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Cystinuria, which is the cause of cystine urolithiasis, accounts for 2-6% of all urinary stones in children, has a low prevalence with a high recurrence rate, making this metabolic disorder a therapeutic challenge in pediatric population. The aim of this work is evaluate the efficacy and safety of retrograde intra-renal surgery (RIRS) in pediatric patients with cystinuria and kidney stones smaller than 2 cm. Prospective study of 64 stones treated in 22 retrograde intra-renal surgeries (RIRS) in cystinuric pediatric patients with renal or proximal ureteral stones less than 2 cm. Average age of 9.5 years. The following data were analyzed: demographics, stone characteristics and surgical data with intra- and postoperative complications. Location of the stones was 68.7% in the calyces, 20.3% in the renal pelvis, and 9.3% in the ureteropelvic junction; 41% of cases had multiple locations. The average cystinuria level before the procedure was 825 mg/dL. The anatomy of 73% of the interventions increased the difficulty of flexible ureteroscopy and decreased stone free rates, because distorted renal anatomy was present: sclerosis of the pelvis or infundibulum, abnormal calyceal dilations, or excluded calyces. Intraoperative complications occurred in 18.2% of the procedures. Reno-vesical ultrasound was performed in all patients in the first postoperative month, with an SFR of 59%. Cystinuric patients are a challenge for pediatric urologists, decreasing the effectiveness of RIRS. However, it could be better treatment than SWL and with fewer complications than PCNL in the pediatric population with this disease.

Aim: To assess and compare the outcomes associated with ureteroscopy and laser fragmentation (URSL) for extremes of age group (≤10 and ≥80 years). Methods: Retrospective consecutive data were collected from two European centres for all paediatric patients ≤10 undergoing URSL over a 15-year period (group 1). It was compared to consecutive data for all patients ≥80 years (group 2). Data were collected for patient demographics, stone characteristics, operative details, and clinical outcomes. Results: A total of 168 patients had 201 URSL procedures during this time (74 and 94 patients in groups 1 and 2 respectively). The mean age and stone sizes were 6.1 years and 85 years, and 9.7 mm and 13 mm for groups 1 and 2 respectively. While the SFR was slightly higher in group 2 (92.5% versus 87.8%, p = 0.301), post-operative stent rate was also significantly higher in the geriatric population (75.9% versus 41.2%, p = 0.0001). There was also no significant difference in pre-operative stenting (p = 0.886), ureteric access sheath use (UAS) (p = 0.220) and post-operative complications. Group 1 had an intervention rate of 1.3/patient as compared to 1.1/patient in group 2. The overall complications were 7.2% and 15.3% in groups 1 and 2 respectively (0.069), with 1 Clavien IV complication related to post-operative sepsis and brief ICU admission in group 2. Conclusion: The paediatric population had a marginally higher incidence of repeat procedure, but the overall SFR and complications were similar, and post-operative stent insertion rates were much better compared to geriatric patients. URSL is a safe procedure in the extremes of age groups with no difference in the overall outcomes between the two groups.

Introduction: Incidence of urolithiasis in children has increased in recent years and with technological advancements and miniaturization of surgical instruments, pediatric urologists have acquired an impressive arsenal for their treatment. Retrograde intrarenal surgery (RIRS) has gained widespread popularity as it is a natural extension of semirigid ureteroscopy and can be done through natural orifice minimizing the morbidity of percutaneous access. The aim of this narrative review is to describe how RIRS has evolved over the decades in children and if the age-related anatomical difference impacts reported outcomes especially stone-free rate (SFR) and complications. Materials and Methods: An electronic literature search from inception to October 15, 2021 was performed using Medical Subject Heading terms in several combinations on PubMed, EMBASE, and Web of Science without language restrictions. A total of 2022 articles were founded and 165 articles were full-text screening. Finally, 2 pediatric urologists included 51 articles that summarize the available literature regarding the development and use of RIRS in children. Results: RIRS as of today is well established as a superior modality for all stones in all locations compared with extracorporeal shockwave lithotripsy both in children and adults. The passive dilation has decreased the need of active ureteral dilation, but the need to perform prestenting is not defined yet. Regarding the use of the ureteral access sheath, the literature tends to lean toward its placement in most cases, but we do not know its long-term effects over the growth of children. Finally, the SFR has increased as the experience of pediatric urologists increases, as well as the number of complications has decreased. Conclusion: RIRS in pediatrics has crossed many milestones, yet many areas need further research and larger data are required to make RIRS the procedure of choice for renal stone management in children across all age groups.

