EAU Guidelines on Paediatric Urology C. Radmayr (Chair), G. Bogaert, H.S. Dogan, R. Kocˇvara, J.M. Nijman (Vice-chair), R. Stein, S. Tekgül Guidelines Associates: L.A. ‘t Hoen, J. Quaedackers, M.S. Silay, S. Undre European Society for Paediatric Urology © European Association of Urology 2018 TABLE OF CONTENTS PAGE 1. INTRODUCTION 8 1.1 Aim 8 1.2 Panel composition 8 1.3 Available publications 8 1.4 Publication history 8 1.5 Summary of changes 8 1.5.1 New and changed recommendations 9 2. METHODS 10 2.1 Introduction 10 2.2 Peer review 11 2.3 Future goals 11 3. THE GUIDELINE 11 3.1 Phimosis 11 3.1.1 Epidemiology, aetiology and pathophysiology 11 3.1.2 Classification systems 11 3.1.3 Diagnostic evaluation 12 3.1.4 Management 12 3.1.5 Follow-up 12 3.1.6 Summary of evidence and recommendations for the management of phimosis 12 3.2 Management of undescended testes 13 3.2.1 Background 13 3.2.2 Classification 13 3.2.2.1 Palpable testes 14 3.2.2.2 Non-palpable testes 14 3.2.3 Diagnostic evaluation 14 3.2.3.1 History 14 3.2.3.2 Physical examination 14 3.2.3.3 Imaging studies 14 3.2.4 Management 15 3.2.4.1 Medical therapy 15 3.2.4.1.1 Medical therapy for testicular descent 15 3.2.4.1.2 Medical therapy for fertility potential 15 3.2.4.2 Surgical therapy 16 3.2.4.2.1 Palpable testes 16 3.2.4.2.1.1 Inguinal orchidopexy 16 3.2.4.2.1.2 Scrotal orchidopexy 16 3.2.4.2.2 Non-palpable testes 16 3.2.4.2.3 Complications of surgical therapy 17 3.2.4.2.4 Surgical therapy for undescended testes after puberty 17 3.2.5 Undescended testes and fertility 18 3.2.6 Undescended testes and malignancy 18 3.2.7 Summary of evidence and recommendations for the management of undescended testes 18 3.3 Hydrocele 19 3.3.1 Epidemiology, aetiology and pathophysiology 19 3.3.2 Diagnostic evaluation 19 3.3.3 Management 19 3.3.4 Summary of evidence and recommendations for the management of hydrocele 20 3.4 Acute scrotum 20 3.4.1 Epidemiology, aetiology and pathophysiology 20 3.4.2 Diagnostic evaluation 20 3.4.3 Management 21 3.4.3.1 Epididymitis 21 3.4.3.2 Testicular torsion 21 3.4.3.3 Surgical treatment 21 3.4.4 Follow-up 22 2 PAEDIATRIC UROLOGY - LIMITED UPDATE MARCH 2018 3.4.4.1 Fertility 22 3.4.4.2 Subfertility 22 3.4.4.3 Androgen levels 22 3.4.4.4 Unanswered questions 22 3.4.5 Summary of evidence and recommendations for the management of acute scrotum in 22 3.5 Hypospadias 23 3.5.1 Epidemiology, aetiology and pathophysiology 23 3.5.1.1 Epidemiology 23 3.5.2 Risk factors 23 3.5.3 Classification systems 23 3.5.4 Diagnostic evaluation 23 3.5.5 Management 24 3.5.5.1 Indication for reconstruction and therapeutic objectives 24 3.5.5.2 Pre-operative hormonal treatment 24 3.5.5.3 Age at surgery 24 3.5.5.4 Penile curvature 24 3.5.5.5 Urethral reconstruction 25 3.5.5.6 Re-do hypospadias repairs 25 3.5.5.7 Penile reconstruction following formation of the neo-urethra 26 3.5.5.8 Urine drainage and wound dressing 26 3.5.5.9 Outcome 26 3.5.6 Follow-up 27 3.5.7 Summary of evidence and recommendations for the management of hypospadias 28 3.6 Congenital penile curvature 28 3.6.1 Epidemiology, aetiology and pathophysiology 28 3.6.2 Diagnostic evaluation 28 3.6.3 Management 28 3.6.4 Summary of evidence and