaneous right native nephrectomy, the patient underwent preemptive living-donor kidney transplantation obtained from his mother. Intraoperatively, we noted that dissection of the external iliac vessels was difficult because of dense adhesions. Arterial clamping was performed immediately below the bifurcation of the internal iliac artery to prevent further aortic dissection of the external iliac artery. After end-to-end anastomosis to the internal iliac artery was completed and the vascular clamp was released, the kidney began to produce urine immediately. CONCLUSION: This case suggests that kidney transplantation in patients undergoing endovascular aortic repair for aortic dissection can be performed by adequately applying a vascular clamp proximal to the internal iliac artery during vascular anastomosis.

The impact of pre-transplantation nephrectomy on quality of life in patients with autosomal dominant polycystic kidney disease.

PURPOSE: In selected ADPKD patients, a nephrectomy is required in the work-up for a kidney transplantation. Because the impact of this procedure is unknown, we investigated the effect of pre-transplantation nephrectomy on quality of life in this group. METHODS: In this retrospective cohort study all ADPKD patients, ≥ 18 years, who received a kidney transplantation in 2 ADPKD expertise centers between January 2000 and January 2016, were asked to participate. Quality of life was assessed using three validated questionnaires on three time points. Nephrectomy was performed in preparation for transplantation. RESULTS: Two hundred seventy-six ADPKD patients (53 ± 9 years, 56.2% male) were included. 98 patients (35.5%) underwent native nephrectomy in preparation for transplantation, of which 43 underwent bilateral nephrectomy. Pre-transplantation, ADPKD-IS scores were worse in the nephrectomy group vs. no-nephrectomy group (physical: 2.9 vs. 2.3, p < 0.001; emotional: 2.0 vs. 1.8, p = 0.03; fatigue: 3.0 vs. 2.3, p = 0.01). Post-transplantation and post-nephrectomy, ADPKD-IS scores improved significantly in both groups, with a significantly higher improvement in the nephrectomy group. During follow-up, all scores were still better compared to pre-transplantation. Observed physical QoL (ADPKD-IS physical 1.3 vs. 1.7, p = 0.04; SF-36 physical 50.0 vs. 41.3, p = 0.03) was better post-transplantation after bilateral nephrectomy compared to unilateral nephrectomy. In retrospect, 19.7% of patients would have liked to undergo a nephrectomy, while the decision not to perform nephrectomy was made by the treating physician. CONCLUSION: This study shows that pre-transplantation nephrectomy improves quality of life in selected ADPKD patients. Bilateral nephrectomy may be preferred, although the risk of additional complications should be weighted.

Native nephrectomy in patients with autosomal dominant polycystic kidney disease in the kidney transplant program - single-center retrospective results of 2000-2020.

INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disease that leads to chronic renal failure in about half of patients. It is a multisystemic disease with a predominance of kidney involvement, which significantly worsens the patient's health. Controversial issues include the indication and the timing and technique of nephrectomy of native polycystic kidneys. METHODS: A retrospective observational study focused on the surgical aspects of patients with ADPKD who underwent native nephrectomy at our institution. The group included patients operated on in the period 1/1/2000-31/12/2020. A total of 115 patients with ADPKD were enrolled (14.7% of all transplant recipients). We evaluated the basic demographic data, type of surgery, indications and complications in this group. RESULTS: Native nephrectomy was performed in 68 out of a total of 115 (59%) patients. Unilateral nephrectomy was done in 22 (32%) patients and bilateral in 46 (68%). The most common indications were infections (42 patients, 36%), pain (31 patients, 27%), hematuria (14 patients, 12%), gastrointestinal reasons (1 patient, 1%), respiratory reasons (1 patient, 1%), obtaining a site for transplantation (17 patients, 15%) and suspected tumor (5 patients, 4%). CONCLUSION: Native nephrectomy is recommended in symptomatic kidneys, or in asymptomatic kidneys when it is necessary to obtain a place for kidney transplantation, and in kidneys where a tumor is suspected.

Introduction of laparoscopic nephrectomy for autosomal dominant polycystic kidney disease as the standard procedure.

