Renal angiomyolipomatosis and bleeding aneurysms in a tuberous sclerosis context: selective artery embolization in a girl with end-stage renal failure.

Recent developments in endovascular radiological techniques and devices have rendered embolization a major therapeutic option prior to surgery in many renal vascular or neoplastic diseases. A 19-yearold female patient, with a diagnosis of tuberous sclerosis complex (TSC) in childhood, was admitted with severe anemia. Polycystic kidney disease in end-stage renal failure appeared four years before and the patient has been undergoing peritoneal dialysis. The patient's medical history also included bilateral renal angiomyolipomas (AMLs). One year earlier, a unilateral endovascular embolization was performed to repair a bleeding aneurysm at the right renal upper pole. A second bilateral ruptured renal aneurysm was diagnosed at admission. To continue with peritoneal dialysis and prevent intrarenal hemorrhage and intraperitonal bleeding, an urgent bilateral renal AE was performed. Two months later she underwent a bilateral retroperitoneal nephrectomy. The posterior surgical approach, preserved the peritoneal surface area and adequate conditions to continue dialysis. At histology, bilateral AMLs were confirmed and a renal cell carcinoma of the right kidney was concurrently discovered. She undergoes continuous peritoneal dialysis. Urgent selective renal AE represents a feasible treatment for bilateral AML bleeding. It is safe and feasible before performing nephrectomy in patients with end-stage renal failure.

Laparoscopic Cholecystectomy for Symptomatic Cholecystic Disease in Children: Defining Surgical Timing.

Background: Laparoscopic cholecystectomy (LC) is the standard of care for gallbladder (GB) pathologies. We evaluated clinical, ultrasonographic (US) data as well as histopathological findings in children affected with symptomatic cholecystic disease (SCD) who underwent LC, with the aim of defining surgical timing. Methods: We reviewed our cases who underwent elective LC (ELC) or urgent LC (ULC). Clinical, US, surgical and histological features were used to create different risk scores. Results: We considered 26 children (17 ELC/9 ULC). US signs were not different in the two groups (p > 0.05). Operating times were longer in ELC than in ULC (p = 0.01). Histopathological evaluation revealed fibrosis and atrophy in both ELC and ULC. The clinical risk score was higher in ELC compared to ULC (p < 0.001). An increased operative risk score was noted in patients with systemic inflammatory signs (OR1.98), lithotherapy (OR1.4.3) and wall thickening ≥3 mm (OR2.6). An increased histopathological risk score was detected in children with symptom duration >7 days (OR3.61), concomitant hematological disease (OR1.23) and lithotherapy (OR3.61). Conclusion: Criteria adopted in adults cannot be adopted to detect the severity of GB damage in children. A dedicated clinical and US score is mandatory to define the most appropriate surgical timing.

Emphysematous Pyelonephritis Following Ureterovesical Reimplantation for Congenital Obstructive Megaureter. Pediatric Case Report and Review of the Literature.

Introduction: Emphysematous pyelonephritis (EPN) is a rare, life-threatening necrotizing infection of the kidney. To date, very few cases of EPN have been described in the pediatric age. The first case of EPN in a toddler occurring after ureterovesical reimplantation for congenital obstructive megaureter is reported with a literature review. Case Report: A 23-month-old male, with a prenatal diagnosis of obstructive megaureter and incomplete duplication of the left urinary tract, was admitted to our Unit where he underwent surgery to treat increased dilatation of the renal pelvis and appearance of an obstructive curve. The latter was revealed at renal scintigraphy, the exam highlighted the radiographic aspect of the cortical renal parenchymal sufferance. At admission preoperative exams were normal, and no recurrent urinary tract infections were documented. Surgical removal of the left stenotic ureteral common tract of the incomplete duplex collecting system was accomplished; ureterovesical reimplantation was performed without ureteral recalibration. No intraoperative complications were recorded. In the immediate postoperative period, urosepsis and the patient's lethargic condition led to life-threatening conditions requiring urgent admission to the intensive care unit. Biochemical analysis showed leukocytosis, anemia, increased C-reactive protein, prolonged prothrombin time, pancytopenia, hyponatremia. Abdominal sonographic evaluation revealed the presence of gas in the left kidney. Unilateral EPN (Class 2) was confirmed by CT- scan. Escherichia coli was cultured from peripheral blood and antimicrobial therapy was started. No additional interventions were required. The child was discharged 14 days postoperatively with normal renal function. Conclusion: EPN is a serious condition that can occur after surgical treatment for urinary tract obstruction. Early detection of air in the kidney should be considered a sign of complicated urinary tract infection. Immediate aggressive resuscitation and antimicrobial therapy are effective and curative with a positive outcome.