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ABSTRACT Introduction: Dismembered open pyeloplasty described by Anderson and Hynes is the "gold standard" for the treatment of ureteropelvic junction obstruction. The aim of our study was to compare the results of open (OP) vs laparoscopic (LP) vs robotic (RALP) pyeloplasty. Material and Methods: A multicenter prospective review was conducted of pyeloplasty surgeries performed at five high-volume centers between 2014 and 2018. Demographic data, history of prenatal hydronephrosis, access type, MAG3 renogram and differential renal function, surgery time, length of hospital stay, and complication rate (Clavien-Dindo) were recorded. Access type was compared using the Kruskal-Wallis, Chi-square, or Fisher's exact tests. Results: A total of 322 patients were included: 62 OP, 86 LP, and 174 RALP. The mean age was 8.13 (r: 1-16) years, with a statistically significant lower age (mean 5 years) in OP (p < 0.001). There were no significant differences in the distribution of the side affected. Operative time was 110.5 min for OP, 140 min for LP, and 179 min for RALP (p < 0.0001). Hospital stay was significantly shorter in the RALP group than in the other groups (p < 0.0001). There were no differences in postoperative complications and reoperations between the three groups. Conclusions: Minimally invasive surgery for the management of UPJO in children is gaining more acceptance, even in patients younger than 1-year-old. Operative time continues to be significantly shorter in OP than in LP and RALP. Hospital stay was shorter in RALP compared to the other techniques. No differences were found in complication rates, type of complications, and reoperation rate.

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INTRODUCTION: Dismembered open pyeloplasty described by Anderson and Hynes is the "gold standard" for the treatment of ureteropelvic junction obstruction. The aim of our study was to compare the results of open (OP) vs laparoscopic (LP) vs robotic (RALP) pyeloplasty. MATERIAL AND METHODS: A multicenter prospective review was conducted of pyeloplasty surgeries performed at five high-volume centers between 2014 and 2018. Demographic data, history of prenatal hydronephrosis, access type, MAG3 renogram and differential renal function, surgery time, length of hospital stay, and complication rate (Clavien-Dindo) were recorded. Access type was compared using the Kruskal-Wallis, Chi-square, or Fisher's exact tests. RESULTS: A total of 322 patients were included: 62 OP, 86 LP, and 174 RALP. The mean age was 8.13 (r: 1-16) years, with a statistically significant lower age (mean 5 years) in OP (p < 0.001). There were no significant differences in the distribution of the side affected. Operative time was 110.5 min for OP, 140 min for LP, and 179 min for RALP (p < 0.0001). Hospital stay was significantly shorter in the RALP group than in the other groups (p < 0.0001). There were no differences in postoperative complications and reoperations between the three groups. CONCLUSIONS: Minimally invasive surgery for the management of UPJO in children is gaining more acceptance, even in patients younger than 1-year-old. Operative time continues to be significantly shorter in OP than in LP and RALP. Hospital stay was shorter in RALP compared to the other techniques. No differences were found in complication rates, type of complications, and reoperation rate.

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BACKGROUND: Epstein-Barr virus (EBV) is a DNA virus with oncogenic potential, especially in immunocompromised patients. EBV can promote smooth muscle proliferation, resulting in EBV-associated smooth muscle tumors (EBV-SMT). METHODS: We report a case of a 10-year-old child with end-stage renal disease secondary to hypoplastic crossed and fused kidneys who underwent kidney transplantation. EBV serology was unknown for the donor and negative for the recipient; three months after he had a primary EBV infection. Two years after the transplantation, percutaneous nephrostomy was performed because of a drop in the estimated glomerular filtration rate and severe dilatation of the graft. Nephrography showed contrast enhancement of the pelvis of the graft kidney and proximal ureter, with a clear blockage at the level of the mid ureter and no passage towards the bladder. A 1.5-cm tumor was found causing intraluminal compression of the mid ureter. RESULTS: Complete resection of the tumor and distal ureter was performed leaving a short proximal ureter. A tension-free uretero-ureteroanastomoses was achieved using the native ureter. There were no surgical complications. Histologic evaluation showed spindle-shaped muscle cells, moderate pleomorphism, and inflammatory infiltration. Immunohistochemical staining was positive for muscle-specific actin. Epstein-Barr encoding region (EBER) in situ hybridization was positive, confirming the diagnosis of EBV-associated SMT. CONCLUSIONS: EBV-SMT is an exceedingly rare oncological entity that may develop in either the graft or any other organ. The clinical findings are location related. EBV seroconversion following transplantation might be a risk factor for the development of SMT in solid organ recipients.

