Pyeloplasty vs. nephrectomy for ureteropelvic junction obstruction in poorly functioning kidneys (differential renal function <20%): a multicentric study.

INTRODUCTION: Management of poorly functioning kidneys with ureteropelvic junction obstruction (UPJO) is controversial, with some recommending direct nephrectomy and others direct pyeloplasty, and others temporary diversion. This study aimed to determine whether pyeloplasty is associated with higher complication rate than nephrectomy under these circumstances and whether it allows for functional recovery. METHODS: A retrospective review of 77 patients undergoing surgery for UPJO associated with a differential renal function (DRF) < 20% at 4 centres between January 2000 and December 2015 was conducted. Short- and long-term complications between pyeloplasties (n = 47) and nephrectomies (n = 16) and the changes in the DRF after pyeloplasty were compared. RESULTS: Patients undergoing nephrectomy had significantly lower median pre-operative DRF (p < 0.001) and were significantly more likely to undergo a minimally invasive approach (p = 0.002) than those undergoing pyeloplasty. No postoperative variable was statistically different between groups. After a mean follow-up of 63 (10-248) months, no statistically significant difference was found in intra-operative, early, late, and overall complications between pyeloplasty and nephrectomy. Pyeloplasty failed in 3% (2/62) of cases. Of the patients undergoing successful pyeloplasty, 36 had a pre-operative and a postoperative renogram, and functional recovery >5% was observed in 13 (36%), whereas the DRF remained unchanged in 16 (45%). Only postnatal diagnosis was associated with a significantly higher chance of functional recovery (odds ratio [OR] = 4.13, p = 0.047). DISCUSSION: Nephrectomy was more commonly performed using a minimally invasive approach and required less-intense follow-up than pyeloplasty. Moreover, complications after pyeloplasty, although occasional, required additional surgery which was never required after nephrectomy. Moreover, it should be considered that conservative treatment might be a third option in some of these patients. CONCLUSIONS: In the study patients, pyeloplasty was not associated with significantly higher morbidity than nephrectomy. Need for deferred nephrectomy seems exceptional in decompressed kidneys even though renal function remains poor. Of the pyeloplasty cases not requiring additional surgery, one-third showed an improvement in DRF and a postnatal diagnosis was the only predictive factor for renal functional recovery.

The role of renal biomarkers to predict the need of surgery in congenital urinary tract obstruction in infants.

INTRODUCTION: The diagnosis of renal function impairment and deterioration in congenital urinary tract obstruction (UTO) continues to be extremely challenging. The use of new renal biomarkers in this setting may favor early renal injury detection, allowing for a reliable choice of optimal therapeutic options and the prevention or minimization of definitive renal damage. OBJECTIVE: The aim of the study was to investigate a selection of promising biomarkers of renal injury with the intention of evaluating and comparing their profile with clinically based decisions for surgical intervention of infants with congenital obstructive uropathies. STUDY DESIGN: The first-year profile of renal biomarkers, serum creatinine (sCr), serum and urine cystatin C (CyC), neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), transforming growth factor beta-1 (TGF-ß1), retinol-binding protein (RBP), and microalbuminuria (µALB), was analyzed in a cohort of 37 infants with congenital UTO, divided into three subgroups, 14 cases with grade III unilateral hydro(uretero)nephrosis, 13 cases with grade III bilateral hydro(uretero)nephrosis, and 10 cases with low urinary tract obstruction (LUTO), compared with 24 healthy infants matched by gestational age and birth weight. Serum and urine samples were stored at -70 °C and thereafter analyzed by quantitative enzymatic immunoassay. RESULTS: Compared with the control group (Figure), all renal biomarker values were significantly increased in patients (P ≤ 0.02). In the unilateral hydronephrosis and LUTO group, RBP (P ≤ 0.043), NGAL (P ≤ 0.043), KIM-1 (P ≤ 0.03), and TGF-ß1 (P ≤ 0.034) values dropped significantly after surgery. Neutrophil gelatinase-associated lipocalin alone and in combination with urine and serum CyC demonstrated the best performance in determining the need for surgery (area under the curve, 0.801 and 0.881, respectively). Biomarker profile analysis was suggestive of surgical intervention in 55.4% (7/13) of non-operated cases, and most of the biomarker values were above the cutoff levels within at least 3 months before the clinically based surgical decision in 58% (14/24) of all operated patients. DISCUSSION: To the best of the authors' knowledge, this is the first study to present the clinical use of selected group of serum and urinary biomarkers in the setting of UTO to distinguish between patients who would benefit from surgery intervention. The most promising results were obtained using NGAL, RBP, TGF-ß1, and KIM-1, especially in the unilateral hydro(uretero)nephrosis and LUTO subgroups when compared with the control group. CONCLUSIONS: Urine biomarkers, alone and in combination, demonstrated high potential as a non-invasive diagnostic tool for identifying infants who may benefit from earlier surgical intervention.

