Urological Anomalies Research Articles

Social and Clinical Impact of Congenital Urological Malformations in a Developing Country: The Need for a Transdisciplinary Way of Treatment

Introduction The prognosis of congenital anomalies (CAs) can be improved if detected and treated accurately. Given the complexity of some anomalies, it is almost always necessary to approach them with an interdisciplinary team. Our objective was to contact patients with congenital urological anomalies (CUAs) and follow them up during their first years of life and evaluate their clinical status, as well as their social and health care limitations. Method Based on the Bogota Congenital Malformations Surveillance Program (BCMSP), we have contacted by phone all the patients with CUAs and evaluated their follow-up. We have included all the registered patients from 2006 until 2015. A standardized questionnaire was applied by a trained staff. The questions assessed on each call included: evaluation of the clinical status of the patient, the clinical treatments and evaluations performed by clinical and surgical subspecialties, health care limitations, and social barriers. The first call was made at the 2nd month, then every 3 months during the 1st year and every 6 months thereafter. Results A total of 277 patients were contacted, 97.3% of whom have an increased risk of mortality or significant disability. The malformation related mortality was of 38.1%. Only 38.7% of the patients were evaluated by a specialist, while 57.4% where still waiting to be seen by a specialist. Ninety eight percent of the limitations related to the health care system were the long waiting lists to be seen by a specialist. Conclusion Many of the pathologies that we have found belong to the group that has a significant reduction in mortality when treated accurately and promptly. However, we have a profound problem in our health care system, in that many of the patients have not been seen by a specialist, which results in a worse prognosis and recovery rate.

Sacrococcygeal teratoma type IV presenting with urine retention in infants

Abstract Background Sacrococcygeal teratoma (SCT) is the commonest tumor in neonatal period. The type IV variant is a concealed presacral tumor which presents a diagnostic challenge as symptoms are only apparent due to the mass effects of the tumor. A common presentation is urine retention. We herein present two cases of patients with type IV tumors who presented with acute urinary retention with the aim of highlighting the challenges in diagnosis of the concealed tumor which apart from its malignant potential has serious obstructive effects on the urinary tract that can lead to irreversible kidney damage. Case presentation We report two infants a 2 month old female and an 11 month old female who presented with acute urine retention. Both infants had been previously managed in peripheral facilities by urethral catheterization in an attempt to relieve the obstruction which remained persistent. On assessment at our facility both infants were noted to have a pre-sacral mass that was palpable on DRE and confirmed by CT scan. One infant had associated bilateral hydronephrosis from the obstructive effects of the mass on the urinary system. We used a combined abdominal and posterior sacral approach to resect the tumor in both patients with compete resolution of symptoms. Conclusion SCT type IV presents a diagnostic challenge to the clinician due to its less apparent nature. Patients may present with urologic anomalies due to bladder outlet obstruction and in cases in which diagnosis is delayed, hydronephrosis and renal parenchymal disease may be an associated finding. Clinical assessment in infants presenting with urine retention and constipation of unknown origin should always include a digital rectal examination with particular attention to the posterior rectal wall which can reveal a presacral mass.

The Modified Gil-Vernet Antireflux Surgery: A Successful Technique for High-Grade Vesicoureteral Reflux Correction in Children-Long-Term Follow-Up.

Introduction Vesicoureteral reflux (VUR) is a common urologic anomaly in children. Many techniques have been offered to manage this condition, in which one of them is modified Gil-Vernet antireflux surgery. The study fullfiled to evaluate the efficacy and safety of modified Gil-Vernet antireflux surgery in correction of high-grade VUR. Materials and Methods A retrospective study in which we evaluated efficacy, safety, and complications of modified Gil-Vernet antireflux surgery as a choice procedure for high-grade reflux in all patients who underwent it since 2000 to 2016 at 2 hospitals of Kermanshah University of medical sciences that all of them were done by one surgeon. Results 183 patients with 290 high-grade refluxing units (grade IV or V) were reviewed. 182 refluxing units were grade IV, and 108 units were grade V. There were 76 (41.54%) patients with unilateral and 107 (58.46%) patients with bilateral VUR. Reflux in high-grade group corrected completely in 278 (95.86%) refluxing units and 175 patients (95.62%). Conclusions Our results are remarkable and compatible with other techniques' results. This simple and safe technique can correct bilateral VURs simultaneously; thus, it is rational to be considered for high-grade VUR correction. According to our results, we suggest the modified Gil-Vernet antireflux procedure for high-grade VUR correction as a simple, safe, and successful technique. This trial is registered with 67145/86/1233.

