Evaluating TIMP-2 and IGFBP-7 as a predictive tool for kidney injury in ureteropelvic junction obstruction.

A major challenge in the management of ureteropelvic junction obstruction (UPJO) is the selection of patients who would benefit from surgical treatment. Tissue inhibitor metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) indicate renal cell stress and are associated with cell cycle arrest. The [TIMP-2] [IGFBP7] ratio (Nephrocheck®) has been recently applied in patients in intensive care units patients to predict the development of acute kidney injury. In this study, we evaluated the performance of these biomarkers performance to distinguishing obstructive hydronephrosis (HN) from non-obstructive HN. MATERIALS AND METHODS: Consecutive patients with UPJO were enrolled in this study. Urinary [TIMP-2] [IGFBP7] and clinical characteristics (hydronephrosis grade, differential renal function, and drainage half-time) were measured in the following groups: 26 children with obstructive HN at initial diagnosis (group 1A) and after six months of dismembered pyeloplasty (group 1B); 22 children with non-obstructive HN (group 2), and 26 children without any urinary tract condition, as the control group (group 3). RESULTS: Comparing the initial samples, [TIMP-2] [IGFBP7] had higher levels in the HN groups and lower levels in the control group; however, no difference was observed between the HN groups (obstructive vs. non-obstructive). After six months of follow-up, patients who underwent dismembered pyeloplasty showed stability in the urinary concentration of [TIMP-2] [IGFBP7]. All patients with [TIMP-2] [IGFBP7] higher than 1.0 (ng/mL)2/1000 had diffuse cortical atrophy on ultrasonography. CONCLUSIONS: We showed that urinary levels of urinary [TIMP-2] [IGFBP7] are higher in children with HN than controls. Nephrocheck® is not reliable in predicting the need for surgical intervention for pediatric patients with UPJO.

Can extended upper pole ureterectomy prevent ureteral stump syndrome after proximal approach for duplex kidneys?

INTRODUCTION: Symptomatic duplex kidneys usually present with recurrent urinary tract infection due to ureteral obstruction (megaureter, ureterocele or ectopic ureter) and/or vesicoureteral reflux. Upper-pole nephrectomy is a widely accepted procedure to correct symptomatic duplex systems with poor functioning moieties, also known as upper or proximal approach. The distal ureteral stump syndrome (DUSS) can be a late complication of this approach. There is no consensus upon the length of ureteral dissection and the better approach to symptomatic disease in duplex systems, so we aim to identify if extended ureteral dissection can prevent DUSS in top-down approach. MATERIALS AND METHODS: Forty-four consecutive patients with symptomatic duplex system were retrospectively classified into two groups: those with limited ureteral excision after heminephrectomy (HN) (group-1) and those with extended ureterectomy after HN (group-2). Patients were followed-up for at least 36 months regarding outcomes of distal ureteral stump. RESULTS: Overall complication was 20%. A total of 8 patients required unplanned further surgery in Group-1 (30%) whereas only 1 patient required unplanned surgery in group 2 (6%) (p=0.07). Subgroup analysis showed that Group-1 presented more DUSS requiring surgery during follow-up than group-2 (p=0.04). Factors possibly affecting complications incidence (such as ureterocele or ectopic ureter) did not differ between groups (p=0.72 and p=0.78). CONCLUSION: Upper pole nephrectomy should be performed with extended distal ureteral dissection to prevent ureteral stump complications.

Patients with encrusted ureteral stents can be treated by a single session combined endourological approach.

PURPOSE: To describe our experience in the management of retained encrusted ureteral stents using a single session combined endourological approach. MATERIALS AND METHODS: Patients with retained encrusted ureteral stents who had been submitted to a single session combined endourological approach from June 2010 to June 2018 were prospectively evaluated. Patients were divided according to the Forgotten-Encrusted-Calcified (FECal) classification. The stone burden, surgical intervention, number of interventions until stone free status, operation time, hospital stay, complications, stone analysis, and stone-free rate were compared between groups. ANOVA was used to compare numerical variables, and the Mann-Whitney or Chi-square test to compare categorical variables between groups. RESULTS: We evaluated 50 patients with a mean follow-up of 2.9±1.4 years (mean±SD). The groups were comparable in terms of age, sex, laterality, BMI, comorbidities, ASA, reason for stent passage, and indwelling time. The stone burden was higher for grades IV and V (p=0.027). Percutaneous nephrolithotomy was the most common procedure (p=0.004) for grades IV and V. The number of procedures until the patients were stone-free was 1.92±1.40, and the hospital stay (4.2±2.5 days), complications (22%), and stone analysis (66% calcium oxalate) were similar between groups. The stone-free rate was lower in grades III to V (60%, 54.5%, and 50%). CONCLUSIONS: The endoscopic combined approach in the supine position is a safe and feasible technique that allows removal of retained and encrusted stents in a single procedure. The FECal classification seems to be useful for surgical planning.