Diagnostic work-up and laparoscopic correction of an ectopic ureter.

INTRODUCTION: A duplex renal collecting system is a common congenital anomaly in children. Continuous dribbling (especially if after the toilet-training period) should raise suspicion of the presence of an ectopic ureter, which is most often associated with ureteral duplication. This video will demonstrate the complete diagnostic work-up necessary in these cases. CASE REPORT: A 10-year-old girl presented with continuous dribbling. Ultrasonography and computerized tomography depicted a duplex system on the left side, with the upper pole ureter ectopically inserting into the vaginal cavity and good upper pole renal parenchyma. A careful urethrocystoscopy showed a topic right ureteral orifice and a topic lower pole left ureteral orifice. Retrograde pyelography was performed and displayed normal left lower pole anatomy. A vaginography was performed, which showed reflux to the ectopic ureter. Vaginoscopy clearly identified the ectopic ureteral orifice. A guide wire was introduced through this meatus and retrograde contrast injection confirmed the diagnosis of an ectopic ureter. RESULTS: At laparoscopy, a larger upper pole ureter and a normal lower pole ureter on the left side were identified. A termino-lateral ureteroureteral anastomosis was performed. After the procedure, the child reported immediate resolution of urinary dribbling. CONCLUSION: In order to optimize its surgical correction, efforts should be made to appropriate localization of the ectopic ureter.

27 years of experience with the comprehensive surgical treatment of prune belly syndrome.

INTRODUCTION: Prune belly syndrome (PBS) presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism. As a result, urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. OBJECTIVES: To report experiences with comprehensive surgical management of prune belly syndrome. MATERIAL AND METHODS: From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. According to individual needs, treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract, and perform bilateral orchiopexy and circumcision, which were performed in one procedure. Urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation with evidence of significant stasis and/or vesicoureteral reflux was associated with recurrent urinary tract infections (UTI). Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions. The median age at surgery was 16 months and children were followed for a median of 143 months. RESULTS: Abdominal appearance and tonus were improved in 90% of the children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children and long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Lower urinary tract reconstruction was performed in most children (95.6%); on late follow-up, continence was observed in 81% of them, while incontinence was present in 19% and usually associated with polyuria. Adequate bladder emptying was possible in most boys (82.6%), while the remaining required clean intermittent catheterization. Pre-operative UTI was present in 89.1% and urinary sepsis in 15.2%. Postoperatively, the incidence of laboratorial UTI was significantly reduced to 39.1%, while urinary sepsis was absent. Bilateral orchiopexy was performed in all children, with 85% of the testes becoming normal in size and well located in the scrotum. CONCLUSIONS: Comprehensive surgical treatment is feasible and has good long-term results. A considerable incidence of reoperations due to complications or progression of the disease was observed. The long-term results for reno-ureteral anatomy and function, bladder function, infection, testicular size and location, as well as abdominal aspect and tonus, show that comprehensive surgery is an adequate method for managing children with PBS.

Comparison of videolaparoscopic versus open surgery for benign renal diseases in children.

