Autosomal dominant polycystic disease is a multisystem inherited condition affecting the kidneys and is an important cause of end-stage renal disease. Patients with autosomal dominant polycystic disease experience symptoms related to size and cystic nature of their kidneys, which can be difficult to manage. Traditionally, the only surgical option for management was open bilateral /unilateral native nephrectomy, which carried with it significant morbidity and mortality. Therefore, it was deemed unsafe and rarely performed.
However, surgery for autosomal dominant polycystic disease has evolved rapidly with the advent of minimally invasive surgery and improved medical management of end-stage renal failure patients. Laparoscopic and hand-assisted laparoscopic techniques have been adopted and have demo-nstrated reduced morbidity. The timing of this intervention in relation to transplant is controversial and presents a major challenge in managing this patient population.
Key words : Renal, Polycystic kidney syndrome
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited diseases of the kidneys affecting 1 in 500 to 1000 people. It has a typical onset in the fourth decade of life, and is responsible for 5% to 10% of all end-stage renal failures, with up to 50% of ADPKD patients progressing to end-stage renal failure and requiring renal replacement therapy by the age of 60 years.1
Autosomal dominant polycystic disease is a multisystem condition characterized by renal manifestations such as bilaterally enlarged cystic kidneys, early hypertension and progressive renal failure; as well as extrarenal manifestations such as liver cysts, diverticular disease, mitral valve prolapse, and intracranial cerebral “berry” aneurysms.2
Many patients experience symptoms from their enlarged kidneys, which cause a local mass effect that compresses the adjacent structures and results in abdominal pain, early satiety, and abdominal distension. Furthermore, the cysts are prone to rupture and hemorrhage, exposing the patient to an increased risk of recurrent bouts of urinary tract infections and renal calculi.3 The management of the symptoms and other abdominal complications of ADPKD is complex and has represented a significant technical challenge to surgeons. Debate about the indications, surgical approach, and timing (especially in relation to renal transplant) persists. A review of the literature regarding the progression of surgical intervention in ADPKD was therefore performed.
Indications of bilateral nephrectomy in autosomal dominant polycystic
There is no clear guidance or agreement on the indications for bilateral nephrectomy in ADPKD and large variations exist regarding the criteria for surgery.4 Most studies have reported indications that fall into 3 broad categories:
First, abdominal symptoms: typically the result of compression of local structures and cyst rupture or hemorrhage, which expose the patient to recurrent urinary tract infections and life-threatening sepsis.3,5 It also may be the result of renal capsular distension, experienced as a chronic pain syndrome, typically in hemodialysis-dependent patients who benefit from bilateral nephrectomy even without subsequent transplant (in terms of their Health Related Quality of Life score).6
Second, anatomic considerations: concern about the physical space available for a future renal transplant (without concomitant nephrectomy) if the large cystic kidneys encroach on pelvis may encourage the surgeon to consider nephrectomy to avoid complicating a future transplant procedure.7,8 And third, suspicious cystic lesions: these lesions represent a small but clinically important (and controversial) proportion of nephrectomies because of the reported 3-fold increase in the incidence of renal cell carcinoma in patients with ADPKD and end-stage renal failure in comparison to non-ADPKD patients with end-stage renal failure.9
Open bilateral nephrectomy for autosomal dominant polycystic disease
Bilateral nephrectomy surgery has been thought to be a dangerous, and therefore an unnecessary, pro-cedure that exposes patients to unacceptable levels of morbidity and mortality. Bennett and associates report a rate of 38% in major complications and 3% in mortality. Mendelssohn and associates have shown a similarly undesirable 12% morbidity and 5% mortality rates; most of which is attributable to intraoperative hemorrhage, ascites, and abdominal sepsis.10,11 This results in a sharp fall in the number of bilateral nephrectomy procedures undertaken and reflects the attitudes of surgeons and patients to this procedure and the potential risks associated with it. Furthermore, associated intraoperative blood loss ran the risk of creating an unnecessary transfusion requirement, which for potential transplant recipients, exposes them to a significant risk of ABO sensitization and a subsequently limited pool of donor organs.