Influence of low free thyroxine on progression of chronic kidney disease.

BACKGROUND: Hypothyroidism is highly prevalent in patients with chronic kidney disease (CKD) and has been associated with poorer clinical outcomes, including faster decline of kidney function. However, there is no consensus whether low free thyroxin (LFT) affects the rate of estimated glomerular filtration rate (eGFR) decline and how the presence of proteinuria influences the progression of renal dysfunction in hypothyroidism. METHODS: We assessed thyroid status, proteinuria, and progression of eGFR by Modification of Diet in Renal Disease equation and CKD-EPI equation in a cohort of CKD patients followed in general nephrology clinics. We estimated the association of LFT levels, and the degree of proteinuria on progression of eGFR. We adjusted for other covariables: age, gender, body mass index, diabetes, hypertension, HbA1c, uric acid, cholesterol, and triglycerides levels.. RESULTS: One thousand six hundred ten patients (64 ± 15 years, 46.8% men, 25.3% diabetic) were included. At beggnining of follow up eGFR was between 45 and 60, 30-45 and 15-30 ml/min/1.73m2 in 479 (29.8%), 551(34.2%), and 580(36.0%) patients, respectively. LFT levels were available at initial evaluation in 288(17.9%) patients and 735(48.5%) had assessment of proteinuria (19.6% with LFT vs. 15.4% without LFT, p = 0.032). Median follow-up time was of 21 months, and 1223(76%) had at least 1 year of follow up. Overall, eGFR decline per month was - 0.05(- 0.26, 0.23) ml/min/1.73m2, reaching 1.7(1.3, 2.4) ml/min/1.73m2 by the end of study period. Similar results were obtained using CKD-EPI. Multivariable mixed linear analysis showed that proteinuria and age were independently associated with eGFR decline, with no effect of LFT, and no interaction between proteinuria and LFT. In patients without proteinuria, there was an improvement of eGFR despite the presence of LFT. CONCLUSIONS: We confirmed a faster rate of eGFR declined in patients with proteinuria. However, despite the pathophysiological rational that hypothyroidism can lead to increased rate of CKD progression, we failed to demonstrate an association between LFT and rate of CKD progression. We conclude that the benefit of hypothyroidism treatment in CKD patients needs to be evaluate in prospective studies.

Clinical Outcomes of the Upper Urinary Tract after Ureteral Clipping for Treatment of Low Functioning or Nonfunctioning Renal Moieties.

PURPOSE: A growing body of evidence suggests that it is safe to ligate the ureter of poorly functioning renal moieties during renal transplantation. We present clinical outcomes and data on hydronephrosis progression in pediatric cases associated with ectopic ureters and obstructive ureteroceles. MATERIALS AND METHODS: We prospectively collected data for 35 consecutive patients (23 females and 12 males) who underwent ureteral clipping between February 2011 and August 2016. Patients were divided into 4 groups consisting of 1) duplex system with ectopic ureter (45.7%), 2) duplex system with a large ureterocele (11.4%), 3) other duplex system (8.6%) and 4) single system kidneys (34.3%). Patients were followed for clinical outcomes and hydronephrosis trends. Comparisons included preoperative and postoperative anteroposterior diameter, maximal ureteral diameter and ureterocele size. RESULTS: Median age at surgery was 59 months (IQR 11 to 120, range 5 to 216). Median ± SD operative time was 108.9 ± 31.1 minutes (range 20 to 180) and median length of stay was 7.5 hours (IQR 6 to 19, range 5 to 336). Immediate resolution of urinary incontinence was observed in all 16 ectopic ureter cases. After a median ± SD followup of 20.8 ± 13.8 months (IQR 8.5 to 30, range 6 to 50) 97.2% of the patients remained asymptomatic. No significant differences were observed between initial and last anteroposterior diameter measurements except in group 1 (p = 0.001). All ureteroceles demonstrated a significant decrease in median ± SD size after clipping (from 2.7 ± 0.41 to 0.53 ± 0.92 cm, p = 0.003). Pyonephrosis developed in 1 patient, who underwent laparoscopic nephrectomy. CONCLUSIONS: Ureteral clipping appears to be a reasonable, safe and effective option for pediatric patients in the reported settings, with the potential to be simpler and quicker than extirpative or reconstructive procedures.