Necessity of performing voiding cystourethrography for children with unilateral multicystic dysplastic kidney.

The purpose of this study was to resolve the clinical question as to whether all patients with unilateral multicystic dysplastic kidney (MCDK) should receive voiding cystourethrography (VCUG). This is a retrospective study using cross-sectional analysis. Seventy-five children with unilateral MCDK were enrolled, excluding patients with other genetic or chromosome abnormalities, spinal cord diseases, or anal atresia. We reviewed their records from medical charts and calculated risk factors for abnormal VCUG using multivariate logistic regression analysis. Abnormal VCUG findings were present in 24 of 75 patients (32.0%), specifically, vesicoureteral reflux (VUR) in 8 (10.6%), including high-grade VUR in 2 (2.7%), and only lower urinary tract or bladder disease in 16 (21.3%). In multivariate analysis, only abnormal findings by ultrasonography was an independent risk factor for abnormal VCUG findings with statistical significance in multivariate analysis (OR 6.57; 95% CI 1.99-26.26; P = 0.002). When we excluded five patients who showed similar findings by ultrasonography and VCUG, abnormal findings by ultrasonography were again calculated as an independent risk factor (OR 4.44; 95% CI 1.26-28.42; P = 0.02). Sensitivity, specificity, positive predictive value, and negative predictive value of abnormal findings by ultrasonography to predict urologic anomalies by VCUG in these children were 83%, 59%, 49%, and 88%, respectively. Two children required a third ultrasonography to detect abnormal findings. We can select, using only abnormal findings by ultrasonography, children with unilateral MCDK who should undergo VCUG. We would also like to emphasize that ultrasonography should be performed repeatedly to detect congenital anomalies of the urinary tract.

A Rare Case of Triplicate Ureter

Ureter triplication is an extremely rare congenital anomaly of the urinary system with approximately 100 cases reported in medical literature, since it was first described in 1870. It is classified into 4 types, and commonly associated with ipsilateral or contralateral urological anomalies. In this report, we present a case of a pediatric Type III-triplicate ureter associated with ipsilateral vesicoureteral reflux and contralateral idiopathic renal atrophy that was discovered during kidney transplant work-up. Diagnosis was made using multislice computed tomography and was confirmed intra-operatively.

GENETICS OF VESICOURETERAL REFLUX

Primary vesicoureteral reflux (VUR) is known to be the most common urological anomaly in children, witch affects 1-2% of pediatric population and 30-40% of children, developing urinary tract infections (UTIs). The hereditary and familial nature of VUR is well established and in several studies was proved that siblings of children with VUR have a higher incidence of reflux than it is in general pediatric population. Familial clustering of VUR implies that genetic factors have an important role in its pathogenesis, but to date no single major locus or gene for VUR has been identified and most researchers acknowledge that VUR is genetically heterogeneous. Improvements in genome wide scan techniques and continuously increasing knowledge of the genetic basis of VUR should lead us to further insights on VUR pathogenesis.

Clinical features of children with multicystic dysplastic kidney.

To evaluate the clinical features of patients with multicystic dysplastic kidney (MCDK). The medical files of children diagnosed with MCDK between January 2008 and November 2015 were retrospectively reviewed. The demographic, clinical, laboratory and radiological data were evaluated. Of 128 children with MCDK enrolled in the study, 82 (64.1%) were male, and 46 (35.9%) were female (P< 0.05). MCDK were located on left and right sides in 66 (51.6%) and 62 children (48.4%), respectively (P > 0.05). Antenatal diagnosis was present in 64 patients (50%). The mean age at diagnosis was 2.8 ± 2.7years (range, 0-8years), and follow-up duration was 4.5years. Fifteen patients (20.8%) had vesicoureteral reflux. Of these, four underwent endoscopic surgical correction. Other associated urological anomalies were ureteropelvic junction obstruction (n = 6), hypospadias (n = 1), and kidney stones (n = 1). On technetium-99m dimercaptosuccinic acid scintigraphy, which was performed in all patients, no significant association between grade of reflux and presence of scarring was seen. Hypertension was diagnosed only in one child (0.8%) who required antihypertensive treatment. The prevalence of unilateral undescended testicle in children aged <1year in the 82 male patients was 4.9%. Seventy-six patients (59.4%) developed compensatory hypertrophy in the contralateral kidney during a 1year follow-up period. Of the total, only seven children (5.5%) had undergone nephrectomy. MCDK follows a benign course with relatively few sequelae, and therefore these patients should be closely followed up and conservatively managed.