Minimally invasive approaches
With the advent of minimally invasive surgery and technical advances in laparoscopic surgery, new surgical approaches have emerged and more surgeons are intervening surgically in ADPKD. After the first reported laparoscopic nephrectomy was performed by Bales and associates12 via a transperitoneal approach, laparoscopic expertise developed quickly, and bilateral laparoscopic nephrectomies were soon safely performed in ESRD patients, typically for posttransplant hypertension refractory to medical treatments.13,14
However, the kidneys in these studies were smaller, noncystic, and had predictable anatomy, making their laparoscopic removal a technically distinct challenge to that of the kidneys of an ADPKD patient. Elashry and associates was the first team to successfully perform a laparoscopic nephrectomy in an ADPKD patient, but because of the technically challenging nature of the surgery in ADPKD patients, a unilateral laparoscopic nephrectomy was all that could be performed safely.15
More recently, laparoscopic bilateral nephrectomy in ADPKD has been achieved, proving to be a safe and effective alternative to open surgery, with reduced length of hospital admission, less postoperative pain, improved cosmesis, and an accelerated time to oral diet.16-19 These benefits are far reaching, especially in posttransplant patients with oral immunosuppressive medications requiring prompt resumption of their antirejection medication regimens and an increased risk of nosocomial infection with each additional day in hospital.
The studies describing the experiences of laparoscopic bilateral nephrectomy in ADPKD also have demonstrated significantly longer operative times with the laparoscopic surgical approaches when compared with open surgery. This prompted the adoption of hand-assisted laparoscopic (HAL) techniques, which had been in use in nephrectomy surgery shortly after the pure laparoscopic approach was introduced. Reduced operative times were seen since the adoption of HAL techniques.20,21 Hand-assisted laparoscopic bilateral nephrectomy in ADPKD is now a favored approach by many centers, with growing evidence showing its effectiveness and in some studies, demonstrating superiority to a pure laparoscopic approach. Hand-assisted laparoscopic nephrectomy has not been shown to carry any additional risk, yet it reduces operative time when compared to a pure laparoscopic approach.19 Furthermore, there is the ability to use the fingers for blunt dissection, receive tactile feedback, and manually manage bleeding to reduce blood loss and therefore transfusion of blood products.22
Transplant and bilateral nephrectomy
Perhaps the most controversial area of the debate in bilateral nephrectomy in ADPKD is the timing of native kidney removal in renal transplant patients. Most transplant centers would agree that despite advances in nutritional support, recombinant erythropoietin, and medical management of ESRD, pretransplant bilateral nephrectomy with a “dialysis bridge” presents major risks regarding vascular and peritoneal complications that render it undesirable in patients with residual native function and those imminently anticipating receipt of a donor allograft (ie, from a living donor).
Native nephrectomy at the time of transplant also has been uncommon because of the procedure being associated with a high complication rate. It is hindered by carrying a range of risks such as encountering an unanticipated complication in the nephrectomy stage of the procedure (which often precedes the implantation of allograft), delay (or cancellation) in donor organ arrival, intraabdominal sepsis from rupture of an infected cyst from the native kidney, large fluid shifts associated with hypotension and consequent deleterious hypoperfusion of the allograft, and prolonged anaesthetic time.8,23
Some studies have shown promising results with concomitant nephrectomy. Nunes and associates demonstrated that in 143 unilateral native nephrectomies, at the time of transplant, there was no difference in graft survival or length of hospital admission when compared with 16 transplant patients with no nephrectomy.24 The native nephrectomy group did, however, have longer operative times, increased blood product and fluid requirements and the unilateral native nephrectomy approach although evidently beneficial, does not address the inherent risks of the remaining cystic kidney; exposing the recipient to problems such as cyst infection, rupture or hemorrhage, which are all compounded complications in the transplant population.
