Search

Objective Vesicoureteral reflux (VUR) is a common finding in the pediatric population with the risk of repeated infections and renal damage. There is little is known about the natural history of primary bilateral high-grade reflux. Herein we present our experience in the management of primary high-grade bilateral VUR and the long-term outcome of renal function in this specific group of patients. Materials and methods We retrospectively evaluated all patients with congenital bilateral VUR between 2006 and 2014. Records were reviewed for patient age at diagnosis, antenatal history, clinical presentation, the grade of VUR on voiding cystourethrogram (VCUG), presence of scars on dimercaptosuccinic acid (DMSA) scan, indications for surgical intervention, and surgical approaches. Clinical and radiological outcomes of this subgroup of patients were assessed. Results A total of 67 patients with bilateral VUR were identified, of whom 31 (20 boys and 11 girls) had primary high-grade (grade IV and V) bilateral VUR. The mean age at diagnosis was seven months. DMSA scans showed renal scars in 19 patients (61%) and eight of them were bilateral. Surgical intervention was necessary for 81% of patients with a success rate of 58% after endoscopic correction and 100% after reimplantation. Chronic kidney disease (CKD) developed in 13 patients (42%) after a mean follow-up of eight years. Conclusions Primary bilateral high-grade VUR carries a high rate of surgical intervention. The endoscopic correction has an acceptable success rate and efficient long-term outcome. Nevertheless, a significant proportion of patients progresses to CKD even after VUR management.

Summary Introduction and objective Ureteropelvic junction obstruction (UPJO) is a common pathology in pediatric urology practice. Indications for intervention are well established while the ideal management of children with UPJO in poorly functioning kidneys is still debatable. We aimed in this study to evaluate the outcome of pyeloplasty in patients with UPJO and low DRF ≤20%. Study design We retrospectively evaluated 218 children with congenital UPJO from two tertiary hospitals between 2008 and 2018. We included only those with primary unilateral UPJO and DRF ≤20% on diuretic renography. Open dismembered pyeloplasty with stenting was carried out in all. Patients with bilateral UPJO, solitary kidney, DRF above 20%, association with other urinary anomalies and patients who underwent previous renal or ureteric surgeries and patients with missed follow-up were excluded. Clinical and radiological outcomes of this subgroup of patients were assessed. Results A total of 21 patients (12 boys, 9 girls) with a median age at surgery of 2 years were included. Preoperative DRF ranged from 8 to 20% with a mean of 15% ± 3.5%. The median follow-up period was 2.5 years. Renal ultrasound showed improved HN in 16 patients (4 had a complete resolution) and stable in the remaining 5. Most of the improvement was noted in the first postoperative ultrasonography 16/21 (76%). Diuretic renography showed improved DRF by 10–15% in 8 patients (38%) while the remaining patients showed stable DRF with a non-obstructive curve with a mean improvement in DRF of 8 ± 5%. No postoperative complications were encountered and none of our patients required reintervention during follow-up. Discussion The ideal management of children with UPJO and poorly functioning kidneys is still arguable. In our study we evaluated the outcome of pyeloplasty in patients with a low DRF ≤ 20% aiming to test the patients’ variables that can affect the improvement of DRF and HN grade after surgical intervention, however, none of these preoperative variables seemed to be a significant predictor. Conclusions Children with unilateral UPJO and DRF ≤ 20% have a favorable outcome after pyeloplasty. Improvement of HN in the first postoperative renal ultrasound is a good indicator for success.

Background Crossed fused renal ectopia (CFRE) is a rare congenital malformation. Renal bladder ultrasound (RBUS) is a good tool for establishing the diagnosis, however, additional imaging with a voiding cystourethrogram (VCUG) and dimercaptosuccinic acid scan (DMSA) might be required. We assessed the need for postnatal evaluation and the long-term consequences in patients with this diagnosis. Methods A retrospective review of the records of all patients diagnosed with CFRE between 2004 and 2014 was done. We included all patients who underwent postnatal evaluation with RBUS, DMSA and VCUG. Long-term radiological and functional outcomes were assessed. Results A total of 29 patients with CFRE were identified. Majority of cases were detected antenatally (79%) and left to right crossed ectopia constituted the majority of cases (65%). RBUS revealed associated hydronephrosis (HN) in 11 patients (38%). DMSA scans confirmed the diagnosis in all patients and showed impaired renal function in 10 ectopic kidneys (34%). Vesicoureteral reflux was detected in 12 patients (41%); one third of them needed surgical intervention. Extra-urinary anomalies were present in 14 patients (48%) mainly of cardiac origin. After a mean follow-up of 4.5 years, 4 patients (14%) with extra-urinary anomalies developed chronic kidney disease and two of them died. Conclusions Crossed fused renal ectopia is commonly associated with both urinary and extraurinary malformations. Postnatal RBUS is usually sufficient for diagnosis, however, additional imaging may aid for confirming the diagnosis and detection of associated urinary anomalies. Presence of extra-urinary malformations with crossed fused ectopia carries a higher risk of morbidity. Type of the Study Retrospective study [diagnostic/prognostic study]. Level of Evidence Level IV.