recommendations for the management of congenital penile curvature 29 3.7 Varicocele in children and adolescents 29 3.7.1 Epidemiology, aetiology and pathophysiology 29 3.7.2 Classification systems 30 3.7.3 Diagnostic evaluation 30 3.7.4 Management 30 3.7.5 Summary of evidence and recommendations for the management of varicocele 31 3.8 Urinary tract infections in children 31 3.8.1 Epidemiology, aetiology and pathophysiology 31 3.8.2 Classification systems 32 3.8.2.1 Classification according to site 32 3.8.2.2 Classification according to episode 32 3.8.2.3 Classification according to severity 32 3.8.2.4 Classification according to symptoms 32 3.8.2.5 Classification according to complicating factors 32 3.8.3 Diagnostic evaluation 33 3.8.3.1 Medical history 33 3.8.3.2 Clinical signs and symptoms 33 3.8.3.3 Physical examination 33 3.8.3.4 Urine sampling, analysis and culture 33 3.8.3.4.1 Urine sampling 33 3.8.3.4.2 Urinalysis 34 3.8.3.4.3 Urine culture 34 3.8.3.5 Imaging 35 3.8.3.5.1 Ultrasound 35 3.8.3.5.2 Radionuclide scanning 35 3.8.3.5.3 Voiding cystourethrography 35 3.8.3.6 Bladder and bowel dysfunction 35 3.8.4 Management 35 3.8.4.1 Administration route 35 PAEDIATRIC UROLOGY - LIMITED UPDATE MARCH 2018 3 3.8.4.2 Duration of therapy 36 3.8.4.3 Antimicrobial agents 37 3.8.4.4 Chemoprophylaxis 39 3.8.4.5 Monitoring of UTI 40 3.8.5 Summary of evidence and recommendations for the management of UTI in children 40 3.9 Day-time lower urinary tract conditions 41 3.9.1 Terminology, classification, epidemiology and pathophysiology 41 3.9.1.1 Filling-phase (storage) dysfunctions 42 3.9.1.2 Voiding-phase (emptying) dysfunctions 42 3.9.2 Diagnostic evaluation 42 3.9.3 Management 43 3.9.3.1 Specific interventions 44 3.9.4 Summary of evidence and recommendations for the management of day-time lower urinary tract conditions 45 3.10 Monosymptomatic nocturnal enuresis - bedwetting 45 3.10.1 Epidemiology, aetiology and pathophysiology 45 3.10.2 Diagnostic evaluation 45 3.10.3 Management 46 3.10.3.1 Supportive treatment measures 46 3.10.3.2 Conservative wait and see approach 46 3.10.3.3 Nocturnal enuresis wetting alarm treatment 46 3.10.3.4 Medical therapy 46 3.10.4 Summary of evidence and recommendations for the management of monosymptomatic enuresis 47 3.11 Management of neurogenic bladder 48 3.11.1 Epidemiology, aetiology and pathophysiology 48 3.11.2 Classification systems 48 3.11.3 Diagnostic evaluation 49 3.11.3.1 History and clinical evaluation 49 3.11.3.2 Laboratory & Urinalysis 49 3.11.3.3 Ultrasound 49 3.11.3.4 Urodynamic studies/videourodynamic 49 3.11.3.4.1 Preparation before urodynamic studies 49 3.11.3.4.2 Uroflowmetry 50 3.11.3.5 Urodynamic studies 50 3.11.3.6 Voiding cystourethrogram 50 3.11.3.7 Renal scan 50 3.11.4 Management 50 3.11.4.1 Early management with intermittent catheterisation 50 3.11.4.2 Medical therapy 50 3.11.4.3 Management of faecal incontinence 52 3.11.4.4 Urinary tract infection 52 3.11.4.4.1 Urinary tract infection and clean intermittent catherisation 52 3.11.4.5 Sexuality 52 3.11.4.6 Bladder augmentation 53 3.11.4.7 Bladder outlet procedures 53 3.11.4.8 Catheterisable cutaneous channel. 