Characteristics of urinary tract infections in children up to 7 years of age

&amp;lt;p&amp;gt;Urinary tract infection (UTI) is defined as a presence of significant bacteriuria in a properly collected urine sample, followed by various clinical manifestations - from asymptomatic bacteriuria to acute pyelonephritis. It is most common in infancy and particularly in females. There is a higher risk of UTI in children with underlying urological anomalies, voiding dysfunction and constipation. The subsequent long-term complications such as hypertension and chronic renal insufficiency might arise in children with recurrent pyelonephritis due to renal scarring. The early and proper diagnosis of UTI needs to be based on the recognition of clinical signs and symptoms of UTI, microscopic examination of urine sediment, urine culture and radiological findings. The objective of various medical imaging studies, including kidney and bladder ultrasound, voiding cystourethrography and technetium-99m-dimercaptosuccinic acid renal scintigraphy, is to identify urinary tract abnormalities, as well as risk factors for recurrent infections. The UTI that has been diagnosed requires a 7-14-day course of antimicrobial therapy in symptomatic and especially febrile children. In most children, the early diagnosis and treatment of recurrent UTI is of utmost importance. In this way the development of permanent kidney damage, as well as permanent consequences will be prevented.&amp;lt;/p&amp;gt;

The causative microorganisms and the underlying urological anomalies in children with urinary tract infection

Background: Urinary tract infection (UTI) is among the most common bacterial infection in children and may be a presentation of variety of underlying urinary tract abnormalities. It is important to diagnose and treat UTI to prevent the long-term complications like renal scarring, hypertension and chronic renal failure. The aim of this study was to find the causative organism and underlying urological anomalies in children with UTI.Methods: A prospective study was carried in children (aged between one month to 12 years) admitted with symptomatic UTI in a tertiary hospital from May 2013 to June 2017.The children were investigated for UTI and the urine culture was positive in 121 patients. The confirmed cases of UTI were subjected to imaging studies as per guidelines. Final study included 116 patients as five patients were excluded due to incomplete investigations.Results: Out of 116 patients, 26 (22.41%) were below 1year ,68 (58.62%) were between 1 to 5 year and 22 (18.96%) were more than 5 years of age. There were 44 (37.93%) males and 72 (62.06%) females. E. coli was the commonest organism causing UTI (78.44%). Urinary tract abnormalities were found in 49 (42.24%) cases. VUR was detected in 23 (46.9%). The other urinary tract abnormalities observed include pelviureteric junction (PUJ) obstruction, posterior urethral valve, urolithiasis, bladder neck obstruction, ureterocele, ectopic kidney, cystic kidney, bladder diverticulum, Extrarenal pelvis, and renal scar respectively.Conclusions: In view of underlying urological anomalies and potential for renal parenchymal damage, UTI should be diagnosed at an earliest and imaging studies should be done in all confirmed cases as per guidelines.

Case 253: Thrombosed Umbilical Venous Varix in an Infant

History A 3-month-old previously healthy girl presented to an outside institution with a 4-day history of low-grade fever, irritability, and a tender "knot" in the upper abdomen. Ultrasonography (US) was performed at an outside hospital. US images were not available for review; however, they showed a mass in the left hepatic lobe, per the outside report, and the patient was referred to our institution for further evaluation. Her parents reported a normal full-term pregnancy, with regular prenatal care and normal prenatal US findings. The baby was born after an uncomplicated gestation. She was delivered at term via an uncomplicated cesarean section due to a maternal history of cesarean section. The perinatal course was uncomplicated, and there was no history of umbilical catheterization, per the parents. On arrival at our institution, the patient had a temperature of 38.2°C. All other vital signs were normal. Palpation revealed a tender and firm mass in the periumbilical region; otherwise, physical examination findings were normal. Results of laboratory work-up were normal, except for elevated white blood cell count (26 600/mm3 [26.6 × 109/L]; normal, 6000-17 500/mm3 [6-17.5 × 109/L]). The patient underwent US followed by intravenous contrast material-enhanced (10 mL ioversol, Optiray 320; Medtronic, Santa Rosa, Calif) computed tomography (CT) on the same day.