Introduction: Although pediatric ureteroscopy has been increasingly performed, the evidence for its use in pediatric lower pole stones (LPS) is sparce. In this study we look at the effectiveness and outcomes of flexible ureteroscopy and laser lithotripsy (FURSL) in the management of LPS for a pediatric population. Materials and Methods: Data were collected from two large European tertiary endourology centers that specialize in pediatric kidney stone management. The study was registered as an audit at the respective hospitals. All data were cross-checked and analyzed using electronic operative notes, discharge records, laboratory systems, and patient correspondence. The inclusion criteria were patients

Background Inherited kidney diseases are one of the leading causes of chronic kidney disease (CKD) that manifests before the age of 30 years. Precise clinical diagnosis of early-onset CKD is complicated due to the high phenotypic overlap, but genetic testing is a powerful diagnostic tool. We aimed to develop a genetic testing strategy to maximize the diagnostic yield for patients presenting with early-onset CKD and to determine the prevalence of the main causative genes. Methods We performed genetic testing of 460 patients with early-onset CKD of suspected monogenic cause using next-generation sequencing of a custom-designed kidney disease gene panel in addition to targeted screening for c.428dupC MUC1. Results We achieved a global diagnostic yield of 65% (300/460), which varied depending on the clinical diagnostic group: 77% in cystic kidney diseases, 76% in tubulopathies, 67% in autosomal dominant tubulointerstitial kidney disease, 61% in glomerulopathies and 38% in congenital anomalies of the kidney and urinary tract. Among the 300 genetically diagnosed patients, the clinical diagnosis was confirmed in 77%, a specific diagnosis within a clinical diagnostic group was identified in 15%, and 7% of cases were reclassified. Of the 64 causative genes identified in our cohort, 7 (COL4A3, COL4A4, COL4A5, HNF1B, PKD1, PKD2 and PKHD1) accounted for 66% (198/300) of the genetically diagnosed patients. Conclusions Two-thirds of patients with early-onset CKD in this cohort had a genetic cause. Just seven genes were responsible for the majority of diagnoses. Establishing a genetic diagnosis is crucial to define the precise aetiology of CKD, which allows accurate genetic counselling and improved patient management.

Se ha informado que la trombosis de un vaso sanguíneo extraespermático es una causa inusual de escroto agudo. Describimos el caso de un niño de 7 años que presentó una hinchazón escrotal izquierda dolorosa aguda. Las imágenes demostraron una masa escrotal izquierda heterogénea, que fue resecada quirúrgicamente. El estudio anatomopatológico reveló una estructura vascular trombosada. Tres meses después, se produjo otra trombosis venosa extraespermática y también se resecó. Se realizó un estudio completo de trombofilia. Se identificó una mutación heterocigótica del gen de protrombina G20210A como la causa del evento trombótico. Una trombosis venosa debe considerarse como una posible causa de escroto agudo en pacientes europeos caucásicos, debido a su mayor prevalencia. Revisamos la mutación del gen de protrombina G20210A, que está asociada con la trombosis. Thrombosis of an extraspermatic blood vessel has been reported to be an unusual cause of acute scrotum. We describe the case of a 7-year-old boy who presented with an acute painful left scrotal swelling. Imaging demonstrated a heterogeneous left scrotal mass, which was surgically resected. The anatomopathologic study revealed a thrombosed vascular structure. Three months later, another extraspermatic venous thrombosis occurred and was also resected. A complete thrombophilia study was done. A G20210A prothrombin gene heterozygous mutation was identified as the cause of the thrombotic event. A venous thrombosis should be considered as a possible cause of acute scrotum in European Caucasian patients, due to its increased prevalence. We reviewed the G20210A prothrombin gene mutation, which is associated with thrombosis.