Transperitoneal laparoscopic pyeloplasty in children: does upper urinary tract anomalies affect surgical outcomes?

OBJECTIVE: To assess the feasibility and outcomes of laparoscopic pyeloplasty in children with complex ureteropelvic junction obstruction (UPJO) and compare to children with iso-lated UPJO without associated urinary tract abnormalities. MATERIAL AND METHODS: Medical records of 82 consecutive children submitted to transperitoneal laparoscopic pyeloplasty in a 12-year period were reviewed. Eleven cases were con-sidered complex, consisting of atypical anatomy including horseshoe kidneys in 6 patients, pelvic kidneys in 3 patients, and a duplex collecting system in 2 patients. Patients were di-vided into 2 groups: normal anatomy (group 1) and complex cases (group 2). Demographics, perioperative data, outcomes and complications were recorded and analyzed. RESULTS: Mean age was 8.9 years (0.5-17.9) for group 1 and 5.9 years (0.5-17.2) for group 2, p=0.08. The median operative time was 200 minutes (180-230) for group 1 and 203 minutes (120-300) for group 2, p=0.15. Major complications (Clavien ≥3) were 4 (5.6%) in group 1 and 1 (6.3%) in group 2, p=0.52. No deaths or early postoperative complications such as: urinoma or urinary leakage or bleeding, occurred. The success rate for radiologic improvement and flank pain improvement was comparable between the two groups. Re-garding hydronephrosis, significant improvement was present in 62 patients (93.4%) of group 1 and 10 cases (90.9%) of group 2, p=0.99. The median hospital stay was 4 days (IQR 3-4) for group 1 and 4.8 days (IQR 3-6) for group 2, p=0.27. CONCLUSIONS: Transperitoneal laparoscopic pyeloplasty is feasible and effective for the management of UPJO associated with renal or urinary tract anomalies.

Reoperative Laparoscopic Ureteropelvic Junction Obstruction Repair in Children: Safety and Efficacy of the Technique.

PURPOSE: Failure after pyeloplasty for ureteropelvic junction obstruction in children may occur in up to 10% of cases. Therapeutic options include Double-J® stent placement, endoscopic treatment and reoperation. Laparoscopic and robotic reoperative modalities seem safe and efficacious, although pediatric series are limited in the literature. We report the largest known series of reoperative laparoscopic ureteropelvic junction obstruction repair in children and compare this approach to primary laparoscopic pyeloplasty. MATERIALS AND METHODS: We reviewed all children undergoing laparoscopic pyeloplasty at a single institution from 2004 to 2015. Reoperative laparoscopic ureteropelvic junction obstruction repair was compared to primary pyeloplasty. Groups were analyzed regarding demographics, operative time, complications, length of hospital stay and success, defined by improvement of symptoms, ultrasound and renogram. RESULTS: We identified 11 cases of reoperation (8 redo pyeloplasties and 3 ureterocalycostomies) and 71 primary pyeloplasties. Groups were not different in age, gender or weight. Median followup was 37 months. Median time between primary pyeloplasty and reoperation was 34 months. Median operative time was 205 minutes for the reoperative group and 200 for primary pyeloplasty (p = 0.98). Length of stay was longer in the reoperative group (p = 0.049), although no major complications were recorded in this group. All reoperative cases and 96% of primary pyeloplasty cases remained asymptomatic following surgery (p = 0.99). Postoperative improvement was similar for both groups on ultrasound (90% for reoperation vs 92% for primary pyeloplasty, p = 0.99) and renogram (80% vs 88%, p = 0.6). CONCLUSIONS: Laparoscopy seems to be safe and effective for management of failed pyeloplasty in children. Based on our data, reoperation is as safe and effective as primary pyeloplasty.

One - staged reconstruction of bladder exstrophy in male patients: long - term follow-up outcomes.