V09-05 SIX-WEEK-OLD INFANT ROBOTIC PYELOPLASTY: KEY POINTS FOR SAFETY AND PROFICIENCY

You have accessJournal of UrologyPediatric Urology1 Apr 2018V09-05 SIX-WEEK-OLD INFANT ROBOTIC PYELOPLASTY: KEY POINTS FOR SAFETY AND PROFICIENCY Maria Veronica Rodriguez, Nimrod Barashi, Joseph Rodriguez, and Mohan S Gundeti Maria Veronica RodriguezMaria Veronica Rodriguez More articles by this author , Nimrod BarashiNimrod Barashi More articles by this author , Joseph RodriguezJoseph Rodriguez More articles by this author , and Mohan S GundetiMohan S Gundeti More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2018.02.2177AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Ureteropelvic junction obstruction (UPJO) is one of the most common pediatric urologic congenital anomalies. Anderson-Hynes dismembered pyeloplasty has been the gold standard for the intervention of this pathology. Nowadays, robotic surgery has provided a safe alternative to manage these patients with minimally invasive surgery and, even though the procedure is frequently performed by pediatric urologists across the US, there is still a steep learning curve to perform infant robotic pyeloplasty. We aim to describe a robotic-assisted laparoscopic pyeloplasty in a 6-week-old patient with grade 4 hydronephrosis and UPJO in addition to key technical points for safety and proficiency. METHODS The crucial points are patient positioning, proper port placement; particularly the inferior working ports, given the close proximity to the bladder, instrumentation and docking and the inherent bowel distension that children usually have. We present a robotic pyeloplasty in a 6-week old infant with UPJO. The patient was placed in a lateral position, close to the edge of the table. With an open Hassan technique approach, a 12-mm umbilical camera port and two 8-mm ports were placed in the midline in addition to a 5-mm assistant port that was positioned on the lower midclavicular line. There was a distended bowel, therefore an orogastric tube was placed to improve visualization and avoid bowel injury. The Gerota's fascia was incised and the pelvis was hitched with a subcutaneous suture. Then, the narrow ureteropelvic junction was recognized and the pelvis was transected just below the renal hilum vessels, which was followed by ureter spatulation. Ultimately, the anastomosis of the posterior wall with the spatulated ureter was performed in a continuous fashion and subsequently, a double J stent was placed percutaneously to complete the anterior wall anastomosis. RESULTS The patient tolerated the procedure well and was discharged on postoperative day 2 without any complications. Operative time was 143 minutes and, estimated blood loss was found to be less than 5 mL. The Foley catheter was removed prior to discharge. At 1 month follow-up, the patient had well healed abdominal surgical incisions, the double J stent was removed and, his renal bladder ultrasound scan showed improvement of the hydronephrosis. CONCLUSIONS We emphasize the key points for safety and proficiency when performing a robotic infant pyeloplasty, given our long-term institutional experience doing robotic surgery in such young patients to optimize outcomes and prevent complications. © 2018FiguresReferencesRelatedDetails Volume 199Issue 4SApril 2018Page: e904 Advertisement Copyright & Permissions© 2018MetricsAuthor Information Maria Veronica Rodriguez More articles by this author Nimrod Barashi More articles by this author Joseph Rodriguez More articles by this author Mohan S Gundeti More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Crossed Fused Ectopia of Kidney – An Account of Tertiary Healthcare Center Experience