Medical and surgical priorities have changed dramatically following the COVID-19 pandemic declaration. The rapid spread of the virus and the high number of cases has saturated the health system in our country and has forced many hospitals to redistribute resources to care for infected patients. This has led to asignificant reduction in surgical activity, in some cases reaching the point of delaying all elective interventions by performing only urgent interventions. The decrease in the number of infections with the progressive desaturation of hospitals has currently allowed us to enter a new phase of "de-escalation" or transition in order to recover our surgical activity in pediatric urology, which was practically canceled. It is proposed how surgical care activities such as outpatient care should be deal twith, in addition to the different circuits that patients must maintain and, above all, their and professional safety.Las prioridades médicas y quirúrgicas han cambiado drásticamente tras la declaración de pandemia de COVID-19. La rápida propagación del virus y el elevado número de casos ha saturado el sistema sanitario de nuestro país y obligado a muchos hospitales a redistribuir sus recursos para atender a los pacientes infectados. Esto ha llevado a una reducción significativa de la actividad quirúrgica llegando al extremo de demorar todas las intervenciones electivas, realizándose únicamente las intervenciones urgentes. La disminución del número de contagios con la progresiva desaturación de los hospitales nos ha permitido en la actualidad entrar en una nueva fase de “desescalada” o de transición para poder recuperar nuestra actividad quirúrgica dentro de la urología pediátrica que fue prácticamente cancelada. Se propone cómo se debe afrontar la actividad asistencial quirúrgica así como la actividad de consultas externas, además de establecer los distintos circuitos que deben mantener los pacientes para su seguridad y la de los profesionales que los atienden.

Medical and surgical priorities have changed dramatically at the time of this pandemic. Scientific societies around the World have provided rapid guidance, underpinned by the best knowledge available, on the adaptation of their guidelines recommendations to the current situation. There are very limited scientific evidence especially in our subspecialty of pediatric urology. We carry out a review of the little scientific evidence based mainly on the few publications available to date and on the recommendations of the main scientific societies regarding which patients should undergo surgery, when surgery should be performed and how patient visits should be organize.

We present an extremely rare case of a 3-year-old child with a primary carcinoid tumor of the prostate. A 3-year-old boy presented with failure to thrive, constipation, recurrent respiratory tract infections, and pain in the genital area. His karyotype was normal and cystic fibrosis and coeliac disease were excluded prior to further investigation. An abdominopelvic computed tomography scan revealed a prostatic mass. Transrectal ultrasound-guided prostate biopsy was therefore performed and pathological examination revealed a carcinoid tumor. A robotic radical prostatectomy was performed. As this is an innovative surgical approach, we describe the surgical technique used.

A nine months old girl with prenatal diagnosis of bilateral hydronephrosis underwent serious studies and in view of the presumed diagnosis of right functional annulment, a right laparoscopic nephroureterectomy was performed. The anatomopatological result was suggestive of tuberculosis without previous contact with the disease. Postoperatively, the child received antituberculosis therapy (ATT) for a full 8 months (isoniazid, rifampin, pyrazinamide and ethambutol for 2 months and isoniazid and rifampin for 6 months) and the follow-up tests revealed improvement of ureterohydronephrosis in left kidney.

To perform a retrospective evaluation of surgical complications and morbidity in patients undergoing radical retropubic prostatectomy (RRP) as elective treatment for organ-confined prostate cancer in our center with previous transurethral resection of the prostate (TURP).Between 1980-2004 we performed 59 radical prostatectomies in patients with previous TURP. We analyze the morbidity and mortality of the RRP, its functional outcomes, and the accordance between clinical and pathological stage.Mean time between TURP and RRP was 16 months. Mean patient age at the time of diagnosis was 63 years. Clinical stage: 16 T1a, 18T1b, 20T1c, 3T2a, 2T2b. Average surgical time was 180 minutes. Intraoperative events: technical difficulties in the dissection of the gland 57%, bladder neck preservation 27%, neuro vascular bundles preservation 3.39%, ureter ligation 1.69%, rectal laceration 1.69, urethrorrhagia 1.69%, urinary leak 5%, and blood transfusions 11.8%. Postoperative complications: urinary tract infection 10.17%, wound infection 10.17%, pelvic hematoma 5.08%, deep vein thrombosis 1.69%, and one sudden death of unknown cause one month after surgery. Pathologic report: 49pT2b, 8 pT3 and 2pT4. Late complications: erectile dysfunction 85.7%, vesicourethral anastomosis stenosis 10.3%, and complete urinary incontinence 3%.Radical retropubic prostatectomy in patients with previous TURP is technically more difficult and has comparable outcomes to RRP patients without previous TURP.

