Laparoscopic nephrectomy for autosomal dominant polycystic kidney disease

Background The authors reviewed their experience with laparoscopic nephrectomy for autosomal dominant polycystic kidney disease to evaluate whether patient-related or surgery-related factors influence operative outcomes.Methods A retrospective review was carried out of 22 consecutive laparoscopic nephrectomies performed by one surgeon in a university setting between March 1998 and March 2003. The impact of patient factors (body mass index, preoperative hemoglobin level, preoperative blood urea nitrogen and creatinine, kidney size and side, prior abdominal surgery, dialysis) and surgical factors (surgeon experience and preoperative embolization) on short-term outcomes (estimated blood loss, transfusion requirements, operative time, conversion, intra- and postoperative complications and length of stay) was analyzed using the Students t-test, Pearson correlation, and Mann–Whitney and Fisher tests.Results A total of 19 patients underwent 22 nephrectomies. The average patient age was 49 years (range, 36–65 years) and the average body mass index was 31.4 kg/m² (range, 20.4–64.5 kg/m²). Fourteen patients (68%) were receiving dialysis. Fifteen right (68%) and 7 left (32%) nephrectomies were performed. The median kidney size was 22 cm (range, 8–50 cm). Five patients (23%) had preoperative embolization. The median operative time was 255 min (range, 95–415 min). There were no mortalities. The intraoperative complication rate was 18% (1 vena cava laceration, 1 cecal perforation, 1 dialysis fistula thrombosis, 1 intrarenal bleeding requiring conversion), and the postoperative complication rate was 32% (1 myocardial infarction, 1 urgent laparotomy for clinical peritonitis, 1 minor bile fistula, 1 AV fistula thrombosis, 2 incisional hernias, 1 urinary retention). Four procedures (18%) were converted (1 for vena cava laceration, 1 for cecal perforation, 1 for intrarenal bleeding, 1 for adhesions). The median blood loss was 400 ml (range, 100–5000 ml). Eight patients (36%) received transfusions (median, 2 units). The median length of stay was 4 days. The patients who required blood transfusions had lower preoperative hemoglobin levels. Preoperative embolization did not affect surgical outcome. However, surgeon experience significantly reduced operative time.Conclusions Laparoscopic nephrectomy for autosomal dominant polycystic kidney disease is a safe procedure, providing patients with a short hospital stay. Complication and conversion rates are relatively high.Presented at the 11th International Congress of the European Association for Endoscopic Surgery and other Interventional Techniques (EAES), Glasgow, 15–18 June 2003     

Hand-assisted laparoscopic nephrectomy in autosomal dominant polycystic kidney disease

BACKGROUND: Among patients with renal insufficiency secondary to autosomal dominant polycystic kidney disease (ADPKD), the onset of refractory urinary infection, hypertension, pain, or hematuria often necessitates a nephrectomy. However, the huge size of these kidneys makes a standard laparoscopic approach difficult, and the increased fragility of these patients makes an open nephrectomy risky. A compromise position has been found in the realm of hand-assisted laparoscopic techniques, especially for patients in need of a bilateral nephrectomy. TECHNIQUE: Hand-assisted laparoscopic nephrectomy (HALN) is performed via a hand-assist device placed in the midline. A subxiphoid midline port and a midclavicular subcostal port are placed on the ipsilateral side. The right hand is inserted for left nephrectomy and the left hand for a right nephrectomy. The laparoscope is introduced into the subxiphoid port, and the surgeon's primary working instrument is passed via the midclavicular port. Occasionally, it is helpful to place a 5-mm subcostal port in the midaxillary line to aid in retracting the kidney. Once the kidney is devascularized, it is removed via the 7- to 8-cm hand-assist incision; drainage of cysts may be necessary during extraction to reduce the kidney size so that it can be withdrawn. If a bilateral approach is to be done, then after the first nephrectomy, the lateral 5-mm port is closed, and the table is rolled such that the contralateral side is elevated about 30 degrees to 45 degrees; a subcostal midclavicular 12-mm port is placed, and, if needed, a 5-mm port is inserted subcostally in the midaxillary line for renal retraction. RESULTS: Seven bilateral hand-assisted laparoscopic nephrectomy cases have been reported. In two reports, the mean operating times were 4.8 and 5.5 hours. The mean estimated blood loss was <350 mL. CONCLUSION: The hand-assisted laparoscopic approach makes both unilateral and bilateral nephrectomy feasible in ADPKD patients with acceptable morbidity.     