Kramer and associates conducted a study looking at bilateral native nephrectomy (open) at the time of surgery and showed in the 20 cases reviewed that graft survival was 100%, and that there was a significant reduction in patient symptoms across the board.25 This study was small and although in keeping with some larger studies (eg, Drognitz and associates with 75 patients), it does not manage to dispel the fears of simultaneous surgery.26
More recently, combined approaches have emerged. Lucus and associates, in an adapted series, compared unilateral open nephrectomy via an extended Gibson incision followed by delayed unilateral laparoscopic nephrectomy versus transplant alone and delayed bilateral laparoscopic nephrectomy. Given the unavoidable second procedure this group aimed to prove that a smaller second procedure would mitigate the complications often associated with a lengthy bilateral laparoscopic nephrectomy in a posttransplant patient. They showed that unilateral nephrectomy followed by a delayed unilateral laparoscopic nephrectomy resulted in a reduced mean blood loss, intraoperative time, and did not affect significantly the complication rate.27
Because of the remarkable technical advances over the last 2 decades within transplant and minimal access surgery, we are at a point where laparoscopic bilateral nephrectomy is safe and effective in most patients. But challenges remain. One of the main challenges facing the field is the ability to remove extremely large kidneys laparoscopically. Lipke and associates reported an increased incidence of conversion from laparoscopic to open surgery in patients with kidneys of a volume larger than 3500 mL in their series of 18 patients.28 Furthermore, Ivey and associates showed that larger kidneys were not only associated with conversion to open surgery with a mass greater than 1500 g, but also had longer operative times and increased blood loss.29 Some surgeons have attempted strategies to reduce the size of kidneys intraoperatively with bursting of cysts, but this leads to spillage of cystic contents and is associated with increased pain, peritonitis, ileus, and may even be a source of seeding in cases of suspected malignancy.18 More recently however, Bansal and associates, in their series of 39 patients undergoing transperitoneal laparoscopic bilateral nephrectomy in ADPKD with massively enlarged kidneys (mean 1515 g; range, 412-4590 g), had no conversions to open surgery, no excessive blood loss or prolonged operative times.30
There remain major challenges facing the management of symptomatic ADPKD patients who are likely transplant recipients. The morbidity and complications associated with a “dialysis bridge” after bilateral native nephrectomy in the pretransplant phase are many, including vascular access and peritoneal related complications. For patients who have simultaneous transplant and native nephrectomy, they are exposed to the risk of cyst related complications as well as on-going symptoms up to the time of transplant, which is not necessarily predictable or defined. Furthermore, they face the challenge of anatomic space for the allograft that although could be created at the time of transplant in a simultaneous procedure, involves a larger, more complex and technically demanding procedure, which is associated with multiple risks, especially those related to the nephrectomy stage of the operation.8,23
A proposed approach to these patients that addresses these issues would include patients having a unilateral nephrectomy before transplant allowing them to remain independent of dialysis, reduce their risk of cyst-related complications, and creating space for the future allograft. After this, a transplant procedure would be performed with no additional intraoperative procedures. Thus, avoiding unnecessary complications and hazards associated with per-forming a simultaneous nephrectomy of a large cystic kidney, with the benefit of a less challenging transplant procedure because of the space created by the previous unilateral nephrectomy. Finally, these patients would have a delayed laparoscopic unilateral nephrectomy once they had recovered from their transplant, benefiting from the minimally invasive nature of this procedure and the well-established safety record.
Bilateral nephrectomy in ADPKD is a rapidly evolving area, influenced by technological advances in minimally invasive surgery and transplant. It is a safe and effective way of managing the symptoms associated with polycystic kidneys in patients on dialysis and transplants recipients, which can be performed in most cases via a minimally invasive approach. The optimal timing and type of surgery in patients receiving a renal transplant remains controversial, as does the management of extremely large cystic kidneys. More outcome-focused data are required to determine the most appropriate surgical management pathway for symptomatic patients with ADPKD.
Volume : 13
Issue : 3
Pages : 209 - 213
DOI : 10.6002/ect.2015.0104
From the 1London Postgraduate School of Surgery, London; 2Oxford
School of Surgery, Oxford; and 3Imperial College London, London, UK
Acknowledgements: The authors have no conflicts of interest to declare. No funding was received for this study.
Corresponding author: Fungai Dengu, Postgraduate School of Surgery, Department of Core Surgical Training, 32 Russell Square House, London WC1B 5DN, United Kingdom
Phone: +44 77 9984 4383