INTRODUCTION: Laparoscopic nephrectomy in children has become a reasonable alternative to open nephrectomy and has replaced open surgery for many kidney diseases. The aim of our study is to evaluate transperitoneal videolaparoscopic procedures in benign renal disease compared with the open surgical approach. PATIENTS AND METHODS: 34 children aged between 17 days and 15 years (mean, 6.14 years) were divided into two nephrectomy groups. The first underwent transperitoneal videolaparoscopic nephrectomy and was composed of 21 patients (12 female and 9 male) aged between 2 months and 15 years (mean, 7.42 years). The second group underwent open nephrectomy and was composed of 13 patients (6 female and 7 male) aged between 17 days and 11 years (mean, 3.91 years). The groups were compared for time of anaesthesia, operating time, length of hospital stay, postoperative pain and time to restore oral intake. Short and long term complications were also evaluated. Statistical analysis was performed by a Student's t test, with a p value < 0.05 being considered significant. The study was approved beforehand by the Scientific Ethics Committee in our institution. RESULTS: Statistically significant differences were observed only for the length of hospital stay. No cases in the laparoscopic group were converted into open surgery. There were no immediate or late complications. Blood loss was negligible and so it was not necessary to administer any transfusions. CONCLUSIONS: In our experience, transperitoneal videolaparoscopic nephrectomy has similar results to those of open nephrectomy, except for hospitalisation times.

Laparoscopic management of intersexual states.

Laparoscopy has gained acceptance as the ideal method of surgical treatment of the internal [figure: see text] genital organs in patients with intersex disorders. The intersexual states for which laparoscopy is needed are female and male pseudohermaphroditism, true hermaphroditism, and Turner's syndrome. In these patients, the indications for laparoscopy are the removal of normal gonads and ductal structures that are contrary to the assigned gender and the removal of dysgenetic gonads that are nonfunctional and that present potential for malignancy. In addition to being a minimally invasive surgery, one of the main advantages of this method is the lack of scars, a fact much appreciated by patients and their parents. Generally, gonadectomy is a straightforward operation because the gonads present with an accessible pedicle. Laparoscopic orchidopexy has been standardized and can be performed in patients in whom the testis must be relocated to the scrotum. The removal of ductal structures is also easily performed in most cases, whereas hysterectomy with resection of the vagina may present some difficulties owing to the location of these structures. In patients with a long vaginal component of the urogenital sinus, the distal segment must be removed by a retrograde perineal access, usually performed simultaneously with genitoplasty. Endocrinologists must be aware of the application of this method of treatment in intersex patients, and urologists proficient in laparoscopic techniques must extend their field of work in this area.

Pheochromocytoma treated by laparoscopic surgery.

OBJECTIVE: To evaluate the results of the laparoscopic technique in the treatment of adrenal pheochromocytoma. METHOD: Ten patients, 7 men and 3 women, between 10 and 67 years of age (mean 48) with pheochromocytoma underwent transperitoneal laparoscopic adrenalectomy and were evaluated retrospectively, based on clinical, laboratory, and pathological diagnosis. In all cases there was a solid unilateral adrenal tumor, 5 on the left side and 5 on the right side, whose greater diameter varied from 7 to 80 mm (mean 32). Nine of the 10 patients were chronically hypertensive or had already had hypertensive crises. One patient was normotensive, but presented metabolic alterations suggestive of adrenergic hyperfunction. RESULTS: No deaths occurred in this series. There were two (20%) conversions to open surgery, one due to venous bleeding and one due to the difficulty of dissection behind the vena cava in a patient presenting a partially retro-caval tumor. Surgical time in the 8 non-converted cases ranged from 70 to 215 minutes (mean 136). One patient (10%) received blood transfusion, and another (10%) presented two complications - acute renal failure and a subcutaneous infection. Both had been converted to open surgery. None of the non-converted cases was transfused or presented complications. Hospital discharge occurred between the 2nd and 11th post-operative day (mean 3). The pathological exam of the surgical specimens confirmed the diagnoses of pheochromocytoma in all 10 cases, one of them associated with an aldosterone-producing cortical tumor. CONCLUSIONS: Laparoscopic adrenalectomy for selected patients presenting pheochromocytoma is feasible and provides good results.

[Vesicoureteral reflux in children]. / Refluxo vésico-ureteral em crianças.

