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.

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.

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.

[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.

Successful Treatment of Giant Common and Proper Hepatic Artery Aneurysm Complicating Autosomal Dominant Polycystic Kidney Disease.

Hepatic artery aneurysms (HAAs) complicating autosomal dominant polycystic kidney disease (ADPKD) are extremely rare. On account of the large number of renal cysts in ADPKD, ingenuity is required to safely perform surgery for HAA. Here, we present the case of a 60-year-old man with a common and proper HAA of >60 mm complicated with ADPKD. In this patient, difficulty in maintaining the field of view was expected during surgery. Hence, we performed a hybrid approach with celiac-to-right hepatic artery bypass by puncturing the femoral artery and cannulating the splenic artery to allow celiac-splenic artery balloon occlusion in case of rupture. More than 5 years postoperatively, the graft is patent without intervention.

Repeated aortic dissection in a patient with autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease is a systemic disorder associated with cardiovascular complications. However, there are few reports on autosomal dominant polycystic kidney disease-associated aortic dissection. Herein, we present a rare case of a 46-year-old man with autosomal dominant polycystic kidney disease who underwent endovascular repair for acute type B aortic dissection three years after his initial open surgery for acute type A aortic dissection. The postoperative course was uneventful, and he is doing well two years after the endovascular repair. Clinicians should be aware of the potential for occurrence of aortic dissection in patients with autosomal dominant polycystic kidney disease, including the possibility of recurrence.

Staged versus concurrent native nephrectomy and renal transplantation in patients with autosomal dominant polycystic kidney disease: A systematic review.

BACKGROUND: Patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD) frequently undergo native nephrectomy before transplantation. The nephrectomy may be a staged procedure or undertaken simultaneously with transplantation. When performed simultaneously, the transplant procedure is more prolonged, involves a larger operative field and incision. There is also a concern of a greater risk of graft loss with simultaneous nephrectomy and transplantation. Moreover, staged surgery may allow nephrectomy to be performed before immunosuppression introduction via a smaller incision or involving a minimally invasive approach. However, staged nephrectomy may require a period of dialysis not otherwise necessary if a transplant and nephrectomy were simultaneous. Moreover, only a single procedure is needed, implying the avoidance of a prior nephrectomy and its attendant morbidity in a patient with chronic renal insufficiency. To account for these issues, this study aims to compare the cumulative morbidity of two-staged procedures versus a single simultaneous approach in term of morbidity and graft outcomes. OBJECTIVES: This study aims to systematically review the literature to determine whether a staged or simultaneous approach to native nephrectomy in ADPKD is the optimal approach in terms of morbidity and graft outcomes. METHODS: A literature search of MEDLINE and EMBASE was conducted to identify published systematic reviews, randomized control trials, case-controlled studies and case studies. Data comparing outcomes of staged and simultaneous nephrectomy for patients undergoing kidney transplantation was extracted and analyzed. The main outcomes analyzed were length of hospitalization, blood loss, operative time, other early postoperative complications and risk of graft thrombosis. Meta-analysis was conducted where appropriate. RESULTS: Seven retrospective cohort studies were included in the review. There was a total of 385 patients included in the analysis, of whom 273 patients underwent simultaneous native nephrectomy and kidney transplantation. Meta-analysis showed an increased cumulative operative time in staged procedures (RR 1.86；95% CI 0.43-3.29 p = 0.01) and increased risk of blood transfusions (RR 2.69; 95% CI 1.92-3.46 p < 0.00001). For the transplant procedure, there were no significant difference in the length of stay (RR 1.03; 95% CI -2.01-4.14 p = 0.52), major postoperative complications (RR 0.02; 95% CI -0.15-0.10 p = 0.74) and vascular thromboses (RR 1.42 95% CI 0.23-8.59 p = 0.7). CONCLUSION: The results suggest that staged nephrectomy followed by kidney transplantation is associated with a longer cumulative operative time and increased cumulative risk of blood transfusions. There is no evidence to suggest that performing a simultaneous nephrectomy and kidney transplant procedure increases the perioperative mortality rate, major postoperative complication rates or risk of vascular thrombosis.

Endovascular aneurysm repair for aorto-iliac artery pathologies in patients with autosomal dominant polycystic kidney disease.