PURPOSE: Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disorder and accounts for 5-10% of all cases of kidney failure. 50% of ADPKD patients reach kidney failure by the age of 58 years requiring dialysis or transplantation. Nephrectomy is performed in up to 20% of patients due to compressive symptoms, renal-related complications or in preparation for kidney transplantation. However, due to the large kidney size in ADPKD, nephrectomy can come with a considerable burden. Here we evaluate our institution's experience of laparoscopic nephrectomy (LN) as an alternative to open nephrectomy (ON) for ADPKD patients. MATERIALS AND METHODS: We report the results of the first 12 consecutive LN for ADPKD from August 2020 to August 2021 in our institution. These results were compared with the 12 most recent performed ON for ADPKD at the same institution (09/2017 to 07/2020). Intra- and postoperative parameters were collected and analyzed. Health related quality of life (HRQoL) was assessed using the SF36 questionnaire. RESULTS: Age, sex, and median preoperative kidney volumes were not significantly different between the two analyzed groups. Intraoperative estimated blood loss was significantly less in the laparoscopic group (33 ml (0-200 ml)) in comparison to the open group (186 ml (0-800 ml)) and postoperative need for blood transfusion was significantly reduced in the laparoscopic group (p = 0.0462). Operative time was significantly longer if LN was performed (158 min (85-227 min)) compared to the open procedure (107 min (56-174 min)) (p = 0.0079). In both groups one postoperative complication Clavien Dindo ≥ 3 occurred with the need of revision surgery. SF36 HRQol questionnaire revealed excellent postoperative quality of life after LN. CONCLUSION: LN in ADPKD patients is a safe and effective operative procedure independent of kidney size with excellent postoperative outcomes and benefits of minimally invasive surgery. Compared with the open procedure patients profit from significantly less need for transfusion with comparable postoperative complication rates. However significant longer operation times need to be taken in account.

Supine PCNL in autosomal dominant polycystic kidney disease.

AIMS AND OBJECTIVES: Standard percutaneous nephrolithotomy (PCNL) has routinely been performed in prone position in autosomal dominant polycystic kidney disease (ADPKD) with nephrolithiasis. The objectives of our present study is to ensure optimum access to the renal collecting system, reducing operative time, and anesthetic morbidity during supine PCNL in ADPKD with nephrolithiasis. METHODS: Seven patients were selected randomly. There were no preference for age, gender, size, location and laterality of stone, or BMI . All the patients fit into the AUA guideline criteria for management by percutaneous nephrolithotomy. Preoperative, perioperative, and follow up data were collected prospectively. RESULTS: Seven patients underwent supine PCNL in approximately 2.5 year in modified supine position. There was no intraoperative, post operative, or on follow up complications in any patient. In all the patients stones were cleared completely in single sitting. CONCLUSIONS: Supine PCNL in ADPKD with nephrolithiasis is an alternative with similar outcomes to the standard prone PCNL. It provides an additional benefit of performing the procedure in a single position, which is known to reduce total operating time, less anesthesia related complications, less neuromusculoskeletal injury, and reduce physical strain on operating.

The Need for Routine Native Nephrectomy in the Workup for Kidney Transplantation in Autosomal Dominant Polycystic Kidney Disease Patients.

INTRODUCTION: There is no consensus if nor when a native nephrectomy should be performed in the workup for kidney transplantation in ADPKD patients. In our PKD Expertise Center, a restrictive approach is pursued in which nephrectomy is performed only in patients with severe complaints, i.e., in case of serious volume-related complaints, lack of space for the allograft, recurrent cyst infections, persistent cyst bleedings, or chronic refractory pain. We analyzed in a retrospective cohort study whether this approach is justified. METHODS: All ADPKD patients who received kidney transplantation between January 2000 and January 2019 were reviewed. Patients were subdivided into three groups: no nephrectomy (no-Nx), nephrectomy performed before (pre-Tx), or after kidney transplantation (post-Tx). Simultaneous nephrectomy together with transplantation were not performed in our center. RESULTS: 391 patients (54 ± 9 years, 55% male) were included. The majority of patients did not undergo a nephrectomy (n = 257, 65.7%). A nephrectomy was performed pre-Tx in 114 patients (29.2%). After Tx, nephrectomy was performed in only 30 patients (7.7%, median 4.4 years post-Tx). Surgery-related complication rates did not differ between both groups (38.3% pre-Tx vs. 27.0% post-Tx, p = 0.2), nor were there any differences in 10-year patient survival (74.4% pre-Tx vs. 80.7% post-Tx vs. 67.6% no-Nx, p = 0.4), as well as in 10-year death-censored graft survival (84.4% pre-Tx vs. 85.5% post-Tx vs. 90.0% no-Nx, p = 0.9). CONCLUSIONS: This study indicates that with a restrictive nephrectomy policy in the workup for kidney transplantation, only a part of ADPKD patients need a native nephrectomy.

Native nephrectomy and arterial embolization of native kidney in autosomal dominant polycystic kidney disease patients: indications, timing and postoperative outcomes.

INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common causes of a need of renal replacement therapy. The need (elective vs. systematic) and timing of native kidney nephrectomy (before, after or during kidney transplantation) is a matter of debate and alternatives to surgery, mainly transcatheter arterial embolization have been explored. We performed a systematic review to report all available evidence on postintervention outcomes of native nephrectomy and arterial embolization in ADPKD patients. EVIDENCE ACQUISITION: A search on Medline, Embase, and Cochrane databases was performed to identify all studies reporting outcomes of native nephrectomy or arterial embolization in APKDs. EVIDENCE SYNTHESIS: Concerning native nephrectomy, a total of 3626 patients in 37 studies were included with 735, 210 and 2681 patients who underwent native nephrectomy respectively before, after or during kidney transplantation. Major complications were 12.2% in unilateral nephrectomy before transplantation, 25.0% in bilateral nephrectomy before transplantation, 17.7% in unilateral nephrectomy during transplantation, 20.8% in bilateral nephrectomy during transplantation and 23.8% in unilateral and bilateral nephrectomy after transplantation. A total of 230 patients in 7 series of arterial embolization were included. All arterial embolization were performed before transplantation. Mean volume reduction ranged from 36.3% at 3 months to 49% at 6 months. The major postintervention complication rate was 1%. CONCLUSIONS: Unilateral native nephrectomy before kidney transplantation was associated with the lowest major postoperative complication rate and appears to be the preferred strategy. Arterial embolization reduces kidney volume by 49% at 6 months. Arterial embolization could be considered when the reduction in size of the native kidney is not urgent.

Recurrent urinary tract infections in kidney transplant patients with polycystic kidneys.

INTRODUCTION: Kidney transplantation is now a routine method used to treat end-stage renal disease. About 10 % of kidney transplant patients are patients with autosomal dominant polycystic kidney disease (ADPKD). After successful kidney transplantation, recurrent urinary tract infections also occur in initially asymptomatic patients. MATERIAL AND METHODS: The group included 320 patients after kidney transplantation. We compared patients with ADPKD versus patients without ADPKD in terms of the presence of recurrent urinary tract infections. THE RESULTS: The incidence of recurrent urinary tract infections (rIMCs) was 18% in patients without ADPKD and 48% in patients without ADPKD. Nephrectomy after kidney transplantation due to recurrent urinary tract infections eliminated this infectious complication (in 86% of patients). CONCLUSION: Kidney transplant patients with ADPKD have a significantly higher incidence of recurrent urinary tract infections. Removal of polycystic kidneys is a suitable solution if the infection persists.

[Bilateral laparoscopic nephrectomy for polycystic kidney disease].

A clinical case of surgical treatment of a patient with autosomal dominant type of polycystic kidney disease, stage 5 of chronic kidney disease and secondary arterial hypertension is presented in the article. The technique of single-stage bilateral laparoscopic nephrectomy, patented by the authors, is described. The practicability and safety of a simultaneous bilateral procedures was demonstrated, as well as the advantage of laparoscopic access for this type of surgical interventions. The successful and prompt procedure allowed the patient to undergone to allotransplantation of a cadaveric kidney as soon as possible.

Utilizing the same incision for staged renal transplant in patients with polycystic kidney disease requiring hand-assisted laparoscopic nephrectomy.

BACKGROUNDS: Many autosomal dominant polycystic kidney disease (ADPKD) patients undergo nephrectomy and subsequent renal transplantation. We report our outcomes after hand-assisted laparoscopic nephrectomy (HALN) where a Rutherford-Morrison incision is used as a hand-port site and kidney extraction site, as well the future incision site for staged transplantation. METHODS: A retrospective review was performed on all adult nephrectomies for ADPKD by the Transplant Surgery department at Westmead Hospital between June 2011 and June 2021. Outcomes were compared between HALN, laparoscopic nephrectomy (LN) and open nephrectomy (ON) including operation time, hospital length of stay (LOS), post-operative complications, subsequent transplantation and post-transplantation wound complications. RESULTS: Twenty-two HALN, 8 LN and 5 ON were performed during the study period. Median kidney weights for HALN, LN and ON were significantly different (1575, 403, 3420 g respectively, P = 0.001). There was a significant difference in LOS between the HALN and ON (5.8 versus 9.8 days, P = 0.04), but not between HALN and LN (5.8 versus 5.1, P = 0.06). There was no significant difference for operation time (P = 0.34) and major complication rates (P = 0.58). There were 8 HALN, 5 LN and 2 ON who have had subsequent renal transplantation with one wound complication, an incisional hernia in the HALN group. CONCLUSION: Our HALN is associated with a shorter LOS and similar complication rate to ON and can be efficiently performed for significantly larger kidneys than LN without a significant difference in operation time or LOS. The same Rutherford-Morrison incision site can be used for transplantation.