Purpose: The aim is to assess the outcomes of different approaches for the management of renal stones associated with horseshoe kidneys (HSKs) in our institution over a 12-year period. Methods: A retrospective review of 144 patients with HSKs who presented from 2000 to 2012 was performed. Twenty-eight patients (19.4%) were found to have renal stones. Demographic data were collected; the method of treatment and the outcomes of stone management were reviewed. We excluded patients with non-functioning moieties and associated genitourinary anomalies, and those with incomplete data. Results: We included 25 patients, of which 16 males (64%) and 9 females (36%), with a mean age of 37 years. Mean serum creatinine level was 66 mmol/L. Eleven patients with a stone size 2 cm were treated with percutaneous nephrolithotomy. One patient with a 10 mm stone was treated using flexible ureteroscopy. No significant perioperative complications were encountered. Conclusions: Indications, methods of treatment, and outcomes of management of stones associated with HSKs were comparable to those for stones associated with normal kidneys. Tailored approach based on stone size is highly recommended. ESWL accompanied with ureteric stenting is a promising strategy for the management of stones associated with HSKs in selected patients requiring intervention.

Introduction: Fetal hydronephrosis (HN) occurs in around 5% of pregnancies and its prognosis depends mainly on the grade of the dilation.We attempted to determine the fate of isolated, unilateral, high-grade HN in children with antenatal diagnosis, emphasizing the risk factors for progression.Methods: We retrospectively evaluated 424 children (690 kidney units) with antenatal HN in the period between 2010 and 2014. We included only those patients with isolated high-grade HN (Society for Fetal Urology [SFU] Grade 3 or 4). Patients with bilateral HN or unilateral HN associated with dilated ureter or reflux and patients with missed followup were excluded. The prognosis of HN (whether improved, stabilized, or progressed) and the need for surgical intervention in this subset of patients was evaluated.Results: A total of 44 children (34 boys and 10 girls) were identified. Ultrasounds showed SFU Grade 3 HN in 24 (54%) and SFU Grade 4 HN in 20 (46%). After a mean followup of three years (range 1‒5), 10 children (23%) needed surgical intervention; four Grade 3 HN (16%) and six Grade 4 HN (30%). The majority of children with differential renal function (DRF) ≥40% (69.5%) were stable or improved. Five girls (50%) and five boys (17%) progressed and required surgical intervention. No patient with a renal pelvis anteroposterior diameter (APD)

Objective To assess the need for postnatal evaluation and the long-term consequences in patients with prenatal diagnosis of unilateral renal agenesis (URA). Materials and Methods A retrospective review of the records of all patients with prenatal diagnosis of URA between 2004 and 2013 was done. We included all patients who underwent postnatal evaluation with renal bladder ultrasound (RBUS), dimercaptosuccinic acid scan, and a voiding cystourethrogram. Long-term follow-up included physical examination and assessment of contralateral renal growth. Results A total of 46 children (24 boys and 22 girls) with URA were identified. Ultrasounds showed associated hydronephrosis in 9 patients (19%). Dimercaptosuccinic acid scans confirmed the diagnosis of URA in all patients and showed renal scars in 2 solitary kidneys (4%). Vesicoureteral reflux was diagnosed in 4 patients (12%); all were of low grade and managed conservatively. After a mean follow up of 4 years, RBUS showed compensatory hypertrophy in 31 patients (67%), 3 patients (6%) developed chronic kidney disease, and 2 patients (4%) developed hypertension. Conclusion Prenatally detected cases of URA should be promptly evaluated. RBUS is found to be sufficient for diagnosis. However, additional imaging may be indicated for confirming the diagnosis and detection of other associated urinary anomalies. Serial assessment of the contralateral solitary kidney growth and serum creatinine measurement will be helpful to assess kidney function on the long term.

Background— Aortopulmonary collaterals (APCs) have been associated with increased morbidity after the Fontan operation. We aimed to quantify APC flow after bidirectional cavopulmonary connections and Fontan completions, using phase-contrast MRI, and to identify risk factors for the development of APCs. Methods and Results— APC blood flow was quantifiable in 24 of 36 retrospectively analyzed MRI studies. Sixteen studies were performed after the bidirectional cavopulmonary connections (group A) and 8 after the Fontan operation (group B). APC blood flow was calculated by subtracting the blood flow volume through the pulmonary arteries from that through the pulmonary veins. The ratio of pulmonary to systemic blood flow (Qp/Qs) was 0.93�0.26 in group A and 1.27�0.16 in group B. APC flow was 1.42 (0.58 to 3.83) L/min/m 2 and 0.82 (0.50 to 1.81) L/min/m 2 in groups A and B, respectively. The mean inaccuracies corresponded to 7.9�14.5% and 7.1�13.6% of ascending aortic flow in groups A and B, respectively. Qp/Qs was negatively correlated with a younger age at the time of the bidirectional cavopulmonary connections operation ( r =0.62, P =0.01) and positively correlated with the age at the time of the Fontan completion ( r =0.81, P =0.01). Patients with a previous right-sided modified Blalock-Taussig shunt had more collateral flow to the right lung than those without. Conclusions— APC blood flow can be noninvasively measured in bidirectional cavopulmonary connections and Fontan patients, using MRI in the majority of patients and results in a significant left-to-right shunt.

Pulmonary vein pathologies often present a diagnostic challenge. Among the different imaging modalities used for the evaluation of pulmonary veins, magnetic resonance is the most comprehensive in assessing anatomy and pathophysiology at the same time. Bright blood cine sequences and contrast-enhanced magnetic resonance angiography outline the course and connections of the pulmonary veins. Phase-contrast velocity mapping measures flow patterns, velocities, and volumes throughout the pulmonary circulation. This paper reviews contemporary utilization of magnetic resonance in the evaluation of pulmonary venous abnormalities in children, based on our experience over the last 5 years and on that of other investigators. We summarize how magnetic resonance imaging enhances our understanding of pulmonary vein physiology and how it can influence the diagnostic approach to children and adults with a pulmonary venous pathology, and we discuss its limitations.