Long-term outcome of renal transplantation in autosomal dominant polycystic kidney disease

This study was performed to determine the long-term outcome of renal transplantation in 54 patients with end-stage renal failure secondary to autosomal dominant polycystic kidney disease (ADPKD) and in 107 patients with renal diseases other than ADPKD or diabetes mellitus matched by gender, age, year of transplantation, and source of the allograft. The overall patient survival and patient survival with a functioning first renal allograft were similar in both groups. Infection and cardiovascular accidents were the leading causes of early and late death in both groups. No cause of death was greatly overrepresented in the ADPKD group. Serious complications from extrarenal manifestations of ADPKD following renal transplantation included a ruptured intracranial aneurysm in one patient, a dissection of the ascending thoracic aorta in one patient, and infected hepatic cysts in two patients. Neoplasia (other than skin or cervical) occurred in four ADPKD patients and in one control patient and included one lymphoma in each group. Two ADPKD and one control patient had monoclonal gammopathies of undetermined significance. No complications related to the retention of native kidneys were detected in 12 ADPKD patients with a mean follow-up of 3 years. Cysts were observed in the renal allografts of some patients in both groups at autopsy and in a prospective computed tomography (CT) study of the allograft. However, we failed to detect a significant difference in the occurrence and number of the cysts between ADPKD and control patients.     

Pretransplant nephrectomy in patients with autosomal dominant polycystic kidney disease

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease and a frequent cause of end-stage renal failure. Transplantation in patients with ADPKD is associated with specific cyst-related problems, especially urinary tract infections (UTI). Although pretransplant nephrectomy has been applied in this group of patients, evidence of the benefits of this strategy is lacking. Therefore, we compared the outcomes and posttransplant complications among patients with or without pretransplant nephrectomy. PATIENTS AND METHODS: ADPKD patients (73) transplanted from cadaveric donors were reviewed retrospectively with regard to posttransplant complications and outcomes. The groups either underwent pretransplant unilateral nephrectomy (n = 30) or were transplanted with native kidneys intact (n = 43). RESULTS: Two patients underwent simultaneous bilateral nephrectomy due to a large size of the polycystic kidneys interfering with the transplant operation. Overall postransplant complications were more frequent in the group without nephrectomy (34% vs 20%); however, the difference was not statistically significant. Most complications were related to cyst infections with 3 deaths (12%) due to lethal septicemia in the group without nephrectomy. No infection-related deaths were noted in the group with pretransplant nephrectomy. CONCLUSIONS: Graft and patient outcomes as well postransplant complications were similar in both groups, independent of previous nephrectomy. It seems that pretransplant unilateral nephrectomy should not be routine and has no advantage over transplantation with both native kidneys intact, although this conclusion is limited by the small number of patients. An Individualized approach should be applied especially when there has been a history of cyst-related infection.     

Native nephrectomy in transplant patients with autosomal dominant polycystic kidney disease

INTRODUCTION

This study examined the clinical indications and timing for native nephrectomy (NN), together with the associated pathological findings in transplant patients with autosomal dominant polycystic kidney disease (ADPKD) at our institute over a period of 20 years.

METHODS

A retrospective review was performed of ADPKD patients who had undergone both kidney transplantation and NN. Patients were identified from the kidney transplant database between 1988 and 2008 at Guy''s and St Thomas'' Hospital and the notes reviewed. All NN specimens were re-reviewed and reported according to current guidelines.

RESULTS

There were 157 kidney transplants performed for ADPKD (114 cadaveric and 43 living donor). Of these, 31 required NN (28 bilateral). The timing of NN was pre-transplant in 10 cases, at the time of the transplant in 1 case and post-transplant in 20 cases. The indications for NN were urinary tract infection (n=14, 45%), pain (n=12, 39%), tumour suspicion (n=3, 10%), haematuria (n=1, 3%) and space (n=1, 3%). Mortality in this NN series was 3%, with a 65% surgical morbidity rate. The length of hospital stay post-NN was significantly longer with open compared with laparoscopic techniques (p=0.003). There were two renal cell carcinomas (RCCs) in this series. Both patients presented with macroscopic haematuria (bilateral pT1a papillary RCCs in one case and a pT3b clear cell RCC in the other case). The incidence of RCC in this series of ADPKD transplant patients was 1.3%.