INTRODUCTION: The surgical correction of bladder exstrophy remains challenging. In our institution, the repair has evolved from a staged repair to one-stage reconstruction. The one-stage reconstruction includes; bladder closure, Cantwell-Ransley neourethroplasty and abdominoplasty using groin flaps, without the need of pelvic ostheotomies. Repair of urinary continence (UC) and vesicoureteral reflux (VUR) is done after development of the infant. OBJECTIVE: To present our experience of our modified one-stage reconstruction of bladder exstrophy in male patients. MATERIALS AND METHODS: Medical records of male patients submitted to one-stage reconstruction of bladder exstrophy were analyzed retrospectively. Fifteen exstrophy bladder patients with mean age 4.2±7 years were treated at our institution between 1999-2013. RESULTS: Eleven patients were referred to us after previous surgery. Sixteen procedures were performed; one patient had complete wound dehiscence and needed another reconstruction (6.7%). Mean follow up was 10.3±4.5 years. No patient has had a loss of renal function. Postoperative complications: four patients (26.6%) presented small fistulas, one presented penile rotation. Eleven patients (73.3%) patients underwent bladder-neck surgery. Five (33.3%) required bladder augmentation. Three cases (20%) needed subsequent treatment of VUR. At the time of our review nine (60%) patients achieved UC, two (13.3 %) patient without additional procedure. A mean of 3±1.1 procedures (2-5) was accomplished per children. CONCLUSIONS: One-stage reconstruction minimizes the number of surgical procedures required to achieve UC and potentiates bladder-neck function. The advantages of using groin flaps over current techniques for complete repair are the small risk for penile tissue loss and the avoidance of ostheotomies.

Urethral duplication type influences on the complications rate and number of surgical procedures.

INTRODUCTION: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. MATERIAL AND METHODS: We report thirteen boys with urethral duplication managed in our institution between 1988-2015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described. Patients were classified according to Effmann classification. RESULTS: Mean patient's age was 38.3±34.7 months (3-136 months). Mean follow-up was 7.7±3.4 years (3y8m-14y2m). Type II A2 was the most common pattern (8/13 patients, 61.5%), followed by type IA (3/13 patients, 23%) and IIA1 (2/13 patients, 15.3%). The most frequent clinical manifestations were urinary tract infections (UTI) observed in 11/13 patients (84.6%) and anal urinary leakage, found in 7/13 patients (53.8%). Associated anomalies were found in 9/13 patients (69.2%). Required surgeries were 3.53±2.84 procedures per patient. Considering groups: Type IIA2 4.25±3.28, type IIA1 4±1.41 and type IA 1.33±0.57 needed procedures per patient. Complications rate were 0% for type IA, 50% for type IIA1 and 75% for type IIA2. CONCLUSIONS: Patients with incomplete duplication (type I A or I B) can totally be asymptomatic, with no need of surgical correction. Type IIA2 is the most complex form of duplication to correct and multiple procedures might be required because of the very hypoplastic orthotopic dorsal urethral tissue. Surgical treatment should be individualized and parents should be advised on complications and need of multiple surgeries according to urethral duplication type.

Laparoscopic Pyeloplasty in children with Horseshoe Kidney.

INTRODUCTION: Horseshoe kidney occurs in 1 per 400-800 live births and are more frequently observed in males (M:F 2:1). Ureteropelvic junction obstruction (UPJO) is commonly associated with horseshoe kidneys. The variable blood supply, presence of the isthmus and high insertion of the ureter contribute to this problem. CASE REPORT: An asymptomatic 6 year-old boy presented with antenatal hydronephrosis. Ultrasonography and CT scan demonstrated left UPJO associated with a horseshoe kidney. DMSA showed 33% of function on the left side. DTPA showed a flat curve and lack of washout. A left dismembered laparoscopic pyeloplasty was performed after identification of crossing vessels and abnormal implantation of the ureter. After one year, the child is asymptomatic. DTPA demonstrated a good washout curve. RESULTS: Our cohort consisted of six patients, five males and one female, with a mean age of 6 years (range 6m-17 years) and a mean follow-up of 3 years. Ureteropelvic junction obstruction was more common on the left side. Symptoms appeared only in 34% of the cases. Mean operative time was 198 minutes (range 120-270 minutes). Crossing vessels were common (observed in 50% patients). High implantation of ureter was seen in 67% patients and intrinsic obstruction in 83%. Surgical difficulties were found in two cases. Hospital stay was 4.3 days (3 to 6 days), with only one patient having a mild complication (pyelonephritis). All cases had clinical and radiologic improvement. CONCLUSION: Laparoscopic pyeloplasty is safe and feasible in children with UPJO in horseshoe kidneys, with good results and minimal morbidity.

Outcome Analysis of Asymptomatic Lower Pole Stones in Children.

