Pediatric metanephric adenoma with Fanconi-Bickel syndrome: a case report and review of literature.

BACKGROUND: Metanephric adenoma (MA) is a rare benign renal tumor that resembles renal cell carcinoma and Wilms' tumor in radiological as well as pathological appearance. It can present at any age or gender, and it is extremely rare in the pediatric age group with less than 50 reported cases. Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder of carbohydrate metabolism. Herein, we report a rare incidence of MA in a boy with a genetically confirmed FBS who underwent a nephron-sparing surgery. CASE PRESENTATION: A 21-month-old boy was referred to the pediatric urology clinic for further evaluation of an incidentally discovered left renal mass. His laboratory investigations showed normal renal function, hypophosphatemia, high blood glucose level, markedly elevated serum alkaline phosphatase, and low serum vitamin D. Blood picture showed signs of polycythemia and urinalysis showed glucosuria and aminoaciduria. Genetic testing was positive for Fanconi-Bickel syndrome. Radiological investigations were carried out with abdominal ultrasound and computerized tomography (CT) with intravenous contrast documented a sharply marginated peripheral hypoechoic hypovascular homogeneously enhancing mass at the upper pole of the left kidney measuring 2.0 × 1.8 × 2.0 cm. The child was admitted and started on supportive treatment until his medical condition was stabilized, then underwent elective open left partial nephrectomy via a left upper transverse abdominal transperitoneal incision. The excised renal mass was sent for histopathological assessment and was found to be a tumor composed of tightly packed tubules with no mitotic figures or necrosis and scanty cytoplasm consistent with MA. After good hydration and tumor resection, his polycythemia gradually improved. The patient was discharged home in a good condition with his proper replacement therapies. His follow-up abdominal ultrasound after 12 months showed no signs of recurrence. CONCLUSIONS: Metanephric adenoma is extremely rare in the pediatric age group, especially in those who have a FBS. The only way to diagnose and treat this tumor is by surgical resection as most patients are asymptomatic. A nephron-sparing surgery is better for this age group in which the future renal function is considered.

Bladder function in children with posterior urethral valves: impact of antenatal versus postnatal diagnosis

ABSTRACT Purpose: Posterior urethral valves (PUVs) are the most common cause of congenital bladder obstruction in boys. Our aim was to assess the impact of early diagnosis and fulguration of PUVs on bladder function and compare their functional and urodynamic outcome with children who underwent delayed intervention. Materials and Methods: We retrospectively evaluated 153 patients who underwent primary valve ablation from two tertiary hospitals between 2001 and 2018. Patients have been divided into 2 groups, group 1 included 69 patients who were detected antenatally and underwent early fulguration of PUVs while group 2 included 84 children presented postnatally and underwent delayed valve ablation. The recorded data throughout follow-up in renal function tests, urodynamics and changes in the upper urinary tracts were evaluated and compared. Results: Median age at time of valve ablation was 10 days in group 1 and 7 months in group 2. The median follow-up period was 6.5 and 7 years in group 1 and 2, respectively. Chronic kidney disease (CKD) developed in 15 (22%) boys in group 1 while in group 2 it was observed in 31 (37%), p=0.04. While Q-max, mean bladder capacity and post-void residual (PVR) volumes were comparable in both groups, percent PVR was significantly higher in group 2 (3.27 vs. 1.44, p=0.002). Detrusor overactivity was slightly different in both groups (p = 0.07). Conclusions: Compared to delayed intervention, primary ablation of PUVs during the early neonatal life possibly provides the optimum chance to have optimum renal function without impact on bladder function.

Bladder function in children with posterior urethral valves: impact of antenatal versus postnatal diagnosis.

PURPOSE: Posterior urethral valves (PUVs) are the most common cause of congenital bladder obstruction in boys. Our aim was to assess the impact of early diagnosis and fulguration of PUVs on bladder function and compare their functional and urodynamic outcome with children who underwent delayed intervention. MATERIALS AND METHODS: We retrospectively evaluated 153 patients who underwent primary valve ablation from two tertiary hospitals between 2001 and 2018. Patients have been divided into 2 groups, group 1 included 69 patients who were detected antenatally and underwent early fulguration of PUVs while group 2 included 84 children presented postnatally and underwent delayed valve ablation. The recorded data throughout follow-up in renal function tests, urodynamics and changes in the upper urinary tracts were evaluated and compared. RESULTS: Median age at time of valve ablation was 10 days in group 1 and 7 months in group 2. The median follow-up period was 6.5 and 7 years in group 1 and 2, respectively. Chronic kidney disease (CKD) developed in 15 (22%) boys in group 1 while in group 2 it was observed in 31 (37%), p=0.04. While Q-max, mean bladder capacity and post-void residual (PVR) volumes were comparable in both groups, percent PVR was significantly higher in group 2 (3.27 vs. 1.44, p=0.002). Detrusor overactivity was slightly different in both groups (p = 0.07). CONCLUSIONS: Compared to delayed intervention, primary ablation of PUVs during the early neonatal life possibly provides the optimum chance to have optimum renal function without impact on bladder function.

Pyeloplasty in children with low differential renal function: Functional recoverability.

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.

Comparison between internal double J and external pyeloureteral stents in open pediatric pyeloplasty: A multicenter study.

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.

Primary Bilateral High-Grade Vesicoureteral Reflux in Children: Management Perspective.

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.

The safety of pediatric blunt abdominal trauma in the hands of general surgeons.

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Diabetic foot: presentation and surgical management at Dammam Central Hospital.

Full text is available as a scanned copy of the original print version.