Background: Crossed renal ectopia with fusion is the abnormal migration of the kidney to the opposite side of the insertion of the ureter to the bladder. It is the second most common congenital anomaly of the kidney and urinary tract preceded by horseshoe kidney. The following article serves to highlight the anomaly in a conglomeration of 17 unique cases, managed in our tertiary care centre over a period of 5 years. Materials and Methods: This is a descriptive study analysing the demographic features and the management of seventeen patients diagnosed with crossed renal ectopia with fusion during the period January 2012 to January 2017. Radiological modalities of investigation were modified as per the anomaly. The management plan was devised keeping in mind, the essence of preserving the functional unit. Results: Of the seventeen cases, nine patients were symptomatic and eight were asymptomatic. The most recurring crossed renal ectopia with fusion was L- shaped (n=6), disc shaped (n=6) sigmoid shaped (n=2), inferior (n=1), cake shaped (n=1) and superior ectopia (n=1). Left to right ectopia (n=9) was more common than right to left (n=8). Three patients had a solitary crossed ectopia. Out of nine symptomatic patients, six patients underwent surgical procedures, the remaining three were treated conservatively and advised regular biannual follow-up with imaging. Conclusion: The management of crossed renal ectopia with fusion is individualised according to the underlying urological anomaly and its sequelae. Importance is given to preserve the renal function whenever possible. Reconstructive surgeries like pyeloplasty, ureteric reimplantation, boari flap can salvage some function in these units.

Can sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation?

Background and aimThe sacral ratio (SR) is a well-established tool to quantify sacral development in patients with anorectal malformations (ARM) and can be used as a predictor of fecal continence. We hypothesized that a lower SR correlated with the presence of urologic and renal malformations. MethodsWe retrospectively reviewed the medical records of patients with ARM treated at our center from 2014 to 2016. We measured the lateral SR as a marker for sacral development and assessed the spine for the presence of tethered cord (TC). Urological and renal anomalies, including single kidney, hydronephrosis, hypospadias, vesicoureteral reflux (VUR), ectopic ureter, and penoscrotal transposition were assessed. Analysis of variance (ANOVA), t-tests, and multivariable linear regression were used to test for differences in SR with consideration of associated urologic malformations and tethered cord. Results283 patients with ARM were included for analysis (156 females). The median age was 39months (10–90). Among these, 178 (55.6%) had 1 or more urologic malformations, and 81 (25.3%) had a TC. Hydronephrosis, high-grade VUR (3–5), solitary kidney, and tethered cord were significantly associated with lower SR (p<0.01). In multivariable regression models, the presence of urologic abnormalities remained significantly associated with lower a SR despite the presence or absence of TC (p<0.001). ConclusionSR is a potentially useful indicator of certain urologic anomalies including hydronephrosis, high grade VUR, and solitary kidney in patients with ARM. This association is independent of the presence of TC. A sacral ratio as a part of the VACTERL screening can help the surgeon identify which patients need closer urologic follow up. Level of evidenceIV

Prediction of vesicoureteral reflux in children with febrile urinary tract infection using relative uptake and cortical defect in DMSA scan

Vesicoureteral reflux (VUR) is an important urologic anomaly that causes renal injury in children with febrile urinary tract infection (UTI). The present study aimed to evaluate the associations of abnormalities detected on technetium-99m-labeled dimercaptosuccinic acid (DMSA) scans, focusing on the association with VUR of the levels of relative decrease in kidney function and cortical defects after a first febrile UTI in children. All 171 children underwent ultrasonography, DMSA scan and voiding cystourethrography (VCUG). The features of ultrasound and DMSA scans were compared between patients with (n=48) and without VUR (n=123). The relative uptake (RU) by each kidney was derived from the absolute value of the differences between the value for RU of radionuclide in the right kidney and that in the left kidney. The extent of cortical defects (ECD) was graded according to the number of compartments that contained cortical defect in both kidneys (right upper/right lower, left upper/left lower). Receiver operating characteristic curves were constructed to examine the diagnostic value of these parameters of ultrasound and DMSA scans for predicting VUR. The ratio of patients having hydronephrosis on ultrasound or cortical defects on DMSA scan did not differ significantly between VUR and non-VUR groups. However, the absolute values of the RU and the scores for ECD were significantly higher in the VUR group than in the non-VUR group. The area under the curves for these two parameters were higher than those for the presence of hydronephrosis or the presence of cortical defects or both. Decreased relative function and increased extents of cortical defects on DMSA scan may be associated with the presence of VUR. These findings may assist pediatricians to decide whether febrile UTI children need to undergo VCUG.

