Urological outcome with the use of salvage continent vesicostomy in pediatric reconstructive surgery. A single tertiary center experience.

PURPOSE: To report our experience and results in terms of complications, reoperation rate and urinary continence with the Salvage Continent Vesicostomy (SCV) technique in pediatric patients with history or need of enterocystoplasty and absent appendix. METHODS: Retrospective review of all patients with a history of a continent catheterizable channel surgery performed in our institution between June 2016 and January 2022. Only patients with a SCV surgery with a minimum 6-month post operative follow up were included in this cohort and divided in group 1 (history of previous bladder augmentation) and group 2 (simultaneous bladder augmentation and SCV). Primary outcome of the study was to assess both continence and postoperative complication rates. Early complications were assessed using the Clavien-Dindo classification (I-V). Late complications were focused on the need of further subfascial revision. RESULTS: 84 patients with a history of a continent stoma creation surgery were identified. In 20 of them (12 males) a SCV was performed. The mean age at surgery was 10.38 (range 4.87-15.6) years and the median postoperative follow-up time was 32 (range 6-64) months. Eleven patients were included in Group 1, while 9 patients in Group 2. Early complications occurred in 4 patients (20%), two of them required a re-intervention (Clavien-Dindo IIIb). Stoma subfascial revision was further required in 2 patient (10%). Continence rate at last follow up was 95%. CONCLUSIONS: In our early experience, the salvage continent vesicostomy has proven to be a simple continent stoma technique with acceptable both continence and complication rates that can be used in selected patients with a history or need of bladder augmentation and absent appendix.

"Epstein-Barr virus associated smooth muscle tumour as an unusual cause of ureteric graft obstruction in a child".

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.

Laparoscopic Upper-pole Heminephrectomy for the Management of Duplex Kidney: Outcomes of a Multicenter Cohort.

OBJECTIVE: To analyze the outcomes of patients undergoing upper-pole heminephrectomy surgery and to assess the different variables that may have an impact on outcome, specifically regarding morbidity and the need for further surgeries. MATERIALS AND METHODS: A retrospective study of patients who underwent laparoscopic upper-pole heminephrectomy for a nonfunctional moiety between August 2007 and December 2019 was conducted at 3 centers. A total of 130 patients met the inclusion criteria. A transperitoneal approach was used. The following variables were evaluated: (1) preoperative: presentation, presence of ureterocele and history of ureterocele incision, hydronephrosis grade, presence of vesicoureteral reflux (VUR), and differential renal function (DRF) on renal scintigraphy; and (2) postoperative outcomes: Doppler ultrasound, renal scintigraphy, complications, febrile urinary tract infection, lower urinary tract symptoms, and need for further surgery. Findings were considered statistically significant at P <.05. RESULTS: Postoperative complications were observed in 5 patients (3.8%). Six patients (4.6%) needed further surgeries after heminephrectomy. The presence of ureterocele and VUR was related with the need for further surgeries odds ratio (OR) 4.91, P = .0415) and the occurrence of postoperative febrile urinary tract infection (OR 2.81, P = .0376). A 13.9% incidence of lower urinary tract symptoms was found with no difference between patients with ureterocele and those with an ectopic ureter. Renal scintigraphy showed a median decrease in DRF of 2.7%. No patient had complete loss of function. CONCLUSION: Laparoscopic upper-pole heminephrectomy showed to be a feasible and safe procedure. In most patients this surgery will be the definitive procedure. Patients with both VUR and ureterocele will need special consideration. No significant loss of function in the remaining lower moiety was found.

Bladder Volume Assessment in Pediatric Patients With Neurogenic Bladder: Is Ultrasound an Accurate Method?