PURPOSE: Lower pole renal stones in children usually present incidentally. To our knowledge frequency and need for intervention are unknown. We evaluated the outcomes of asymptomatic, incidentally found lower pole renal stones in children. MATERIALS AND METHODS: We retrospectively reviewed the charts and renal ultrasounds of children with lower pole stones seen during a period of 14 years. The unpaired t-test was used to determine factors that might predict the need for stone intervention, including size, growth rate, type of stone and underlying metabolic disease. The success rate of nonoperative management, defined by spontaneous passage or lack of growth in the absence of symptoms, was calculated. RESULTS: A total of 224 children were found to have lower pole stones. Mean ± SD age at presentation was 95.2 ± 65.3 months. Of the patients 120 (53.6%) spontaneously passed stones. Of the stones 25% remained asymptomatic and did not grow with time. Intervention was performed in 48 children (21.4%). Stones originally managed conservatively that later required intervention were significantly larger at baseline than stones that did not require intervention through the end of followup (median 7 vs 5 mm, p <0.001) and those that grew with time (median stone size 8 mm at surgical intervention, p = 0.01). CONCLUSIONS: Our data suggest that asymptomatic lower pole renal stones can be followed conservatively. Of the calculi 78.6% passed spontaneously or remained asymptomatic and did not grow. Median initial size greater than 7 mm and stone growth with time were significant risk factors that predicted the need for intervention.

Outcome Analysis of Redo Orchiopexy: Scrotal vs Inguinal.

PURPOSE: Redo orchiopexy after previous surgery is technically challenging and requires skills and care to ensure preservation of cord structures. We report our experience with redo orchiopexy in children. MATERIALS AND METHODS: We retrospectively reviewed patients who had undergone redo orchiopexy between January 2004 and May 2015. Variables evaluated included primary procedure, type of redo procedure, operative time, shift of surgical route, operative and postoperative complications, and testicular location at last followup. RESULTS: A total of 3,384 orchiopexies were performed during the study period, with 61 children (1.8%) requiring redo orchiopexy. Mean ± SD patient age at redo orchiopexy was 6.4 ± 3.6 years (range 1.5 to 17.1) and average followup was 24.9 months (2.1 to 99.6). The primary surgical procedure preceding redo surgery was inguinal orchiopexy in 45.9% of the patients, scrotal orchiopexy in 13.1% and laparoscopy in 13.1%, and 27.9% of patients were status post inguinal surgery (hernia/hydrocele repair). Redo surgery was performed by inguinal approach in 33 patients, while 28 children underwent a scrotal approach. There was no statistical difference in intraoperative and postoperative complication rates for the 2 approaches (p = 0.52 and p = 0.26, respectively). However, there was a statistically significant difference in overall operative time between approaches (p = 0.003) with scrotal orchiopexy being significantly shorter (53.1 minutes) compared to inguinal orchiopexy (84.6). CONCLUSIONS: Scrotal and inguinal orchiopexy appear to be viable in managing secondarily ascending testes, with the scrotal approach offering some advantage in terms of length of procedure.

MMP9 overexpression is associated with good surgical outcome in children with UPJO: Preliminary results.

BACKGROUND: Ureteropelvic junction obstruction (UPJO) diagnosed prenatally occurs in 1:150 - 1:1200 pregnancies. Although many studies investigating the molecular changes of this obstructed segment have been performed, the underlying mechanisms are still unclear. The role of extracellular matrix (ECM) components remains controversial, and the investigations in the field of ECM changes, might help the better understanding of the pathogenesis of this common condition. The aim of the present study was to investigate for the first time in the literature whether MMP9 and its specific inhibitors, TIMP1 and RECK, are expressed in a reproducible, specific pattern in UPJ. METHODS: UPJO specimens were obtained from 16 children at the time of dismembered pyeloplasty due to intrinsic UPJ stenosis. Expression levels of the three genes (MMP9, TIMP1 and RECK) were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Then correlated the expression levels of the genes according to grade study population that was divided in 2 categories according to Society of Fetal Urology classification, grade 3 (moderate) and 4 (severe). For DTPA we subdivided the childrens in 2 groups, obstructive (T 1/2 more than 20 min) and partial obstructive (T 1/2 between 10 and 20 min) and success in a surgery was defined as decrease in T 1/2 to less than 20 min, absence of symptoms, improving renal function and decreasing dilatation on successive exams. RESULTS: MMP9 was underexpressed and TIMP1 and RECK were overexpressed in children with obstructive DTPA but the differences were not statistically significant. Overexpression of MMP9 was higher among patients with severe grade of UPJ compared to those with moderate grade. Surprisingly expression levels of MMP-9 was three times higher in children who were successfully treated by surgery (n = 10) (p = 0.072), so those who were followed for at least 1 year after surgery and remained with improvement in renal function and decreasing dilation on intravenous urogram and TIMP-1 was underexpressed in 100 % of this cases (p = 0.00). CONCLUSIONS: We showed an increase in expression of MMP9 and a decrease in expression of TIMP1 in children who improving renal function and decreasing dilation after surgery. We believe that the higher expression of MMP9 in these cases can reflect an increase in degradation and remodeling process that could be used as a marker for surgical outcome.

