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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. 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. 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. 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. Retrospective study [diagnostic/prognostic study]. Level IV. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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%. 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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

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. 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. 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). 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. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]