OBJECTIVE: To define the accuracy of ultrasound to determine bladder volume in pediatric patients with neurogenic bladder (NB). METHODS: Retrospective analysis of children with NB in treatment with urethral clean intermittent catheterization. EXCLUSION CRITERIA: bladder surgeries, and catheterization through a channel different than urethra. Bladder volume was measured with ultrasound using the formula: anteroposterior bladder diameter by side to side diameter by distance from dome to outlet tract by 0.523 (cm3). In the same act, the patient was performed urethral catheterization and the drained volume was measured in millimeters. Finally, postvoid residual volume (PVR) was assessed with ultrasound. RESULTS: We performed 318 measurements in 299 patients, mean age was 9.95 years (standard deviation: 4.6), 59% were female. Most frequent etiologies of NB were myelomeningocele and lipomyelomeningocele. Mean ultrasound-determined bladder volume was 213.9cm3 (range: 20-899 cm3) and mean bladder volume drain through catheterization was 336.4 mL (range: 30-1480 mL; P : .0001). In 67.3% of the patients (n: 214) PVR was not significant, and their mean ultrasound volume was 212.7 mL and the volume evacuated by catheterization was 339.9 mL (P : .0001). In all age groups ultrasound-determined bladder volume was statistically lower than catheterized bladder volume (P : .0001). The mean percentage error of the ultrasound-determined bladder volume was 15.58% ± 44.09. Linear regression analysis and Bland-Altman plot showed low agreement between both measurement techniques. CONCLUSION: In children with NB, ultrasound-determined bladder volume was statistically lower than catheterized bladder volume measured at the same moment, and this relation persisted regardless of sex, age, and the presence of PVR.

Long-term urological assessment and management of cloaca patients: A single tertiary institution experience.

PURPOSE: Preserving renal function and achieving urinary continence are the most important urological goals in the management of cloaca patients. Many prognostic factors have been described, such as the length of the common channel (CC) and urethra, the presence of spinal dysraphism, and associated urological anomalies. The aim of this study was to analyze urinary continence status and need for reconstructive procedures and their correlation with urological risk factors in a series with a long-term follow-up. MATERIAL AND METHODS: The institutional database of patients with anorectal malformations was reviewed. Patients with cloaca who underwent cloacal reconstructive surgery at our institution between January 1995 and May 2015 and who had a minimum postoperative follow-up of 5 years with complete urological care were included. Urologic and spinal anomalies, length of the CC, renal function, urodynamic study patterns, continence status, and urologic reconstructive surgeries were assessed. A CC was defined as long CC when its measure was longer than 3 cm in the cloacogram and then confirmed by cystoscopy. Descriptive statistical analysis was performed. RESULTS: Fifty-five cloaca patients with a mean follow-up of 12 years (5-20) were included. A long CC was documented in 38 patients (69%). The sacral ratio (SR) was <0.4 in the AP projection in 30 (54.5%). Urodynamic evaluation revealed an inadequate detrusor contraction pattern in 65.4% of the cases. A CC >3 cm and SR <0.4 were significantly correlated with this urodynamic finding. A total of 50 patients (91%) achieved urinary continence, but only 30.9% had volitional voiding and 56% needed major urological reconstructive surgeries. Eighteen patients (32.7%) were in stage 2 or more of chronic kidney disease (<90 ml/min/1.73 m2) at the last follow-up visit. CONCLUSION: Cloacal management requires a multidisciplinary and long-term follow-up. Early assessment of prognostic urological factors and accurate stratification of each patient are essential to avoid renal impairment and achieve urinary continence in the future. The length of the common channel and the presence of spinal dysraphism were correlated with the presence of neurovesical dysfunction specifically an abnormal bladder contraction efficiency. A significant number of these patients will need catheterization and reconstructive urinary tract surgeries to attain urinary continence. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level 3. Retrospective comparative study.

Simultaneous bilateral endoscopic surgery in a pediatric patient: Description of a NOVEL technique.

INTRODUCTION: Simultaneous bilateral endoscopic surgery (SBES) is the performance of a percutaneous nephrolithotomy (PCNL) on one side and a retrograde intrarenal surgery (RIRS) on the other kidney simultaneously. OBJECTIVE: Our aim is to report through a video the surgical technique and the step-by-step details of a SBES performed in a pediatric patient. PATIENTS AND METHODS: A 12-year-old male patient with bilateral upper tract urolithiasis. RESULTS: Supine PCNL is performed in Galdakao-modified Valdivia position. Ureteroscopic surgeon performs retrograde pyelography on PCNL kidney. Percutaneous access under fluoroscopic guidance trough the lower calyx and dilated with Amplatz dilators to 17.5 Fr. Storz® 17.5 MIP Nephroscope is introduced and fragmentation with pneumatic lithotripter starts. On the other kidney, after introduction of ureteral sheet 12/14 Fr and digital flexible ureteroscope Flex Xc ® 9.5Fr, pulverization of the stone starts with Laser Holmium YAG (Oddysey®). Surgeons work simultaneously and share fluoro-scopic c-arm. CONCLUSION: SBES can be reproduced in children. Proper planification and preparation of the involved team and OR set up are essential. Further prospective randomized studies are needed to establish safety and efficacy of SBES in pediatric patients.