Malignant paraganglioma in children treated with embolization prior to surgical excision.

BACKGROUND: Paragangliomas (PGL) are rare tumors derived from neural crest cells, whose origins may vary along the chain of the sympathetic nervous system. Such tumors are often characterized by secretion of catecholamines, but sometimes they are biochemically inactive, which makes diagnosis often challenging. Malignant paraganglioma is defined by the presence of this tumor at sites where chromaffin cells are usually not found or by local invasion of the primary tumor. Recurrence, either regional or metastatic, usually occurs within 5 years of the initial complete resection but long-term recurrence is also described. Malignancy is often linked to a SDHB mutation. Preoperative embolization has been applied in the surgical management of PGLs with the objective to decrease intra-operative blood loss and surgery length without complications. CASE PRESENTATION: We report two cases of patients with abdominal or pelvic malignant PGLs who have been treated surgically at our center after preoperative embolization. Surgery was a very challenging procedure with multiple surgical teams involved and embolization did not prevent major blood loss and intraoperative complications. Patients required adjuvant treatment with either chemotherapy or radiotherapy. CONCLUSIONS: Many studies in the adult population have established recommendations for the diagnosis and therapeutic management of PGL, but few studies concern the pediatric population. Because malignant PGL is more important in the pediatric population, screening and early diagnosis of PGL is advisable in children with genetic predisposing. Surgical resection is the mainstay of treatment, but a multimodal approach is often required due to the complexity of cases.  The role of preoperative embolization is not established and in our experience it has provided little benefit and major complications.

Retroperitoneoscopic pyelolithotomy: a good alternative treatment for renal pelvic calculi in children.

INTRODUCTION: Nephrolitiasis, once considered an adult disease, has become increasingly prevalent in children, with na increase from 6% to 10 % annually in past 25 years. Kidney stones in pediatric population can result from metabolic diseases in up to 50% of children affected. Other factors associated with litiasis are infection, dietary factors, and anatomic malformations of urinary tract. Standard treatment procedures for pediatric population are similar to adult population. Extracorporeal shock wave lithotripsy (ESWL), ureterorenoscopy (URS), percutaneous nepfrolithotomy (PCNL), as well as laparoscopic and retroperitoneoscopic approaches can be indicated in selected cases. The advantages of laparoscopic or retroperitoneoscopic approaches are shorter mean operation time, no trauma of renal parenchyma, lower bleeding risk, and higher stone-free rates, especially in pelvic calculi with extrarenal pelvis, where the stone is removed intact. PATIENT AND METHODS: A 10 year-old girl presented with right abdominal flank pain, macroscopic hematuria,with previous history of urinary infections.. Further investigation showed an 1,5 centimeter calculi in right kidney pelvis. A previous ureterorenoscopy was tried with no success, and a double J catheter was placed. After discussing options, a retroperitoneoscopic pielolithotomy was performed. RESULTS: The procedure occurred with no complications, and the calculi was completely removed. The foley catheter was removed in first postoperative day and she was discharged 2 days after surgery. Double J stent was removed after 2 weeks. CONCLUSIONS: Retroperitoneoscopic pielolithotomy is a feasible and safe procedure in children, with same outcomes of the procedure for adult population.

Novel minimally invasive abdominoplasty for selected cases of prune belly syndrome: Step-by-step technique description and clinical indications.

