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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.

Background Vesicoureteral reflux (VUR) is a common finding in pediatric age group. Here in we explored the possible risk factors that affect the rate of resolution in patients with primary unilateral VUR under conservative treatment. Methods Between 2006 and 2014, we retrospectively evaluated all VUR patients and included only patients with primary unilateral VUR. Records were reviewed for patient age at diagnosis, antenatal history, patient gender, mode of presentation, side, and grade of VUR, associated hydronephrosis (HN) on renal ultrasound, presence of scarring and split function on dimercaptosuccinic acid (DMSA) scan. Clinical and radiological outcomes were assessed. Both univariate and multivariate analysis were conducted. Results A total of 68 patients with primary unilateral VUR were included (32 boys and 36 girls) with a mean age of 10 months (range 1–32). Antenatal HN was detected in 50% of patients. In 15 cases (22%), VUR was of high grade (IV–V). Associated HN was evident in 39 patients (57%). DMSA scans showed renal scarring in 16 patients (23%). After a mean follow-up of 7 years, VUR resolved in 49 patients (72%). Significant predictors for VUR resolution were VUR grade, DMSA split function and associated high-grade HN. High-grade HN with VUR was the only significant independent risk factor. Conclusions The rate of resolution in primary unilateral VUR under conservative treatment is significantly affected by VUR grade, DMSA split renal function and the presence of associated HN. Association of high-grade HN with VUR carries a low chance for spontaneous resolution.

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.

Background Aortic pseudoaneurysms are rare but life-threatening complications usually seen after cardiac surgery. The causes could be multifactorial such as infection or trauma. Case presentation We report the surgical management of a postoperative pseudoaneurysm of the ascending aorta caused by methicillin-resistant Staphylococcus aureus in a 2-year-old Middle Eastern boy who had undergone ventricular septal defect closure, subaortic membrane resection, and pulmonary artery de-banding. He was immediately operated on for resection of the aneurysm. A computed tomography scan at 2 months following surgery showed no aneurysm. Antibiotics were continued for 6 weeks and our patient was discharged with negative blood cultures. Conclusion Early diagnosis and appropriate treatment of such rare complication can be lifesaving.

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.

Summary Introduction and Objective Anderson–Hynes dismembered pyeloplasty is considered the standard surgical treatment for ureteropelvic junction obstruction (UPJO). After pyeloplasty, stent drainage remains controversial. The commonly used stents are either an internal double-J (DJ) or an externalized pyeloureteral (PU) stents. We evaluated the outcome of using DJ versus PU stents following open pyeloplasty for UPJO in children. Study design We retrospectively evaluated 175 patients who underwent primary open pyeloplasty in two tertiary hospitals. A total of 110 patients underwent internal DJ stent insertion (63%) while 65 patients (37%) underwent placement of external PU stent. The type of stent used at the time of surgery was according to surgeon preference and experience. Operative time, postoperative hospital stay, overall complications and success rates were compared between the two groups. Results The mean age was 3.8 years, and the mean follow-up was 4 years. Mean operative time was similar in the two groups (145 min). Mean hospital stay was 3.7 and 4.2 days in DJ and PU stent, respectively (p = 0.003) Summary Table . Postoperative complication developed in 9 out of 110 patients with DJ stent (8%), while complications developed in 6 out of 65 patients with PU stent (9%) (p = 0.81). Success rate of pyeloplasty was 95.5% for DJ group versus 97% for PU group (p = 0.63). Discussion Dismembered pyeloplasty remains the standard treatment of choice as a surgical management for UPJO. A debate is still there in respect to the method of PU anastomotic stenting and which stent can be used. The major advantage for external PU stents is that it can be removed safely in the outpatient clinic without any sedation preventing the risk of repeated exposure to general anesthesia. Internal DJ stent provides a shorter hospital stay and comparable complication and success rates compared with PU stent. If we manage to overcome the longer DJ stent duration and facilitate early removal by an easy mode, that does not require another anesthesia at that moment we can find the optimal stent for all pyeloplasty cases. Conclusions The two types of stents are comparable as regard overall complication and success rates after pyeloplasty. Although internal DJ stent insertion provides a relatively shorter hospital stay, a second operating room visit and anesthesia for removal remains unavoidable.

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.

