Retrograde intrarenal surgery for stones associated with renal anomalies: caliceal diverticulum, horseshoe kidney, medullary sponge kidney, megacalycosis, pelvic kidney, uretero-pelvic junction obstruction.

PURPOSE OF REVIEW: Nowadays, due to the increase of imaging diagnosis, we identify easily renal anomalies, and we can choose between a wide range of armamentarium to treat symptomatic stones in those challenging cases. However, there is a lack of evidence and consensus on its use. The aim of this narrative review is to collect all the available data about safety and efficacity of retrograde intrarenal surgery (RIRS) in the treatment of kidney stones associated to a renal anomaly. RECENT FINDINGS: Renal anomalies are uncommon findings and even more if it has to be associated with renal stones. After a literature review of the past 2 years, there are a small number of studies that compare the outcomes in patients who have been treated with minimally invasive modalities and they are mainly focus on RIRS. SUMMARY: It is of extreme importance to know the advances on the stone treatment in anomalous kidneys. With the development of new laser technologies, RIRS is becoming a more interesting technique with high success rate and safety. Further studies are needed to make an accurate statement about the adequate surgical technique for each renal anomaly and also, clinical trials using new laser technologies.

Marginal parent donors-Process and ethics.

BACKGROUND: Pre-emptive kidney transplantation for end-stage kidney disease in children has many advantages and may lead to the consideration of marginal parent donors. METHODS: Using the example of the transplant of a kidney with medullary sponge disease from a parent to the child, we review the ethical framework for working up such donors. RESULTS: The four principles of health ethics include autonomy (the right of the patient to retain control over his/her own body); beneficence (healthcare providers must do all they can do to benefit the patient in each situation); non-maleficence ("first do no harm"-providers must consider whether other people or society could be harmed by a decision made, even if it is made for the benefit of an individual patient) and justice (there should be an element of fairness in all medical decisions). Highly motivated donors may derive significant psychological benefit from their donation and may thus be willing to incur more risk. The transplantation team and, ideally, an independent donor advocate team must make a judgment about the acceptability of the risk-benefit ratio for particular potential donors, who must also make their own assessment. The transplantation team and donor advocate team must be comfortable with the risk-benefit ratio before proceeding. CONCLUSIONS: An independent donor advocacy team that focuses on the donor needs is needed with sufficient multidisciplinary ethical, social, and psychological expertise. The decision to accept or reject the donor should be within the authority of the independent donor advocacy team and not the providers or the donor.

Safety and efficacy of minimally invasive percutaneous nephrolithotomy in the treatment of patients with medullary sponge kidney.

The purpose of this study was to review the safety and efficacy of the minimally invasive percutaneous nephrolithotomy in the treatment of medullary sponge kidney patients with complex renal calculi. Sixteen medullary sponge kidney patients with complex renal calculi underwent minimally invasive percutaneous nephrolithotomy procedures in our center were entered into this retrospective study. The data analyzed included patients' demographics, stone burden, operative time, operative blood loss, length of hospital stay, complications according to the modified Clavien system, and stone-free rate. All the patients in this study had complex renal stones that included 14 multiple stones and 3 partial staghorn calculi. The mean stone surface area was 779.5 ± 421.1 mm(2). Preoperative urinary tract infection was noted in 5 (31.2 %) patients. Minimally invasive percutaneous nephrolithotomy was successfully completed in 15 renal units in 14 patients. Two patients failed the procedure. The mean operative time was 87.3 ± 32.3 min. Mean hemoglobin drop was 25.3 ± 16.5 g/L. An initial stone-free rate of 60 % was achieved after the procedure, and the final stone-free rate was 86.6 % after auxiliary second look and/or shock-wave lithotripsy. Clavien grade I and II complications occurred in 3 (21.4 %) patients including the one (7.1 %) patient who required transfusion. All the complications were managed conservatively. No major complications occurred. This retrospective analysis confirmed that minimally invasive percutaneous nephrolithotomy was a safe alternative treatment for the medullary sponge kidney patients with complex renal calculi. This procedure provided an acceptable stone-free rate and low incidence of high-grade complications. Stone-free rate further could be further improved with auxiliary procedures.