BACKGROUND: Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, bilateral undescended testicles and genitourinary tract anomalies. A variable spectrum of abdominal wall laxity is observed in PBS. We present the first case of a novel technique using a minimally invasive abdominoplasty to specifically address patients with localized abdominal wall weakness in PBS. CASE PRESENTATION: A two-years-old child with PBS presented with recurrent febrile urinary tract infections. Ultrasonography demonstrated a dysplastic right kidney associated with significant ipsilateral ureterohydronephrosis. Voiding urethrocystogram did not show vesicoureteral reflux and DMSA scan depicted a non-functioning right kidney. During laparoscopic right nephroureterectomy and first stage Fowler-Stephens bilateral orchiopexies, a significant right-sided lateral abdominal wall bulging was observed. A minimally invasive laparoscopic abdominoplasty was performed with a one-way running suture using an unabsorbable 2.0 prolene approximating the edges of the musculofascial defect. While undergoing the second-stage Fowler-Stephens orchiopexy, no bulging was observed. CONCLUSION: A minimally invasive abdominoplasty to improve abdominal wall lateral bulging in PBS was feasible and presented good cosmetic result. We anticipate that this technique can be applied for children with PBS with primary lateral abdominal wall bulging, employing one or more suture lines depending on the fascial defect size.

Bilateral external iliac artery pseudoaneurysms causing urinary obstruction and acute renal failure.

BACKGROUND: We report an exceedingly rare case of bilateral external iliac artery pseudoaneurysms causing urinary obstruction and acute renal failure. CASE PRESENTATION: A young man presented with acute severe bilateral testicular pain radiating to the back. Clinical and radiological workup showed bilateral external iliac artery pseudoaneurysms, which caused bilateral ureterohydronephrosis due to urinary obstruction with subsequent renal failure. Management included immediate bilateral external iliac artery endovascular repair and bilateral ureterolysis using a retroperitoneal approach, with resolution of the obstruction and successful endovascular treatment of both pseudoaneurysms. The only identifiable risk factor for cardiovascular disease was cocaine addiction. CONCLUSIONS: This case highlights an unusual and severe clinical presentation of bilateral EIA pseudoaneurysms causing bilateral ureterohydronephrosis and subsequent renal failure. Awareness of this condition may help avoid misdiagnosis and delayed management, which is of utmost importance for a favorable outcome.

Laparoscopic hidden incision endoscopic surgery (hides) nephrectomy VS. Traditional laparoscopic nephrectomy: Non-inferior surgical outcomes and better cosmetic results.

INTRODUCTION: The benefits of laparoscopic total nephrectomy in pediatric patients are well established. Traditional Ports placement (TPP) still follows Clayman's classic description: except for the umbilical scar, the other laparoscopic scars are exposed in the abdomen. Advances in robotic surgery permitted the development of HidES (hidden incision endoscopic surgery) technique, to obtain a better final cosmetic aspect with the scars located intraumbilically and in the hypogastric region, in an area easily hidden by underwear. As robotic surgery is related to higher costs and lacks availability, a pure laparoscopic HidES technique was developed. OBJECTIVES: 1.Evaluate safety and efficacy of pure laparoscopic HidES.2. Compare HidES with TPP nephrectomy series to assess non-inferiority and cosmetic outcomes. STUDY DESIGN: Twenty-one pediatric patients with symptomatic poor functioning kidneys (DMSA<10%) underwent HidES nephrectomy. Their intra and post-operative outcomes were recorded prospectively. HidES group was compared to an equivalent group of thirty-two patients who underwent TPP nephrectomy. RESULTS: There were no conversions to open surgery in the TPP or HidES groups. There was a significant difference in operative time between HidES (53.4min) and TPP (109.4 min), with p = 0.004 and the mean bleeding volume was 65.5 ml. There was no significant difference in bleeding between HidES (71 ml) and TPP (120 ml) (p = 0.06), no intraoperative complications and no complications above Clavien-Dindo II during the 6-week follow-up. Satisfaction reached 100% in HidES group, whereas in TPP satisfaction was 63% (p = 0.004). DISCUSSION: HidES benefit over TPP is to conceal visible scars above underwear, improving cosmetical outcome. A prospective HidES group was compared to a retrospective TPP database due to decreasing number of nephrectomies being performed, which is a limitation of our study. HidES surgeries were performed by pediatric urologists (associate staff) while TPP group surgeries were performed by PGY-5 urological residents assisted by the associate staff, which is a clear limitation and can explain shorter operative times for HidES. During HidES surgeries an improved visualization of the operative field by the inferior trocar positioning was noted, which helps the posterior dissection of renal pedicle. Previous published study comparing robotic TPP and HidES pyeloplasty showed equal results for both groups with cosmetic advantages. Objective satisfaction was assessed with statistically advantage in favor of HidES procedure, consonant with other evidence that supports the benefit of scar location in patient and parental satisfaction. CONCLUSION: HidES nephrectomy proved to be safe, feasible and not inferior to the traditional nephrectomy in experienced hands, with better cosmesis.