Kidney transplantation in children with CAKUT and non-CAKUT causes of chronic kidney disease: Do they have the same outcomes?

Almost half the children who undergo kidney transplantation (KTx) have congenital abnormalities of the kidney and urinary tract (CAKUT). We compared patient, graft survival, and kidney function at last follow-up between CAKUT and non-CAKUT patients after KTx. We divided the analysis into two eras: 1988-2000 and 2001-2019. Of 923 patients, 52% had CAKUT and 48% non-CAKUT chronic kidney disease (CKD). Of the latter, 341 (77%) had glomerular disease, most frequently typical HUS (32%) and primary FSGS (27%); 102 had non-glomerular disease. CAKUT patients were more often boys, younger at KTx, transplanted more frequently preemptively, but with longer time on chronic dialysis. They had less delayed graft function (DGF) and better eGFR, but higher incidence of urinary tract infection (1 year post-KTx). In both eras, 1-, 5-, and 10-year patient survival was similar in the groups, but graft survival was better in CAKUT recipients vs those with primary glomerular and primary recurrent glomerular disease: Era 1, 92.3%, 80.7%, and 63.6% vs 86.9%, 70.6%, and 49.5% (P = .02), and 76.7%, 56.6%, and 34% (P = .0003); Era 2, 96.2%, 88%, and 73.5% vs 90.3%, 76.1%, and 61% (P = .0075) and 75.4%, 54%, and 25.2% (P < .0001), respectively. Main predictors of graft loss were DGF, late acute rejection (AR), and age at KTx in CAKUT group and disease relapse, DGF, early AR, and number of HLA mismatches in recipients with glomerular disease. Graft survival was better in CAKUT patients. DGF was the main predictor of graft loss in all groups. Disease recurrence and early AR predicted graft failure in patients with glomerular disease.

Pediatric Urodynamic Study Without a Preprocedural Urine Culture, Is It Safe in Clinical Practice?

OBJECTIVE: To prove that incidence of UTI after a pediatric urodynamic study (UDS) is low, and that patients without urine culture (UC) analysis prior to a UDS will not have a significant increase in the incidence of UTI (post-UDS UTI). METHODS: Prospective cohort study including consecutive pediatric patients undergoing UDS in a single center for 1 year. Patients were divided in 2 groups: (G1) UDS with a previous negative UC and (G2) UDS without a previous UC analysis. A clean UC was obtained in all patients at the moment of the UDS (UDS-UC). Primary outcome was post-UDS UTI. Secondary outcome was to compare UC cost per patient and cancellation rates in each group. All patients were followed 15 days after the UDS to detect onset of UTI symptoms. RESULTS: Four hundred two patients were included, 198 patients in G1 and 204 patients in G2. Median age was 9 years old. Both groups were similar in terms of demographic and clinical records data except for a proportion of patients on CIC which was larger in G2 (P <.008). Overall incidence of post-UDS UTI was 0.7% (3/402), G2 incidence (0.98%) being slightly higher than G1 (0.50%; P <.58). UDS-UC was positive in 32% of G1 vs 55% in G2 (P <.001). About 98% of patients with positive UDS-UC did not progress to symptomatic UTI. G1 cost was 140% higher than G2. CONCLUSION: Overall incidence of post-UDS UTI is low (0.7%). Patients without UC prior to UDS did not have a significant increase in post-UDS UTI.

Efficacy and safety of Mirabegron as adjuvant treatment in children with refractory neurogenic bladder dysfunction.