Adolescent varicocele: Efficacy of indication-to-treat protocol and proposal of a grading system for postoperative hydroceles

Varicocele is a common urologic anomaly in adolescent males; however, evidence-based treatment guidelines do not exist. Hydroceles are known to be a common complication after surgical therapy, with a wide variation in the reported incidence between 1 and 40%. This study aimed to introduce a standardized indication-to-treat protocol and prove its efficacy by analyzing the outcome of patients. Secondly, it aimed to better define postoperative hydroceles because the wide variation of reported incidence is attributed to a lack of definition. Our standardized treatment protocol included an initial assessment with clinical grading of varicoceles, ultrasound evaluation of testicular volume, and calculation of the atrophy index. Indications for surgical treatment were testicular volume asymmetry >20%, discomfort and pain, or bilateral varicocele. The Palomo procedure (laparoscopically since 2005) was the standard procedure. Postoperative hydroceles were graded according to clinical findings and symptoms: Grade I, sonographic chance finding without clinical correlate; Grade II, palpable but clinically insignificant; Grade III, symptomatic. All patients treated according to the defined protocol were prospectively monitored between January 2001 and December 2015. A total of 129 patients with left varicocele were referred to our institution; 70 fulfilled the indication criteria for surgical treatment. Twenty-eight of these patients were treated for volume asymmetry, 26 of these showed catch-up growth. Forty-two patients were treated for discomfort and pain; the symptoms subsided in all of them. Postoperative hydroceles were detected in 36 patients (51%). In 29 patients this was a sonographic chance finding (Grade I). Three patients showed a palpable but clinically insignificant postoperative hydrocele (Grade II) and four patients (5.7%) showed symptomatic hydrocele (Grade III) where treatment was recommended. The treatment protocol allowed judicious indication for surgery and postoperative outcomes similar to previous reports. The high rate of catch-up growth in operated cases represents a proxy for successful treatment in cases where more precise parameters, like semen quality or paternity rate, were not yet detectable. The introduced grading system for postoperative hydroceles provs to be a valid and appropriate instrument, and promises to be a standardized method for comparing outcomes in future studies. The indication-to-treat protocol proved to be easily applicable, highly efficient, and have outcomes comparable to international literature. The necessity for a standardized grading of postoperative hydroceles was underscored in the data.

Ureteral Triplication Associated With Upper Pole Ureteropelvic Junction Stenosis

Triplication of ureter (TU) is a rare but important disease occurring in children. TU occurs with the early division of the ureteric bud or with three buds arising from mesonephric duct. It is commonly associated with other urological anomalies such as contralateral ureteric duplication, ureteral ectopia and renal dysplasia. Our case is a 3-year-old girl who presented with right triplex ureters and ureteropelvic junction (UPJ) obstruction of the upper pole. Diagnostic tools used in our case study were ultrasound (US), retrograde pyelography and CT urography for imaging. CT urography is a useful diagnostic tool for pediatric patients because it gives more information about anatomical details. J Med Cases. 2018;9(7):195-197 doi: https://doi.org/10.14740/jmc3060w

Stented versus stentless open pyeloplasty in children

Objective The aim of this study was to compare between stenting and nonstenting in dismembered pyeloplasty as regards outcome, early and late postoperative results, and complications. Background Ureteropelvic junction obstruction is a common congenital urologic anomaly. Open pyeloplasty is the standard surgical treatment. Drainage following pyeloplasty using a double J stent prevents subsequent stenosis and urine leakage. Patients and methods This study was conducted on 20 pediatric patients (maximum age 16 years) with a primary diagnosis of ureteropelvic junction obstruction who were divided into two groups: Group 1, the stented group double J (10 cases). Group 2, the nonstented group (10 cases). Classic Anderson–Hynes pyeloplasty was performed for the two groups. Results A total of 20 patients were included in the study. 16 (80%) cases were male and four (20%) cases were female. Their ages ranged from 6 months to 15 years. The mean ± SD age was 6.75 ± 4.53 years. All cases were unilateral; 10 (50%) cases were right sided and 10 (50%) cases were left sided. Postoperative complications included the following: urinary tract infection in only one case in group 1, residual mild hydronephrosis in only one case in group 1 and three (30%) cases in group 2; clot formation in only one case in group 2, and urinoma in only one case in group 2. Conclusion Pyeloplasty with a diverting stent is technically feasible and safe. Only risks for multiple anesthesias and economic burden with stent removal are present. The specific indication when stented pyeloplasty needs to be performed is in redo pyeloplasty, solitary kidney, inflamed renal pelvis, and hugely distended renal pelvis.

