Paediatric stone management: innovations and standards.

PURPOSE OF REVIEW: The aim of this study was to discuss current trends and recent developments in the surgical management of paediatric urolithiasis. RECENT FINDINGS: Medical expulsive therapy (MET) is considered as an option for the treatment of children with ureter stones. Extracorporeal shockwave lithotripsy (SWL) remains as a first option for majority of kidney stones and upper ureter stones. Advances in endourology have led to increased adoption of retrograde intrarenal surgeries (RIRS) and percutaneous nephrolithotomy (PCNL/mini-PCNL/ultra-mini-PCNL/micro-PCNL). SUMMARY: Management of paediatric stone disease has developed significantly in the past decade. However, the evidence in the literature remains disproportional to these advances. Well designed multicentric studies are still needed to compare effectiveness and safety of these procedures.

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.

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.