BACKGROUND: This study aims to evaluate the efficacy and safety of endovascular aneurysm repair (EVAR) of abdominal aortic or iliac artery pathologies in patients with autosomal dominant polycystic kidney disease (ADPKD). METHODS: From January 2014 to December 2019, fifteen consecutive patients (13 men, mean age 69.3 years, range 56-82 years) with abdominal aortic or iliac artery pathologies coexisting with ADPKD underwent EVAR in our department. Their general data, perioperative results and follow-up outcomes were retrospectively reviewed and analyzed. RESULTS: Among the fifteen patients, eleven had abdominal aortic aneurysms, one had isolated abdominal aortic dissection and the other three had iliac artery aneurysms. Three patients had thoracic penetrating aortic ulcer and two had intracranial aneurysms as the comorbidities. All patients underwent EVAR with the aorto-iliac pathologies successfully excluded. The average operative time was 171±73 minutes and average contrast volume was 87±12mL. The average follow-up time was 38.4 months (range 6-60). Aorta-bi-iliac stent-grafts were deployed in fourteen patients, while one patient received tubular stent-graft. Two patients underwent simultaneous TEVAR and EVAR, and One underwent EVAR 3 months after TEVAR. One patient was found to have a hematoma at the site of femoral access 3 days after EVAR. One patient was found to have a Type Ib endoleak 5 months after EVAR, and he recovered well with a secondary endovascular intervention. Contrast-induced nephropathy was observed in two patients (13%) post EVAR. Another patient developed renal failure 20 months after EVAR and was treated with regular hemodialysis. All other patients did not have any reported significant deterioration of renal function during follow-up. No other adverse events, such as death, paraplegia, aneurysm rupture, or open surgery conversion occurred during operation and follow-up. CONCLUSIONS: For patients with abdominal aortic or iliac artery diseases coexisting with ADPKD, EVAR had satisfactory mid-term outcomes, without significantly exacerbating the decline of renal function. However, patients with ADPKD might have multiple vascular lesions, especially intracranial aneurysms, which should be paid enough attention in clinical practice.

Paraneoplastic Polymyositis Due to Renal Cell Carcinoma in a Patient with Autosomal Dominant Polycystic Kidney Disease.

We herein report a 70-year-old man with malaise and muscle weakness that had developed within a month. The patient also had abdominal fullness due to polycystic kidney disease. Severe proximal skeletal muscle weakness and mild elevation of creatinine kinase to 301 IU/L were noted. A muscle biopsy of the right bicep showed polymyositis. Computed tomography showed a right renal mass, and an analysis after right nephrectomy identified clear cell carcinoma. The muscle weakness subsided one month after nephrectomy and intravenous immunoglobulin therapy. Therefore, we suspect that the development of polymyositis in this patient was closely related to renal cell carcinoma.

Simultaneous right-sided nephrectomy with orthotopic liver and kidney transplantation-An alternative method for patients with autosomal dominant polycystic liver and kidney disease.

PURPOSE: In patients suffering from autosomal dominant polycystic liver and kidney disease (ADPLKD), combined organ transplantation often poses a technical challenge due to the large volume of both organs. To simplify the transplantation procedure by improving the exposure of anatomical structures, we introduce a novel surgical technique of orthotopic liver and kidney transplantation. METHODS: The modified simultaneous liver and kidney transplantation technique via a right-sided L-incision included three steps: (1) right-sided nephrectomy in the recipient followed by (2) orthotopic liver transplantation in cava replacement technique and (3) the orthotopic kidney transplantation with arterial reconstruction to the right common iliac artery. RESULTS: In total, seven patients with ADPLKD were transplanted by using the modified transplantation technique. The mean operation time was 342.43 min (±68.77). Postoperative patients were treated for 6.28 days (±2.50) in the intensive care unit and were discharged from the surgical ward approximately 28 days (±5.66) after the operation with normal graft function. Complications associated with the use of the modified technique, such as bleeding, anastomotic stenosis, biloma, or urinoma, did not occur. CONCLUSION: Modified simultaneous liver and kidney transplantation is a safe alternative for patients with ADPLKD. By combining right-sided nephrectomy and orthotopic graft transplantation, the approach optimizes the exposure of anatomical structures and simplifies the transplantation procedure. Additionally, the modified transplantation technique does not require a particular organ explantation procedure and can be applied for all liver and kidney grafts.