Molecular genetics of inherited kidney disease in Saudi Arabia

Human kidneys serve important physiological functions in the body. There is an increasing evidence suggesting that the majority of renal diseases have an underlying genetic component. At least 40 genes have been shown to be involved in kidney development and many more genes are expressed within the kidney and regulate renal physiology. It is observed that genetic and congenital disorders are more common in Arab countries than in industrialized countries. Saudi Arabia is the largest Arab country and estimation of patients with end-stage kidney failure is 136 for each million yearly. Preliminary observations indicate that children in Saudi Arabia probably have a higher incidence of polycystic kidney disease, familial juvenile nephronophthisis, congenital urological anomalies, and familial nephrotic syndrome. Molecular diagnosis would help enormously in prevention and introduce early management which could ensure a better outcome. This is a review article of molecular studies conducted in the Kingdom of Saudi Arabia from 1990 till present time to elucidate disease-causing genes of inherited kidney disease.

An Unusual Case of Low Transverse Vaginal Septum

Background: The complete or imperforate transverse vaginal septum is a rare anomaly of the female reproductive tract. Its site varies in the vaginal canal. The common sites are upper and middle third of the vagina, the low transverse vaginal septum is very rare. It is found in association with urological anomalies and anorectal malformation as well as other Mullerian anomalies especially bicornuate uterus. Case Report: We report a case of a 13-year-old girl with hematometra, hematocolpos and hematosalpinx due to an imperforate complete low vaginal septum. Conclusion: Hematosalpinx presence adversely affects the fertility and obstetric outcomes in the patient. MRI is important diagnostic modality that helps in deciding the management and so as predicting future outcome.

Laparoscopic heminephrureterectomy for duplex kidney in children

Duplication of the upper urinary tract is one of the most common congenital urological anomalies. In patients with critically decreased or lost function of one of the renal segments, heminephrureterectomy is usually the treatment of choice. Until recently, this was an open surgery; in cases of complete removal of the ureter, an additional incision in the iliac region was required. Currently, heminephrureterectomy is increasingly performed laparoscopically. We report the experience in laparoscopic heminephrureterectomy (LHNUE) in 10 clinics in Russia and Belarus. Some of them have already used this technique for 10 years. The study aimed to to improve the treatment results in children with urodynamic dysfunction due to duplicated upper urinary tract. We retrospectively analyzed medical records of 111 children treated from 2007 to 2016. There were 26 (23.4%) boys and 85 (76.6%) girls with mean age 44.6 months (from 2 to 170) at the time of surgery. All children included in the study had complete duplex kidneys, including 51 (45.9%) right-sided and 60 (54.1%) left-sided. All the children underwent LHNUE for a critical decrease or absence of function of the upper or lower segment of the duplex kidney caused by the following pathology: obstruction of the ureterovesical junction with the development of the megaureter of the upper ureteral segment in 57 (51.4%) patients; ureterocele in 28 (25.2%); extra-vesical ectopic ureter with urinary incontinence in 10 (9.0%) girls; high-grade UVR in 16 (14.4%) patients. There were no conversions in this series of patients. The mean operative time was 135 minutes (60-240 min.). All children included in the study were followed for 1 to 9 years after surgery. Complications occurred in 17 (15.3%) patients, of whom 12 (10.8%) required repeat surgery. In one patient with the loss of lower pole function, the treatment result was considered unsatisfactory. LHNUE for duplex kidney is performed by a few clinics and is still at the stage of development and accumulation of experience. Nevertheless, LHNUE, though an effective treatment modality, carries the risk of reducing or losing the function of the retained segment.