INTRODUCTION: When patients with neurogenic bladder become refractory, there are different alternatives, such as the use of ß3-adreceptor agonists. The aim of the present study is to evaluate efficacy and safety of Mirabegron as adjuvant treatment. MATERIAL AND METHODS: 37 patients under 18 years of age who underwent Mirabegron were retrospectively studied. The inclusion criteria were: cases with neurogenic bladder who were under clean intermittent catheterization (CIC) programs and refractory to oral oxybutynin (Group A) and/or onabotulinumtoxinA (Group B). Once refractory neurogenic bladder was confirmed by clinical and/or urodynamic studies, Mirabegron 25 mg/day was indicated and evaluation was performed in the third month without stopping therapy. Systolic/diastolic blood pressure and transaminases were monitored. Paired t test and Pearson's chi - squared test were used. RESULTS: Maximum cystometric capacity increased significantly by 125 mL, from 322 to 446 ml (p < 0.0001). End-filling detrusor pressure decreased significantly by 12 cm H2O, from 44 to 31 cm H2O (p < 0.0001). The variation in both parameters was significant in Groups A and B. The presence of detrusor overactivity increased globally from 21 to 32% after starting Mirabegron, but the intensity of contractions was reduced in 20 cm H2O. Of the 18 patients who were incontinent before, 13 cases (72%) remained dry after initiating therapy with Mirabegron. None of the patients stated having suffered any adverse effects. Blood pressure and transaminases showed no significant difference. None of the patients discontinued treatment due to intolerance to Mirabegron (Summary Table). DISCUSSION: In our study the treatment with Mirabegron improved significantly the clinical and urodynamic parameters. A significant increase in bladder capacity and a significant decrease in end-filling detrusor pressure were observed in both groups. The intensity of overactivity was attenuated. According to the records of the voiding diary, over 70% of the incontinent patients became dry after the administration of Mirabegron. We did not observe any adverse effects. The most important limitations of the present study are its retrospective design, the small size of the sample population and of each group, and the use of only one dose of Mirabegron. CONCLUSIONS: Mirabegron as adjuvant treatment in children with refractory neurogenic bladder increased bladder capacity, reduced intravesical pressure and helped achieve continence in more than two thirds of the sample population. Mirabegron was safe and well tolerated by children.

Laparoscopic Nephro-Sparing Surgery of a Reninoma Tumor in a Pediatric Patient.

OBJETIVE: Reninoma, or juxtaglomerular cell tumor, is a benign neoplasm which causes severe hypertension with elevated plasma renin activity.1-3 Usually, it is well localized on computed tomography scan or magnetic resonance images, and complete resection of the tumor with renal function preservation is the optimal treatment.2-4 To describe the technique of a transperitoneal nephron-sparing laparoscopic surgery in a pediatric patient. MATERIAL AND METHODS: Describe the technique of a minimally invasive nephron-sparing surgery for resection of a reninoma tumor in a child. RESULTS: In a 14-year-old girl with history of severe high blood pressure, ultrasound detected a hypoechoic lesion in right kidney. Angio computed tomography scan confirmed a 20-mm diameter lesion in the anterior surface of the lower pole of right kidney and also informed a lower pole renal artery. Transperitoneal laparoscopic approach was performed using 4 ports. After mobilization of the colon, Gerota's fascia was opened and the tumor identified. Both polar and principal renal arteries were dissected and secured with vessel loops. The tumor was resected using an ultrasonic energy device. Selective clamping of the lower pole renal artery was performed during 8 minutes. The transected renal surface was closed with two continuous barbed sutures and the tumor was removed using a handmade endobag. Perirenal drainage was left for 4 days, there were no perioperative complications and the patient was discharged 2 days after surgery. Pathology confirmed typical reninoma immunohistochemical features. Blood pressure and plasma renin levels returned to normal. Postsurgical scintigraphy informed 40% differential function of the right kidney. CONCLUSION: Reninoma is a benign renal tumor and because of its nature and localization, minimally invasive nephron-sparing surgery should be considered. Strategic planning of the surgery based on preoperative images is essential. If vessel clamping is imperative, minimizing ischemia time and/or selective clamping, when possible, help preserve renal function.