Use of contrast-enhanced ultrasound in pediatrics.

Although contrast-enhanced ultrasound has been shown to provide considerable benefits, particularly in pediatric patients, it is still used relatively rarely in Brazil. It has proven to be a safe technique, and adverse effects are rare. In this review, we address the technique and main applications of contrast-enhanced ultrasound in the pediatric population, including the evaluation of focal liver lesions, abdominal trauma, kidney grafts, liver grafts, bowel loops, and vesicoureteral reflux. It is important for pediatric radiologists to be acquainted with this promising tool, understanding its applications and limitations.

A ultrassonografia com contraste de microbolhas é um método ainda pouco utilizado no Brasil, mas que tem grandes vantagens, ainda mais na faixa pediátrica, como ausência de radiação e dispensa de sedação/anestesia. O contraste de microbolhas tem se mostrado bastante seguro e com raríssimas reações adversas graves. Apresentaremos nesta revisão a técnica para o uso desse contraste na ultrassonografia, bem como as principais aplicações na faixa pediátrica, como avaliação das lesões hepáticas focais, do trauma abdominal, do enxerto hepático e renal nos transplantes, das alças intestinais e da pesquisa de refluxo vesicoureteral. É importante que radiologistas que exerçam funções em centros pediátricos conheçam essa ferramenta promissora.

Urinary extracellular matrix proteins as predictors of the severity of ureteropelvic junction obstruction in children.

INTRODUCTION: Ureteropelvic junction obstruction (UPJO) particularly represents a challenge in regard to management, as not all hydronephrosis (HN) represent a kidney-damaging state. Urinary biomarkers have been proposed as noninvasive tools. Extracellular matrix (ECM) proteins are associated with tissue fibrosis in the setting of UPJO and is poorly explored. OBJECTIVE: To investigate whether urinary ECM proteins are useful to discriminate the severity of urinary obstruction on unilateral UPJO. STUDY DESIGN: Children with unilateral UPJO were prospective enrolled. Urinary (u) matrix metalloproteinases (MMP-1,-2,-9 and TIMP-1,-2) as well as clinical characteristics were measured in the following groups: 26 children with obstructive HN at initial diagnosis and after six months of dismembered pyeloplasty; 22 children with non-obstructive HN at diagnosis and after six months of observation; 26 children without any urinary tract condition, as the control group. Results were assessed statistically using for homogenous groups, a one-way analysis of variances (ANOVA) and for nonparametric groups, Mann-Whitney test or Kruskal-Wallis test was performed. ROC curves were performed. RESULTS: Baseline samples demonstrated a higher concentration of uMMP-1/Cr, uMMP-2/Cr, u-TIMP-1/Cr and u-TIMP-2/Cr in obstructive HN group; uMMP-9/Cr levels were higher in non-obstructive HN group and all studied biomarkers had lower concentrations for the control group. On follow-up, for the obstructive HN group, urinary concentration of uTIMP-1/Cr and uTIMP-2/Cr decreased, and uMMP-1/Cr, uMMP-2/Cr and uMMP-9/Cr increased when comparing preoperative to postoperative values. In the non-obstructive HN group, all proteins analyzed were stable after six months of observation. ROC curves analysis showed a promising diagnostic profile for the detection of obstructive HN for uTIMP-1/Cr (area under the curve -AUC-; of 0.692), uTIMP-2/Cr (AUC of 0.678) and for uMMP-2/Cr (AUC of 0.655). DISCUSSION: The severity of kidney obstruction could correlate with the urinary ECM proteins concentration in this study. This is concordant with prior studies demonstrating that a disruption of the balance of accumulation/degradation of the ECM proteins occur on obstructive uropathy. Limitations of our study include the older age of our patients and that these markers had no influence at all on the surgical decision. CONCLUSION: We demonstrate that obstructive HN have significantly higher uMMP-2, uTIMP-1 and uTIMP-2 concentrations. Particularly, uTIMP-2 levels were correlated to severity of obstruction and therefore, it might be a useful urinary biomarker to correctly allocate children with HN between surgical management vs follow-up. After pyeloplasty, uTIMP-1 and uTIMP-2 presented a progressive decrease postoperatively, which is also highly desirable for urinary markers.