The impact of modern endourological techniques in the treatment of a century old disease--medullary sponge kidney with associated nephrolithiasis.

The remarkable progresses of imagistic and interventional techniques that have been implemented during the last decades facilitated the diagnostic and allowed the treatment indication changes for numerous renal disorders. The purpose of the present lecture was to outline a data review concerning a renal anomaly first described one century ago as well as to evaluate the impact of endourologic technical progresses over the therapeutic management of the respective disease. The medullary sponge kidney (MSK) or Cacchi-Ricci disorder represents a disturbance in the renal development characterized by the cystic type dilation and diffuse precalyceal ducts ectasias. The disease is also known as precalyceal tubular ectasia, pyramidal sponge kidney or cystic dilation of the renal collecting ducts MSK patients are most often asymptomatic, the diagnosis being emphasized in light of the investigations imposed by related complications such as renal stones, urinary tract infections (pyelonephritis), renal tubes acidosis or urine concentration defects.

Medullary sponge kidney: causes and treatments.

Medullary sponge kidney is a little known and little understood disease. A patient with a medullary sponge kidney may undergo decades of suffering in the form of infections and pain before any diagnosis is even made. When a diagnosis is made, it is more than likely to be an incidental finding from a test for another problem. However, on diagnosis there are a number of options available to treat this condition. These include non-invasive treatments such as drug therapy, diet or extracorporeal shock wave lithotripsy, to invasive therapies such as percutaneous nephrolithotomy, ureteroscopy and more advanced surgery. The aim of this article is to highlight this little known condition, outline the effects, and discuss the treatment options available to patients.

A case of medullary sponge kidney (Cacchi-Ricci disease) mimicking a renal mass.

Medullary sponge kidney (MSK) is an uncommon benign congenital disorder, generally asymptomatic. In symptomatic patients the diagnosis is usually made by excretory urography performed in the most frequent complications such as renal stones, urinary tract infections and haematuria. Excretory urography can be very characteristic, showing cystic collections of ectatic collecting ducts like "bunches of grapes" or "bouquet of flowers". When haematuria represents the only symptom, and radiographic findings are not characteristic of MSK, the differential diagnosis with a renal tumor can be very difficult. We report a case of MSK that underwent nephrectomy since clinical and radiological features mimicked a renal tumor.

Calicopyelocystostomy in auto- and allotransplanted kidneys. Report of 2 cases.

A case of calicopyelocystostomy and one of pyelocystostomy are reported. In the first case, the operation was performed on an autotransplanted kidney. The operation was done in a patient with sponge kidney to facilitate spontaneous excretion of calculi. In the second case, the operation was performed in a patient with long-lasting urinary fistula which developed in a cadaver kidney transplant. Although the authors present only 2 cases, calicopyelocystostomy and pyelocystostomy can be recommended as a valuable method in complicated cases of recurrent nephrolithiasis and in patients with nonhealing long-lasting urinary fistulas which developed in transplanted kidneys.

Primary Epstein-Barr virus infection in a renal transplant recipient.

A young girl with familial nephronophthisis and chronic renal failure contracted a primary Epstein-Barr virus infection after renal transplantation. During the illness she developed a clinical picture of fever and pneumonitis which closely resembled the posttransplantation syndrome usually associated with cytomegalovirus, although she had no evidence of cytomegalovirus infection. A younger sibling with the same renal disease was found retrospectively to have apparently had an earlier subclinical Epstein-Barr virus infection.

Unilateral medullary sponge kidney: cause of persistent bacteriuria.

A patient with twenty years of persistent bacteriuria secondary to unilateral medullary sponge kidney, and in whom no antibiotic would sterilize the urine, was cured of her infection by nephrectomy in the presence of contralateral hydronephrosis. A review of the literature shows 23 cases of unilateral medullary sponge kidney in which nephrectomy or partial nephrectomy was apparently equally successful, although no bacteriologic follow-up data are given.