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OBJECTIVE: To describe the effects of oral sirolimus administered before and after surgical resection of slow-flow vascular malformations of the scrotum in pediatric patients. METHODS: Retrospective review of 3 patients presenting with complex lymphatic-venous malformations of the scrotum who received adjuvant oral sirolimus 3 months before and 3 months after surgical resection. Demographic data, clinical course, imaging findings, and management strategies were reviewed for each patient. RESULTS: In each of the 3 patients, there was a significant volume reduction of the lesion within the 3 months after initial dose of sirolimus. Scarce lymphatic leakage during and after surgery was reported, associated with an adequate wound healing. Two years after the last postsurgical dose of sirolimus, all patients remain asymptomatic without any lymphatic leakage or lesion recurrence. CONCLUSION: Combined lymphatic-venous vascular malformations of the male genitalia are rare but associated with high morbidity and challenging treatment options. Pre- and postsurgical adjuvant treatment with oral sirolimus seems to be a promising therapeutic option that provides reduction of the lesion size before surgery and improvement of postsurgical recovery and wound healing.

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INTRODUCTION: We compare open pyeloplasty (OP) versus laparoscopic pyeloplasty (LP) in children in a multicenter, prospective, case-control study. MATERIALS AND METHODS: From May 2007 to March 2009, a program was established at Hospital Garrahan, the reference center, to perform LP with a mentoring surgeon that would attend the institution once a month. Every new case of ureteropelvic junction obstruction (UPJO) diagnosed in the reference institution was offered to participate in the study. If the patient was enrolled, it was scheduled for LP. The following patient diagnosed with UPJO was operated on with open technique and served as a case-control. In three other facilities, patients were only offered LP and had a matched control open case at the reference institution. The first end point of the study was patient recovery: analgesia requirement and length of hospitalization (LOH). The second end point of the study was resolution of UPJO in long-term follow-up for the two techniques. Demographic data, surgical time, perioperative complications, analgesia requirement, analgesia score during hospitalization, LOH, and outcome were recorded. Both groups received the same postoperative indications for pain control. Parents were asked to assess pain in their children every 4 h postoperatively and to complete a pain scale chart to which the nurses were blinded. RESULTS: Fifteen OP and 15 LP were compared. Groups were similar with regard to sex, age, weight, and laterality. Mean surgical time was longer in LP than in OP group (mean 188 versus 65 min) (p < 0.01). Hospitalization was shorter for LP group with a mean of 1.9 versus 2.5 days for OP group (p < 0.05). Postoperative analgesia requirement was significantly higher in the OP group with a mean use of morphine of 1.7 versus 0.06 mg/kg in the LP group (p < 0.05). Pain scores were similar in both the groups. At a mean follow-up of 58 months there were no failures. CONCLUSION: In this prospective comparative cohort, LP was a longer procedure than OP. Both procedures had the same efficacy and complication rates, but patients undergoing LP needed fewer narcotics for pain control and had a shorter hospitalization.

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OBJECTIVE: to describe and incorporate a modification of the fully laparoscopic Mitrofanoff (LM) procedure with the aim of diminishing operative time and technical difficulties, and to reproduce functional results. PATIENTS AND METHODS: Retrospective review of six patients with voiding dysfunction with indication for clean intermittent catheterization who did not require bladder augmentation and who underwent a fully LM procedure between January and November 2011. RESULTS: The mean operative time was 183.3 min with a mean hospital stay of 6.15 days. No complications were recorded during the surgical procedure. Postoperative hematuria is the most frequent complication associated with this technique. One patient presented appendicovesical anastomotic leak that resolved spontaneously after 8 days with abdominal drainage. There were no other postoperative complications on an average 6.8-month follow up. CONCLUSION: We believe fully LM is a feasible and reproducible approach. The U-Stitch appendix implantation technique reduces total operative time and makes the procedure technically easier.

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When the neurogenic bladder is refractory to anticholinergics, botulinum toxin type A is used as an alternative. The neurotoxin type A reduces bladder pressure and increases its capacity and wall compliance. Additionally, it contributes to improving urinary continence and quality of life. This novel therapy is ambulatory with a low incidence of adverse effects. Due to its transitory effect, it is necessary to repeat the injections in order to sustain its therapeutic effect. In these review article we talk about Mechanism of Action, Indications, effects, administration and presentations of the Botulinum Neurotoxin Type A in pediatric patients. Also, we make references to controversial issues surrounding its use. A bibliographic search was done selecting articles and revisions from Pubmed. The key words used were botulinum toxin A, neurogenic bladder, and children. The search was limited to patients younger than 18 years of age and reports written in English in the past ten years.