The images of transcatheter closure of ruptured sinus of valsalva are presented.

Purpose To assess the feasibility of phase-contrast magnetic resonance (PCMR) in quantifying the pulmonary venous return in normal subjects. Materials and Methods PCMR was performed in 12 healthy adult volunteers (mean age 38 years, range 27–60 years; 9 men; body surface area 1.81 ± 0.15 m2) for the ascending and descending aorta, caval veins, main and branch pulmonary arteries, and pulmonary veins. Two readers independently quantified blood flow in all subjects. Results Intraobserver differences were −2.0% (95% confidence interval [CI]: −9.9% to 5.9%), −4.5% (95% CI: −15.6% to 6.5%), and −0.7% (95% CI: −4.5% to 3.0%) for all vessels, pulmonary veins, and other great vessels, respectively. Interobserver differences were −2.0% (95% CI: −10.6% to 6.6%), −3.1% (95% CI: −16.0% to 9.9%), and −1.4% (95% CI: −6.4% to 3.5%) for all vessels, pulmonary veins, and other great vessels, respectively. Pulmonary venous flow volume showed high correlations with the volumes of the pulmonary arterial flow, systemic arterial flow, and systemic venous flow (r = 0.76–0.92, P < 0.005). Conclusion Flow quantification of normal pulmonary venous return using PCMR is feasible with high reproducibility and accuracy. J. Magn. Reson. Imaging 2009;29:588–594. © 2009 Wiley-Liss, Inc.

Objective We describe a case of mitral valve (MV) replacement using modified Melody valve implantation. Methods A 2.5 year-old girl,with a history of ALCAPA repair in April 2012, had undergone MV repair by ring annuloplasty at 1 year of age. She came back with severe MV stenosis (mean gradient, 8 mmHg) and regurgitation. She had a hugely dilated left atrium and a MV annulus of 14 mm. She, then, underwent surgical MV replacement with a modified Melody valve. Results The Melody valve was prepared before the cardiopulmonary bypass. The procedure included stent shortening and adding a bovine pericardial sewing ring. Through a trans-septal approach, the previous MV ring was removed, the pericardial ring patch of the Melody valve was secured to the mitral annulus and the ventricular end of the valve was fixed to the posterior–inferior wall of the left ventricle. The prosthesis was then inflated to size 16 mm. Testing of the valve showed good leaflet coaptation. The atrial septum was closed by fenestrated (4 mm) bovine pericardial patch. TEE showed good valve function with a tiny paravalvular leak and no left ventricular outflow obstruction. The pulmonary veins were also unobstructed. Conclusions The modified Melody valve is a viable option for children MV annular diameters, providing a valid alternative to existing prostheses. The technique is relatively easy and the short term result is very good. This prosthesis will be particularly attractive if maintaining competence after subsequent dilations as the child grows.

Aim The choice between primary and staged repair of atrioventricular septal defect with tetralogy of Fallot (AVSD/TOF) is still controversial. We report our surgical experience with this lesion. Methods Twenty-four patients with AVSD/TOF were repaired between 1997 and 2013. Ten, group 1 (40%), underwent primary repair at a mean age of 29.4 months. Fourteen, group 2 (60%), underwent staged repair at a mean-age of 65.2 months. Mean interval between shunt and repair was 26.5 months. Out of 24 patients, 8 (33%) were repaired by transannular patch, 15 (62%) by pulmonary valve preservation and 1 by pulmonary valve replacement. Results There was one hospital death in group 2. Mean follow-up period was 70 months. In group 1 , 8 patients were alive while 2 were lost to follow-up. Three were re-operated, one for mitral valve repair and left diaphragmatic plication, one for thoracic duct ligation and one for pulmonary valve replacement. In group 2 , 10 patients were alive while 3 were lost to follow-up. One developed biventricular outflow obstruction and is awaiting surgery. One had ventricular septal defect device closure. There were 3 reoperations, one for left atrioventricular valve and pulmonary valve replacement, one for pulmonary valve replacement alone and one for relief of biventricular outflow obstruction. All transannular patch patients had dilated right ventricle with moderate to severe tricuspid valve regurgitation. Conclusion Shunt procedure is a reasonable initial option for many of patients with AVSD/TOF but primary repair can be performed in selected patients with low operative mortality and reasonable morbidity.