CONCLUSIONS

We have demonstrated that the majority of ADPKD patients do not require NN, with only 20% of our series undergoing this procedure. The timing of NN is variable and dictated by indication. NN was only required to make space for transplantation in one case (combined kidney and pancreas transplant). The main indications for NN were recurrent infection and pain, where NN can provide a successful outcome. Laparoscopic NN can be performed safely in patients with ADPKD. Haematuria in such patients should not be assumed to be of benign origin and requires exclusion of urinary tract malignancy as the incidence of RCC in this population is at least as common as in the general population.     

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

BackgroundPatients 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.ObjectivesThis 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.MethodsA 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.ResultsSeven 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).ConclusionThe 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.     

Limitations of laparoscopy for bilateral nephrectomy for autosomal dominant polycystic kidney disease

PURPOSE: We retrospectively studied outcomes following bilateral hand assisted laparoscopic nephrectomy. MATERIALS AND METHODS: We retrospectively reviewed the charts of 18 patients with symptomatic autosomal dominant polycystic kidney disease who underwent bilateral hand assisted laparoscopic nephrectomy. Preoperative radiographic imaging was reviewed retrospectively to determine kidney size based on an ellipsoid shape. A visual analog pain scale with scores of 0 to 10 to assess pain related to autosomal dominant polycystic kidney disease was measured preoperatively and postoperatively. RESULTS: Average patient age was 48.2 years (range 30 to 64). Of the patients 14 successfully underwent bilateral hand assisted laparoscopic nephrectomy, while 4 required open conversion. A total of 16 patients underwent nephrectomy for pain and 2 underwent surgery for frequent recurrent symptomatic urinary tract infections. All patients except 1 underwent renal transplantation before bilateral nephrectomy. There was a significant difference in the volume of the right and left kidneys between the hand assisted laparoscopic and open groups (mean +/- SD 1,043 +/- 672 and 1,058 +/- 603.8 vs 4,052 +/- 548 and 3,592 +/- 1,752 cm(3), p <0.001 and 0.06 respectively). There were 5 complications, including wound infection and protracted ileus in 2 patients each, and incisional hernia in 1. In addition, the difference in mean preoperative and postoperative visual analog pain scores was statistically significant (6.9, range 3 to 10 and 0.5, range 0 to 2, p <0.05). CONCLUSIONS: Bilateral laparoscopic hand assisted nephrectomy is a safe and reliable option in patients requiring removal of the 2 kidneys in a single setting. Rather than performing staged nephrectomies, hand assisted laparoscopic nephrectomy allows the single administration of general anesthesia and provides effective relief of bothersome symptoms in patients with symptomatic autosomal dominant polycystic kidney disease. This procedure is safe in patients with renal transplants. Patients with massive polycystic kidneys with a kidney volume of greater than 3,500 cc are at increased risk for open conversion and they may have improved outcomes if open nephrectomy is attempted from the outset.     

Blood pressure and renal function in autosomal dominant polycystic kidney disease

  The purpose of this study was to identify hypertension in children and adolescents in an early stage of autosomal dominant polycystic kidney disease (ADPKD) by the application of ambulatory blood pressure monitoring (ABPM) over 24 h; 32 children and adolescents (mean age 12.3±4.7 years) were examined. The diagnosis was based on family history and ultrasound examination. In 21 children ADPKD was confirmed by molecular genetic analysis. At the time of the study, 45% patients were asymptomatic and all had glomerular filtration rates (GFRs) ≥65 ml/min per 1.73 m². By ABPM, 11 patients (34%) were defined as hypertensive (systolic or diastolic blood pressure >95th percentile), including 4 with an exclusive nocturnal hypertension. Of 7 patients with daytime hypertension, 4 had normal blood pressure by casual measurements. The nocturnal dip in blood pressure was reduced in 2 patients. Blood pressure correlated with renal size, but not with GFR, concentrating capacity, proteinuria, and plasma renin activity. The study reveals an early trend for increased blood pressure in children with ADPKD, requiring close supervision. Received October 18, 1996; received in revised form and accepted March 11, 1997     

