The treatment of patients with ADPLKD is always individual. The respective strategies are oriented to the patients’ individual symptoms as well as the patient- and organ-specific comorbidities. Controlling the symptoms associated with the cysts and preventing disease-specific complications are the primary aims of any therapy. In up to 90% (17) of all patients with ADPLKD, radiological or surgical interventions such as ethanol injection or cyst fenestration are the preferred therapy. By using these strategies, a transient reduction in symptoms can be achieved in approximately 92% of all patients (8) (9). Unfortunately, in 24% of these patients, the cysts reoccur. Depending on the therapy received, a morbidity rate of 23–50% and a mortality rate of 2–3% can be assumed (8) (9).
In patients with progressive symptoms and resulting comorbidities, transplantation remains the only curative treatment option. Transplantation can decrease the mortality and morbidity associated with the comorbidities and improve the quality of life (2) (3). Simultaneous liver and kidney transplantation, due to the massive cystic enlargement seen in ADPLKD, is technically challenging and varies depending on patients (13) and center-specific (14) (15) factors. This article demonstrates the secure feasibility of a new technique based on simultaneous orthotopic liver and kidney transplantation and right nephrectomy through a single incision in a sample of patients with ADPLKD.
Previously, Jochmans and colleagues (20) also described simultaneous organ transplantation through an individual median laparotomy. The kidney graft was placed in a retroperitoneal heterotopic position in the iliac fossa in a peritoneal pocket that was formed by peeling down the peritoneum from the midline (one-step cLKTx). Jochmans et al. documented a similar mortality and morbidity in the one-step cLKTx and the two-step cLKTx. Furthermore, the operation time (one-stage cLKTx 6.8 h (4.1 h-9.3 h) vs. two-step cLKTx 9.0 h (8.7 h-10.1 h)), as well as the cold ischemic time (one-step cLKTx 8.1 h (6.4 h-9.3 h)), could be reduced using the modified technique (20).
The novelty of our modified technique is right-sided nephrectomy during combined transplantation. Compared to Jochmans et al., our modified liver and kidney transplantation procedure could reduce the operation time by more than one hour (one-stage cLKTx 6.8 h vs. orthotopic simultaneous liver and kidney transplantation 5.45 h). This reduction is due to the right-sided nephrectomy. In addition, the right-sided nephrectomy facilitates the subsequent liver transplantation and the orthotopic transplantation of the kidney through the already prepared retroperitoneum. This surgical strategy results in a reduced intra-abdominal organ volume and improves the representation and detailed view of the anatomical structures during the preparation for hepatectomy and the implantation of the liver graft. Additionally, the vascular structures are openly accessible and can be prepared for subsequent kidney transplantation. Furthermore, the scar surface is reduced, since simultaneous transplantation can be carried out through a single incision.
Apart from the advantages of the modified transplantation technique, the morbidity and mortality rates are comparable with all established simultaneous transplantation techniques. Common perioperative complications after simultaneous transplantation are delayed graft function (18%), infections (24%) and cardiovascular complications (11%) (23). The evaluation of the one-stage cLKTx of Jochmans et al. showed a complication rate of up to 25% according to the Clavien-Dindo classification ≥ III. This rate is similar to that of the classical transplantation method (20) as well as our modified simultaneous liver and kidney transplantation. Furthermore, surgical complications associated with our modification of the surgical technique were not observed. Except in one case, conservative therapeutic management successfully treated all complications. Additionally, frequent complications of heterotopic kidney transplantation, such as lymphocele, could be excluded by orthotropic transplantation of the kidney in the physiological position. Furthermore, the modified simultaneous organ transplantation reduced the ICU and in-hospital time of patients with ADPLKD. The patients spent, on average, 6.28 days (± 2.50) in the ICU and 28 days (± 5.66) in the hospital following the transplantation procedure. In contrast, patients who were transplanted using the standard transplantation technique spent an average of 9 days (± 8.4) in the ICU and in total 33 days (± 9.7) in the hospital (13).
The limitation for applying this modified transplantation technique is the obligatory nephrectomy. Each patient should be individually evaluated as to whether a simultaneous orthotopic liver and kidney transplantation with accompanying right-sided nephrectomy is the therapy of choice, which depends on the clinical symptoms of the patient and the intraoperative course. The median nephrectomy rate ranged from 33% (unilateral) to 67% (bilateral) (20) for all patients with ADPLKD, independent of the respective therapy strategy. Some transplant centers recommend a total nephrectomy before or during the transplantation in this patient group (21). However, simultaneous orthotopic transplantation with accompanying right-sided nephrectomy offers clear advantages over a two-sided procedure by sparing one operation, including narcosis, as well as improving the operative access during the transplantation procedure. In contrast to the indication for nephrectomy, there is less variability in the procedure of orthotopic liver transplantation. Usually, liver transplantation is performed either in vena cava interposition or using the “piggy-back” technique. Studies have consistently shown that there is no significant difference in graft or patient survival between the two techniques. Generally, the 1-year and 5-year survival rates after simultaneous liver and kidney transplantation are 88% and 80%, respectively (22) (2) (3).
The interpretation of the results of the study is subject to further limitations. The small size of our study is one restriction and is due to the relatively rare procedure of simultaneous liver and kidney in the EUROTRANSPLANT region and the relatively rare prevalence of ADPLKD in the general population. Furthermore, simultaneous organ transplantation is considered in patients with type II and III ADPLKD (9) (11) according to Gigot’s classification. Another restriction of the study is the currently missing long-term course of our patients treated with the modified orthotopic liver and kidney transplantation. However, these results are important to provide a comprehensive assessment of the modified technique.
In summary, the modified orthotopic liver and kidney transplantation technique is feasible and promising for patients suffering from polycystic liver and renal degeneration. The procedure yields a comparable mortality and morbidity rate to the classical transplantation method (13) (20). We were also able to demonstrate that our surgical technique reduced the relevant operation-specific parameters (operation time and warm ischemia time) compared to studies conducted using other techniques. Further experience and data are needed, especially in long-term outcomes, to clearly evaluate our promising results. Additionally, long-term surgical complications such as incisional hernia and vascular, bile duct or urinary complications are subjects for further research. Furthermore, the described modified surgical technique must be compared to the already established standard simultaneous orthotopic liver and heterotopic kidney transplantation. A prospective randomized clinical trial is planned to compare the modified orthotropic surgical technique with the clinical standard heterotopic operation procedure. These results will be used to consider broadening the application of the modified orthotropic surgical technique to other indications for combined transplantation in patients with end-stage liver and renal disease.