We report a case in which a meandering right pulmonary vein connecting to the left atrium is associated with hypoplasia of the right lung, horseshoe lung, abnormal pulmonary lobation, and abnormal branching of the pulmonary arteries. We discuss its relationship to the so-called scimitar variant, and to the scimitar syndrome itself.

Background Residual ventricular septal defects (VSDs), ventricular and septal dysfunction and heart block are frequent complications in surgical management of multiple VSDs (Swiss cheese heart). The technique with the lowest risk of morbidity and mortality has still to be identified. Methods Between January 200 and September 2012, 45 patients with a median age of 4.1 years (2 months to 15 years) underwent surgical closure of multiple VSDs. In 22 cases there were associated lesions. Two-stage repair, i.e. pulmonary artery banding (PAB) followed by VSDs closure using different techniques, was adopted in 29 cases (group A). Primary repair was performed in 15 cases (group B). Six out of these 15 cases were managed by the so-called sandwich technique (group B1), whereas in the other nine other techniques of closure were used (group B2). Results The overall mortality was 8.9% (4/45). Three of the four deaths occurred in group A and one in group B2. Follow-up of group A showed that 26 patients had residual VSDs (2 of whom needing early redo surgery), 2 patients had left ventricular dysfunction and one patient developed heart block. In group B1, there were five cases with residual VSDs. In group B2, there were five cases with residual VSDs (only one needing redo surgery), one case with left ventricular dysfunction and one case with heart block. Conclusion Primary repair of multiple VSDs is associated with less morbidity and mortality than staged repair. The sandwich technique has the lowest risk of residual VSDs, septal dysfunction and heart block compared to the other techniques. However, particular attention is needed in positioning the left-sided patch to avoid septal bulging with consequent left ventricular outflow tract obstruction and interference with mitral valve chordae with secondary mitral regurgitation.

Background: Preeclampsia is characterized by development of high blood pressure and proteinuria. It affects 5–8% of all pregnancies and is a major contributor to maternal and fetal morbidity and mortality. There is no single test that fulfils all the criteria for a good predictor of preeclampsia and associated renal damage. Aims & Objectives: To evaluate the role of serum and urine biochemical parameters as early predictors of preeclampsia. To investigate the role of BUN: Creatinine ratio in diagnosing preeclampsia and evaluating prognosis of the disease. Material and Methods: In the present prospective study, one hundred and twenty pregnant women divided into three groups: normotensive (control), women at high risk and with preeclampsia were included. Analyses of different biochemical parameters including BUN: Creatinine were carried out. Results: There was significant difference in the mean value of serum uric acid, blood urea nitrogen, creatinine, urinary protein and BUN: Creatinine ratio in preeclampsia group compared to control group (p < 0.001). There was significant difference (p < 0.05) in serum uric acid between control and preeclampsia group. However, there was no significant change in haematocrit, serum creatinine and urine protein between control and high risk group. Conclusion: BUN: Creatinine ratio in pregnant women with preeclampsia and also in high risk group was significantly increased (t = 15.55, p < 0.001 and t = 8.66, p < 0.001 respectively) in comparison to the control group. This index could be useful in evaluating the severity of preeclampsia and could be used as a predictor in prognosis of preeclampsia and subsequent early renal disease.

Purpose: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. Materials and Methods: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis. We excluded patients with bilateral dilation or other urological abnormalities. The fate of hydronephrosis including resolution, stability or worsening was documented. Results: A total of 66 infants (44 boys and 22 girls) with antenatally diagnosed unilateral urinary tract dilation (23 right and 43 left) were identified. Ultrasounds showed SFU grade 1 hydronephrosis in 32 patients (48%) and SFU grade 2 hydronephrosis in 34 (52%). After a mean follow-up period of 32 months (range 12 to 60), 37 patients (56%) had complete resolution of hydronephrosis while the remaining 29 were stable (44%). None of our patients developed UTIs during follow-up and none required surgical intervention. Conclusions: Prenatally detected, isolated unilateral low-grade hydronephrosis usually have a favorable prognosis. All cases in our cohort showed either stability or resolution of hydronephrosis without any harmful consequences. Based on our findings on medium-term in this category of patients, long-term follow-up is not warranted.