Selective, concurrent bilateral nephrectomies at renal transplantation for autosomal dominant polycystic kidney disease

PURPOSE: An algorithm was developed for performing bilateral nephrectomies for specific indications before or at renal transplantation in patients with autosomal dominant polycystic kidney disease. Outcomes for the living donor arm of the algorithm are reported. MATERIALS AND METHODS: Patients with autosomal dominant polycystic kidney disease and end stage renal disease were evaluated for transplantation. Patients with recurrent pyelonephritis, hemorrhage, pain, early satiety or kidneys that extended into the true pelvis underwent bilateral nephrectomies. Bilateral nephrectomies with concurrent renal transplantation were performed if a living renal donor was identified. If no living donor was identified, pre-transplantation bilateral nephrectomies were done and the patients were listed for cadaveric donor renal transplantation. The living renal donor arm of the algorithm was evaluated by comparing certain parameters for 15 and 17 patients with autosomal dominant polycystic kidney disease who underwent pre-transplantation and concurrent bilateral nephrectomies, respectively, including patient and graft survival, delayed graft function, graft function, length of stay for each surgery, transfusions and complications. RESULTS: No deaths, graft failures or delayed graft function occurred. In the delayed renal transplant group median time from nephrectomy to living donor transplantation was 124 days. Serum creatinine at discharge home and 1 year after transplantation for the pre-transplantation nephrectomy cohort was 2.0 and 1.3 mg/dl, respectively. Seven of the 17 patients with concurrent nephrectomy underwent transplantation before starting renal replacement therapy. A longer mean total hospital stay in the pre-transplantation nephrectomy cohort was the only statistically significance outcome variable. CONCLUSIONS: Selective bilateral nephrectomies at living donor renal transplantation results in decreased total length of stay without compromising patient or graft outcomes and it allows preemptive renal transplantation. Concurrent nephrectomy is safe and it further validates the algorithm for selective, concurrent bilateral nephrectomies for patients with autosomal dominant polycystic kidney disease who undergo living donor renal transplantation.     

Hypertension in autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) has been shown to be associated with a greater than 50 percent incidence of hypertension prior to deterioration in renal function as assessed by glomerular filtration rate. The present study provides evidence for increased cardiac pre-load, as assessed by plasma atrial natriuretic factor (ANF) and cardiac index, in hypertensive as compared to normotensive ADPKD. The hypertensive ADPKD patients exhibited an increased renal vascular resistance as compared to the normotensive patients in spite of comparable glomerular filtration rates. It is hypothesized that the renal involvement of hypertensive ADPKD patients causes an impaired renal response to the observed increase in cardiac index, and also may release a venoconstrictor (such as angiotensin) which contributes to the enhanced cardiac pre-load and thus the hypertension.     

Angiogenesis and autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the growth of multiple cysts that in many cases result in end-stage renal disease. Current strategies to reduce cyst progression in ADPKD focus on modulating cell turnover, fluid secretion, and vasopressin signalling; but an alternative approach may be to target pathways providing “general support” for cyst growth, such as surrounding blood vessels. This could be achieved by altering the expression of growth factors involved in vascular network formation, such as the vascular endothelial growth factor (VEGF) and angiopoietin families. We highlight the evidence that blood vessels and vascular growth factors play a role in ADPKD progression. Recent experiments manipulating VEGF in ADPKD are described, and we discuss how alternative strategies to manipulate angiogenesis may be used in the future as a novel treatment for ADPKD.     

Acute conditions after kidney transplantation in patients with autosomal dominant polycystic kidney disease

There is a high risk of severe complications after kidney transplantation. In patients with autosomal dominant polycystic kidney disease (AD-PKD) the incidence of complications like ischaemic cardiac disease, acute myocardial infarction, pulmonary embolism, perforation of colonic diverticulosis is especially higher. The authors want to indicate another specific complication, rupture of the cyst of own polycystic kidney with retroperitoneal haemorrhage. Within the group of 658 patients who underwent kidney transplantation between January 1981 and January 2000 there were 54 (8.2%) patients with AD-PKD. Four patients with severe retroperitoneal haemorrhage due to rupture of the cyst of own polycystic kidney we present in a short case reports. All cases were fatal. Expect morphologic and functional follow up of the graft it is necessary to follow up polycystic kidney and indicate urgent nephrectomy in the case of any change.     