Simultaneous Robotic-Assisted Bilateral Native Nephrectomy and Kidney Transplantation for Autosomal Dominant Polycystic Kidney Disease in Recipients With High Body Mass Index: Report of 2 Cases.

Robotic kidney transplantation is a safe, reproducible, and less morbid technique in high body mass index and end-stage renal disease. Polycystic kidney disease is a relative contraindication to robotic-assisted kidney transplantation because of the mass effect of the native kidneys on the patient's pelvis that prevents ideal exposure. We report the first 2 cases of robotic-assisted simultaneous bilateral nephrectomy and kidney transplantation for patients with obesity and adult polycystic kidney disease. The recipients were 2 males, 50 and 53 years old, with a body mass index of 35.1 41.6 kg/m2 and 41.6 kg/m2, respectively. Both recipients had suitable living donors. The average operating time was 395 minutes and the estimated blood loss was on average 250 mL. The postoperative course was uneventful and the patients were discharged home on days 4 and 5. Performing robotic nephrectomies simultaneously with kidney transplantation can be done safely, allowing patients with obesity and polycystic kidney disease needing bilateral nephrectomy, to take full advantage of minimally invasive kidney transplantation.

Safety and feasibility of synchronous unilateral nephrectomy and contralateral heminephrectomy for extremely severe autosomal dominant polycystic kidney disease: Techniques and outcome.

PURPOSE: To demonstrate the safety and feasibility of synchronous unilateral nephrectomy and contralateral heminephrectomy in extremely severe autosomal dominant polycystic kidney disease (ADPKD), which corresponds to the Mayo imaging classification classes 1D and 1E. MATERIALS AND METHODS: We retrospectively reviewed patients who underwent unilateral nephrectomy and contralateral heminephrectomy at the Seoul National University Hospital (Seoul, Korea) between May 1, 2016 and August 1, 2021. The preoperative kidney volume was calculated using the ellipsoid equation (length×width×thickness×π/6). The Mayo imaging classification was determined by height-adjusted total kidney volume and age. Using a midline vertical incision, heminephrectomy was performed first by horizontal transection, followed by contralateral nephrectomy. Hilar vessel clamping or resection-bed suturing was not required. RESULTS: In all, nine patients with ADPKD of the highest severity (Mayo class 1D/1E) underwent unilateral heminephrectomy and contralateral nephrectomy for the most common cause of severe abdominal discomfort and malnutrition. All nine patients had end-stage renal disease with hypertension and anemia. The median preoperative total kidney volume was 10,905.8 mL (interquartile range [IQR], 8,170.4-16,227.6 mL). The median operation time was 140 minutes (IQR, 125-185 min) and the median estimated blood loss was 250 mL (IQR, 200-425 mL). Eight of the nine patients were discharged without ICU care or any complications. Delayed pseudoaneurysm occurred in one case and was successfully managed by embolization. All patients were symptom-free for a median follow-up period of 2 years. CONCLUSIONS: Synchronous unilateral nephrectomy and contralateral heminephrectomy are safe and feasible treatment options for severe bilateral ADPKD.

[Lymphocytic leukopenia in two patients affected by polycystic kidney disease waiting for renal transplantation].

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, responsible for 10% of patients on renal replacement therapy. The disease is well known to be associated with many extrarenal manifestations. Leukopenia may also be present, even if it is not commonly identified as a typical extrarenal manifestation. Herein we describe two case reports of ADPKD patients with leukopenia. The first case is about a 47-year-old patient affected by ADPKD, regularly treated with peritoneal dialysis, who showed a progressive reduction of white blood cell count, mostly of lymphocytes. Lymphocytic leukopenia was so severe that, when he was called for transplantation from a deceased donor, he was considered temporarily not eligible. We then describe a second ADPKD patient regularly treated with peritoneal dialysis, who had stable lymphopenia for years. Six years after starting PD, it was necessary to perform bone marrow aspirate to investigate the simultaneous presence of hypogammaglobulinemia together with M-protein and to exclude monoclonal gammopathy. All the exams performed did not show any significant results, the patients were re-included in the waiting list and one of them was transplanted. Given our experience and what is reported in the literature, there seems to be enough evidence to consider leukopenia as an extrarenal manifestation of ADPKD. However, the clinical significance of leukopenia in ADPKD patients is not known. It could be interesting to investigate the leucocytes' function and if ADPKD patients with leukopenia are more susceptible to infection, or not. Moreover, it would be very useful to analyze the relationship between such manifestation and genotype/phenotype.