Objetivo: Nuestro objetivo ha sido realizar una valoración retrospectiva en nuestro centro de las complicaciones quirúrgicas y de morbilidad en pacientes intervenidos de prostatectomía radical retropúbica como tratamiento electivo del cáncer de próstata órgano confinado, con antecedentes de resección transuretral de próstata (RTUp). Métodos: Entre 1980-2004 hemos realizado 59 prostatectomías radicales en pacientes con RTUp previa. Se analiza morbimortalidad de la PRR, resultados funcionales y concordancia entre estadiaje clínico y patológico. Resultados: El tiempo medio transcurrido entre la RTUp y la prostatectomía radical 16 meses. Edad media en el momento de diagnóstico de adenocarcinoma de próstata 63 años. Estadiaje clínico: 16T1a, 18T1b, 20T1c, 3 T2a, 2 T2b. La media de tiempo quirúrgico de la PRR 180 minutos. Incidencias intraoperatorias: Dificultades técnicas en la disección de la glándula 57%, preservación del cuello vesical 27%, preservación de bandeletas neurovasculares 3.39%, ligadura de uréter 1.69%, lesión rectal 1.69%, uretrorragia 1.69%, fístula urinaria 5% y el 11.8% requirió transfusión. Como complicaciones postoperatorias: ITU 10.17%, infección de la herida 10.17%, hematoma pélvico 5.08%, TVP 1.69% y un paciente falleció súbitamente al mes de la cirugía por causa no filiada. Estadío patológico: 49pT2b, 8pT3 y 2pT4. Como complicaciones tardías: Disfunción eréctil 85.7%, estenosis de la anastomosis 10.3% e incontinencia urinaria total 3%. Conclusión: La PRR en pacientes con RTUp previa es técnicamente más dificultosa con resultados comparables con los pacientes sin cirugía prostática previa

Acute bacterial prostatitis (ABP) (NIH Category I), has not undergone any modification in the update of prostatitis classification. ABP was diagnosed in 614 patients in our centre over 9 years (1993-2001). We analyse the clinical pattern of ABP and the role of bladder outlet obstruction in its etiology, as well as whether two different ABP sub-categories could be defined as a function of a history of previous manipulation of the lower urinary tract. The results of the study show that the clinical pattern of a patient suffering from ABP does not differ from the statements of previous publications. On the other hand, patients with ABP have been shown to present with no bladder outlet obstruction. Finally, this study has disclosed the fact that the cases of ABP elicited by previous manipulation of the lower urinary tract (10%) show a different pattern from those cases where no previous manipulation has occurred (90%). The patients with ABP secondary to manipulation are older, have a higher risk of prostate abscess and higher frequency of multiple infections and also infections by pathogens other than Escherichia coli. Due to all of these reasons, it would be advisable to subdivide category I within the classification of prostatitis.

To report a very unfrequent case of penile hematoma secondary to rupture of the superficial dorsal vein of the penis during intercourse.35-year-old patient presenting at the emergency department with a half moon shape hematoma in the dorsal part of the penis, not much painful, developed when initiating intercourse that we believe was a rupture of the superficial dorsal vein of the penis.In spite of the few cases of rupture of the superficial dorsal vein of the penis described, and after differential diagnosis with other causes of acute penis, conservative attitude with anti-inflammatory drugs and compressive bandage was undertaken.The rupture of the superficial dorsal vein of the penis is an unfrequent pathology which should be taken into differential diagnosis in a patient consulting with acute penile edema, the attitude is conservative with complete recovery without sequel.

Purpose To analyse and report the practice, outcomes and lessons learnt from a global series of retrograde intrarenal surgery (RIRS) in a paediatric multicentre series. Methods A retrospective review of anonymized pooled data gathered globally from 8 centres in paediatric patients ( 10 years (Group C). Overall, post-operative complication rate was 13.7%. Chi-square comparisons were used for categorical variables; analysis of variance (ANOVA) or Kruskal-Wallis tests were used for continuous variables. Results 314 patients were analysed. The mean age was 9.54 +/- 4.76 years. Groups A, B and C had 67 (21.3%), 83 (26.4%) and 164 (52.2%) patients, respectively. Mean stone size was 10.7 +/- 4.62 mm. Pre-stenting was performed in 155 (49.4%) of patients, ureteral access sheaths (UAS) was used in 54.5% of patients with majority (71%) utilizing holmium laser for stone fragmentation. All complications were minor (Clavien-Dindo grade 1 and 2). SFR was 75.5%. Conclusions RIRS is acceptable as a first-line intervention in the paediatric population with reasonable efficacy and low morbidity. Complications are slightly higher in patients < 5 years of age, which should be taken into account while counselling patients.