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Ureteral necrosis is an uncommon complication following RT which can adversely affect outcome. Even though authors agree that the native ureter ought to be preserved, there are cases in which graft function can only be salvaged by ureteral substitution. The scant references in the literature on the use of the appendix for left ureteral replacement in children prompted us to report the following two cases in whom the technique was employed and to assess the evolution of graft function in these patients.

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PURPOSE: To present three cases of arterial high flow priapism (HFP) and propose a management algorithm for this condition. MATERIALS AND METHODS: We studied three children with post-traumatic arterial HFP (two patients with perineal trauma and one with penis trauma). RESULTS: Spontaneous resolution was observed in all the patients. The time of resolution by a return to a completely flaccid penis was different: 14, 27 and 36 days in each case. CONCLUSIONS: Absence of long-term damaging effects of arterial HFP on erectile tissue combined with the possibility of spontaneous resolution associated with blunt perineal trauma are suggestive signs for the introduction of an observation period in the management algorithm of HFP. Such a period may help to avoid unnecessary surgical intervention. Thus, these cases reinforce the decision to manage these patients conservatively and avoid angiographic embolization as a first therapeutic choice.

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PURPOSE: To determine whether surgical procedures of the lower urinary tract in patients with uropathies affect evolution of the graft in renal transplantation. METHODS: 156 kidney transplantations were performed in 150 patients with end-stage renal failure due to urologic disorders. The patients were classified into three groups: A, patients who did not require surgery in the lower urinary tract; B, required surgery and preserved adequate bladder function, and C, required surgery due to vesical dysfunction. RESULTS: Graft survival rates at 1 year were 93.38% in group A, 95.45% in group B and 93% in group C. Rates at 5 years post-transplantation were 82.45, 79.85 and 86.58% for each group, respectively (not significant). Complications were vesicoureteral stenosis: 2 in group A, 3 in B and 1 in C; vesicoureteral reflux: 1 in group A, 1 in B and 10 in C; distal ureteral necrosis: 2 cases in group A, 2 in B and 1 in C; upper urinary tract infection: 12, 23.1 and 42.2% in each group, respectively. CONCLUSION: Children with reconstructed urinary tract may be good candidates for kidney transplantation despite the higher frequency of urinary infections. Thus, careful and strict post-surgical urologic follow-up is mandatory.

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We present a case of a 16-year-old adolescent boy with autoimmune pancreatitis and a review of the related literature. The patient was sent from a peripheral medical center, presenting with jaundice, pruritus, weight loss, and hyperglycemia of 20 days' duration. At admission, the patient was icteric, choluric, and acholic. His abdomen was soft and nontender, and the patient felt no pain in his abdomen. He had skin lesions because of scratching. Laboratory findings showed a blood glucose level of 135 mg%; total serum bilirubin, 29.4 mg%; direct bilirubin, 23 mg%; and alkaline phosphatase, 1100 IU/L. Abdominal ultrasound showed an enlarged head of the pancreas that was 30 x 35 mm. The parenchyma was slightly heterogeneous. Abdominal computed tomography showed an enlarged head of the pancreas with a normal body and tail, thickened duodenal wall, and dilated intra- and extrahepatic biliary tract, and the distal choledochus was not visible. Magnetic resonance imaging showed dilated intra- and extrahepatic biliary tract. The choledochus was not visible, and the cystic duct ended abruptly. The pancreas head was enlarged and homogenous, and there were no changes with contrast. Wirsung's duct was not dilated. Laparotomy was performed with a presumed diagnosis of pancreatic head tumor. The pancreas was diffusely indurated and enlarged; biopsy and intraoperative cholangiography were performed. The biliary tract was dilated with no duodenal passage of contrast, and the Wirsung's duct was not observed. Cholecystectomy was performed, and a transcystic drain was positioned. The histopathology was compatible with autoimmune pancreatitis. Prednisone treatment was started with good response. Autoimmune pancreatitis is a very rare entity among children and adolescents. It should be suspected when characteristic clinical signs and radiographic images are associated with a higher level of IgG4. Autoimmune pancreatitis is confirmed by biopsy. Treatment with prednisone often alleviates all the symptoms, as what happened in this case.

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