Nephrotic syndrome and rapid renal failure in autosomal dominant polycystic kidney disease

A 44-year-old man, with autosomal dominant polycystic kidney disease and hypertension under satisfactory control, developed nephrotic syndrome with negative serology. Open renal biopsy revealed focal glomerular sclerosis. Prior to the appearance of heavy proteinuria, serum creatinine was 1.7 mg/dl. After the nephrotic syndrome had been established, renal function deteriorated rapidly and hemodialysis was started within 2.6 years. In patients with autosomal dominant polycystic kidney disease, the appearance of nephrotic range proteinuria along with a rapid decline in renal function indicates the presence of a glomerular lesion, which needs to be investigated by renal biopsy.     

Evaluation of autosomal dominant polycystic kidney disease by DTPA renal scintigraphy

Sixty patients with previously documented autosomal dominant polycystic kidney disease (ADPKD) were investigated using dynamic kidney scintigraphy with^99mTc-diethylenetriaminepentaacetic acid (DTPA). Patients were subdivided in respect of glomerular filtration rate (GFR) as follows: PKD I group (normal GFR), PKD II group (moderately reduced GFR), and PKD III (severely reduced GFR). Seintigraphic features, time activity curves, excretion parameters, global and individual kidney functions were analyzed. Because of GFR dependent sensitivity, in advanced renal failure being only 0.1, and low reproducibility (11% intraobserver, 22% interobserver),^99mTc-DTPA dynamic kidney scintigraphy cannot be generally recommended for the diagnosis of ADPKD, but has to become a routine method for functional evaluation of both global and individual renal functions, as well as degree of excretion disturbances in ADPKD patients.     

Management of end-stage autosomal dominant polycystic kidney disease with hemodialysis and transplantation

This is an analysis of the outcome of 35 patients with end-stage autosomal dominant polycystic kidney disease (ADPKD) at Toronto Western Hospital (TWH) during a 10-year period. The primary treatment in each case was hemodialysis. In the 15 patients managed exclusively with hemodialysis the one- and five-year actuarial survival was 93% and 77% respectively. Twenty patients ultimately received a total of 26 cadaveric renal allografts. Graft survival at one year was 76%. One- and five-year patient survival was 92% and 73% respectively. Beyond 5 years a trend towards increased survival in the transplant group was seen, compared with the exclusively hemodialyzed group. Bilateral nephrectomy prior to transplantation was associated with high morbidity and mortality, and did not change either graft or patient survival. In view of the similar survival and because it is accepted that transplantation offers the highest quality of life amongst the modalities of treatment for end-stage renal failure, transplantation should be considered the treatment of choice for end-stage ADPKD. There is no justification for routine bilateral nephrectomy before renal transplantation.     

Boy with autosomal recessive polycystic kidney and autosomal dominant polycystic liver disease

BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) shows a great phenotypic variability between patients, ranging from perinatal demise to mildly affected adults. Autosomal dominant polycystic liver disease (PCLD) does not manifest in childhood. CASE-DIAGNOSIS/TREATMENT: A boy was reported with the co-occurrence of ARPKD and PCLD. He presented at the age of 16 days with pyelonephritis and urosepsis. Subsequent investigations showed enlarged kidneys and hyperechogenic renal medulla and liver parenchyma. Genetic analysis revealed compound heterozygous mutations in the PKHD1 gene (p.Arg496X and p.Ser1862Leu). After his mother was diagnosed with PCLD, the finding of a liver cyst on ultrasound prompted analysis of the PRKCSH gene, revealing a missense mutation (p.Arg139His). At the most recent follow-up at 13 years of age, the patient's course and clinical examination was uneventful with normal renal and liver function without evidence of portal hypertension. CONCLUSIONS: The patient with ARPKD and PCLD has so far demonstrated a benign clinical outcome, consistent with the great phenotypic variability of ARPKD and, apart from the liver cyst, asymptomatic manifestation of PCLD in childhood. However, close long-term follow-up is mandatory.