54 3.11.4.9 Continent and incontinent cutaneous urinary diversion 54 3.11.5 Follow-up 55 3.11.6 Self-organisation of patients 55 3.11.7 Summary of evidence and recommendations for the management of neurogenic bladder 57 3.12 Dilatation of the upper urinary tract (UPJ and UVJ obstruction) 58 3.12.1 Epidemiology, aetiology and pathophysiology 58 3.12.2 Diagnostic evaluation 58 3.12.2.1 Antenatal ultrasound 58 3.12.2.2 Postnatal ultrasound 58 3.12.2.3 Voiding cystourethrogram 58 4 PAEDIATRIC UROLOGY - LIMITED UPDATE MARCH 2018 3.12.2.4 Diuretic renography 58 3.12.3 Management 59 3.12.3.1 Prenatal management 59 3.12.3.1.1 Antibiotic prophylaxis for antenatal hydronephrosis 59 3.12.3.2 UPJ obstruction 60 3.12.3.3 Megaureter 60 3.12.3.3.1 Non-operative management 60 3.12.3.3.2 Surgical management 60 3.12.4 Conclusion 60 3.12.5 Summary of evidence and recommendations for the management of UPJ-, UVJ-obstruction 61 3.13 Vesicoureteric reflux 61 3.13.1 Epidemiology, aetiology and pathophysiology 61 3.13.2 Diagnostic evaluation 62 3.13.2.1 Infants presenting because of prenatally diagnosed hydronephrosis 63 3.13.2.2 Siblings and offspring of reflux patients 63 3.13.2.3 Recommendations for paediatric screening of VUR 64 3.13.2.4 Children with febrile urinary tract infections 64 3.13.2.5 Children with lower urinary tract symptoms and vesicoureteric reflux 64 3.13.3 Disease management 64 3.13.3.1 Non-surgical therapy 64 3.13.3.1.1 Follow-up 65 3.13.3.1.2 Continuous antibiotic prophylaxis 65 3.13.3.2 Surgical treatment 65 3.13.3.2.1 Subureteric injection of bulking materials 65 3.13.3.2.2 Open surgical techniques 66 3.13.3.2.3 Laparoscopy and robot-assisted 66 3.13.4 Summary of evidence and recommendations for the management of vesicoureteric reflux in childhood 66 3.14 Urinary stone disease 68 3.14.1 Epidemiology, aetiology and pathophysiology 68 3.14.2 Classification systems 68 3.14.2.1 Calcium stones 69 3.14.2.2 Uric acid stones 70 3.14.2.3 Cystine stones 70 3.14.2.4 Infection stones (struvite stones) 70 3.14.3 Diagnostic evaluation 71 3.14.3.1 Imaging 71 3.14.3.2 Metabolic evaluation 71 3.14.4 Management 72 3.14.4.1 Extracorporeal shockwave lithotripsy 73 3.14.4.2 Percutaneous nephrolithotomy (PCNL) 74 3.14.4.3 Ureterorenoscopy 74 3.14.4.4 Open or laparoscopic stone surgery 75 3.14.5 Summary of evidence and recommendations for the management of urinary stones 76 3.15 Obstructive pathology of renal duplication: ureterocele and ectopic ureter 76 3.15.1 Epidemiology, aetiology and pathophysiology 76 3.15.1.1 Ureterocele 76 3.15.1.2 Ectopic ureter 76 3.15.2 Classification systems 76 3.15.2.1 Ureterocele 76 3.15.2.1.1 Ectopic (extravesical) ureterocele 77 3.15.2.1.2 Orthotopic (intravesical) ureterocele 77 3.15.2.2 Ectopic ureter 77 3.15.3 Diagnostic evaluation 77 3.15.3.1 Ureterocele 77 3.15.4 Management 78 3.15.4.1 Ureterocele 78 3.15.4.1.1 Early treatment 78 PAEDIATRIC UROLOGY - LIMITED UPDATE MARCH 2018 5 3.15.4.1.2 Re-evaluation 78 3.15.4.2 Ectopic ureter 79 3.15.5 Summary of evidence and recommendations for the management of obstructive pathology of renal duplication: ureterocele and ectopic ureter 79 3.16 Disorders of sex development 80 3.16.1 Epidemiology, aetiology and pathophysiology 80