The association between vesicoureteral reflux and urinary tract infection represents a significant threat to the kidney, in the form of pyelonephritic scarring. The physiopathologic events that allow the upward migration of bacteria and their inoculation in the renal tissue, causing irreversible damage if not adequately treated, are reasonably understood. Reflux nephropathy is a major cause of childhood hypertension and renal insufficiency. Early diagnosis of reflux, and identification of the pyelonephritic aggression are important for successful therapy. Long-term chemoprophylaxis,to prevent urinary tract infection and renal scarring is the adequate form of treating low-grade vesicoureteric reflux, since spontaneous cure is expected in most of these cases. Although the same approach can be employed in high-grade reflux, surgery is a favorable alternative, with high rates of primary success,reducing the need of long-term medication and the exposure of the kidney to recurring pyelonephritic aggression. The retrograde flow of urine from the bladder to the upper urinary tract is an abnormal situation in the human being, known as vesicoureteral reflux (VUR). It results either from an intrinsic anatomical deficiency of the vesicoureteral junction or from an increased bladder pressure, due to mechanical or dysfunctional vesicourethral obstruction. The recognition of the association between VUR, urinary tract infection (UTI) and renal scars has led to an increased study of this disease in the last two decades.

Transureterocalicostomy: an alternative internal diversion technique.

We report a case of an infiltrating retroperitoneal tumor that completely enveloped and obstructed the mid third of the left ureter. After resection of the tumor, including a 10 cm. long segment of ureter, the upper left ureter was successfully diverted to the upper calix of the right ectopic kidney (transureterocalicostomy). To our knowledge our report represents the first case of such surgery.

Seminal vesicle cyst and ipsilateral renal agenesis.

A case of seminal vesicle cyst with ipsilateral agenesis is presented. The embryologic events, clinical symptoms, and diagnosis are discussed, emphasizing the value of sonography and computer tomography in identifying the cyst. Surgical resection is the treatment of choice.

Retroperitoneal fibrosis: an alternative therapy.

A case of retroperitoneal fibrosis satisfactorily treated with indwelling double J catheters is presented. This method, although reserved for special cases, is an adequate alternative treatment as it allows efficient long-term drainage of kidneys without aggressive surgical dissection of ureters.

Malakoplakia of the urinary tract.

The authors report 4 cases of urinary tract malakoplakia. This rare disease of unknown etiopathogenesis can present with a benign character in the bladder, but when the upper urinary tract is affected, loss of renal function can occur. Treatment aims to control the primary infection, as well as enhance intracellular bactericidal activity, which seems to be compromised in these cases. Cytoreductive surgery may be indicated when this treatment fails.

Steroid therapy in idiopathic retroperitoneal fibrosis: report of two successful cases.

We report 2 patients in whom regression of ureteral obstruction due to recurrent idiopathic retroperitoneal fibrosis was obtained with steroids. Steroids are a valid alternative to surgery in the initial treatment of biopsy-proven benign (idiopathic) retroperitoneal fibrosis.

Prolapsed ureterocele.

Although rare, the prolapsed ureterocele constitutes a urologic emergency that can rapidly evolve to severe conditions due to congestion and necrosis, and to obstruction of the urinary tract. A simple external resection resolves the emergency, allowing a postoperative investigation of the whole urinary tract to be performed, in order to adequately prepare urinary reconstruction.

Keratinizing squamous metaplasia of the upper urinary tract.

We report a case of squamous keratinizing metaplasia of the upper urinary tract. This benign pathology is very rare and difficult do differentiate from other space occupying lesions of the upper urinary tract. Its aetiopathogenesis is still not well understood, but whenever early diagnosis is made, conservative treatment can be employed.

Vesical haemangioma. Report of two cases.

Two cases of vesical haemangioma and a review of the literature are presented. In one of the cases association with cutaneous "Blue Rubber Bleb Nevus" has been found. Vesical haemangioma is a benign tumour, probably of congenital origin, that occurs in young patients. The first symptom is generally haematuria, and diagnosis is confirmed by cystoscopy. The treatment of choice is partial cystectomy, and the most common histologic form is cavernous haemangioma.