Guidelines on Paediatric Urology S. Tekgül, H. Riedmiller, H.S. Dogan, P. Hoebeke, R. Kocvara, R. Nijman, Chr. Radmayr, R. Stein European Society for Paediatric Urology © European Association of Urology 2013 TABLE OF CONTENTS PAGE 1. METHODOLOGY 7 1.1 Introduction 7 1.2 Data identification and evidence sources 7 1.3 Level of evidence and grade of recommendation 7 1.4 Publication history 8 1.4.1 Summary of updated and new information 8 1.5 Potential conflict of interest statement 8 1.6 References 9 2. PHIMOSIS 9 2.1 Background 9 2.2 Diagnosis 9 2.3 Treatment 9 2.4 Conclusions and recommendations on phimosis 10 2.5 References 10 3. CRYPTORCHIDISM 11 3.1 Background 11 3.2 Diagnosis 11 3.3 Treatment 12 3.3.1 Medical therapy 12 3.3.2 Surgery 12 3.4 Prognosis 12 3.5 Recommendations for cryptorchidism 13 3.6 References 13 4. HYDROCELE 14 4.1 Background 14 4.2 Diagnosis 15 4.3 Treatment 15 4.4 Recommendations for the management of hydrocele 15 4.5 References 15 5. ACUTE SCROTUM IN CHILDREN 16 5.1 Background 16 5.2 Diagnosis 16 5.3 Treatment 17 5.3.1 Epididymitis 17 5.3.2 Testicular torsion 17 5.3.3 Surgical treatment 17 5.4 Prognosis 17 5.4.1 Fertility 17 5.4.2 Subfertility 18 5.4.3 Androgen levels 18 5.4.4 Testicular cancer 18 5.4.5 Nitric oxide 18 5.5 Perinatal torsion 18 5.6 Recommendations acute scrotum in children 18 5.7 References 19 6. Hypospadias 22 6.1 Background 22 6.1.1 Risk factors 22 6.2 Diagnosis 22 6.3 Treatment 22 6.3.1 Age at surgery 23 6.3.2 Penile curvature 23 6.3.3 Preservation of the well-vascularized urethral plate 23 6.3.4 Re-do hypospadias repairs 24 6.3.5 Urethral reconstruction 24 2 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 6.3.6 Urine drainage and wound dressing 24 6.3.7 Outcome 24 6.4 Conclusions and recommendations 25 6.5 References 25 7. CONGENITAL PENILE CURVATURE 28 7.1 Background 28 7.2 Diagnosis 28 7.3 Treatment 28 7.4 References 28 8. VARICOCELE IN CHILDREN AND ADOLESCENTS 28 8.1 Background 28 8.2 Diagnosis 29 8.3 Therapy 29 8.4 Conclusions and recommendations 30 8.5 References 30 9. URINARY TRACT INFECTIONS IN CHILDREN 31 9.1 Introduction 31 9.2 Classification 32 9.2.1 Classification according to site 32 9.2.2 Classification according to episode (10) 32 9.2.3 Classification according to severity 32 9.2.4 Classification according to symptoms 32 9.2.5 Classification according to complicating factors (12) 33 9.3 Diagnosis 33 9.3.1 Medical history 33 9.3.2 Clinical signs and symptoms 33 9.3.3 Physical examination 33 9.4 Urine sampling, analysis and culture 33 9.4.1 Urine sampling 33 9.4.2 Urinalysis 34 9.4.3 Urine culture 35 9.5 Therapy 35 9.5.1 Administration route 35 9.5.2 Duration of therapy 35 9.5.3 Antimicrobial agents 36 9.5.4 Chemoprophylaxis 38 9.6 Monitoring of UTI 38 9.7 Imaging 38 9.7.1 Ultrasound 38 9.7.2 Radionuclide scanning 38 9.7.3 Voiding cystourethrography 39 9.8 Bladder and bowel dysfunction 39 9.9 Conclusions and recommendations for UTI in children 39 9.10 References 40 10. DAYTIME LOWER URINARY TRACT CONDITIONS 44 10.1 Background 44 10.2 Definition 44 10.2.1 Filling-phase dysfunctions 45 10.2.2 Voiding-phase (emptying) dysfunctions 45 10.3 Diagnosis 45 10.4 Treatment 45 10.4.1 Standard therapy 45 10.4.2 Specific interventions 46 10.5 References 46 11. MONOSYMPTOMATIC ENURESIS 47 11.1 Background 47 11.2 Definition 48 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 3 11.3 Diagnosis 48 11.4 Treatment 48 11.4.1 Supportive treatment measures 48 11.4.2 Alarm treatment 48 11.4.3 Medication 49 11.5 References 50 12. MANAGEMENT OF NEUROGENIC BLADDER IN CHILDREN 50 12.1 Background 50 12.2 Definition 51 12.3 Classification 51 12.4 Urodynamic studies 51 12.4.1 Method of urodynamic study 52 12.4.2 Uroflowmetry 52 12.4.3 Cystometry 52 12.5 Management 53 12.5.1 Investigations 53 12.5.2 Early management with intermittent catheterisation 53 12.5.3 Medical therapy 53 12.5.3.1 Botulinum toxin injections 53 12.5.4 Management of bowel incontinence 54 12.5.5 Urinary tract infection 54 12.5.6 Sexuality 54 12.5.7 Bladder augmentation 54 12.5.8 Bladder outlet procedures 54 12.5.9 Continent stoma 55 12.5.10 Total bladder replacement 55 12.5.11 Lifelong follow-up of neurogenic bladder patients 55 12.6 References 55 13. DILATATION OF THE UPPER URINARY TRACT (URETEROPELVIC JUNCTION AND URETEROVESICAL JUNCTION OBSTRUCTION) 61 13.1 Background 61 13.2 Diagnosis 61 13.2.1 Antenatal ultrasound 61 13.2.2 Postnatal ultrasound 61 13.2.3 Voiding cystourethrogram (VCUG) 61 13.2.4 Diuretic renography 61 13.3 Treatment 62 13.3.1 Prenatal management 62 13.3.2 UPJ obstruction 62 13.3.3 Megaureter 62 13.3.3.1 Conservative management 62 13.3.3.2 Surgical management 63 13.4 Conclusion 63 13.5 Conclusions and recommendations for UPJ-, UVJ-obstruction 63 13.6 References 63 14. VESICOURETERIC REFLUX IN CHILDREN 64 14.1 Methodology 64 14.2 Background 64 14.3 Diagnostic work-up 65 14.3.1 Infants presenting because of prenatally diagnosed hydronephrosis 66 14.3.2 Siblings and offspring of reflux patients 66 14.3.3 Children with febrile urinary tract infections 67 14.3.4 Children with lower urinary tract symptoms and vesicoureteric reflux 67 14.4 Treatment 67 14.4.1 Conservative therapy 67 14.4.1.1 Follow-up 68 14.4.1.2 Continuous antibiotic prophylaxis (CAP) 68 14.4.2 Surgical treatment 68 14.4.2.1 Subureteric injection of bulking materials 68 4 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 14.4.2.2 Open surgical techniques 69 14.4.2.3 Laparoscopy 69 14.5 Recommendations for the management of vesicoureteric reflux in childhood 69 14.6 References 71 15. URINARY STONE DISEASE 75 15.1 Background 75 15.2 Stone formation mechanisms, diagnosis of causative factors and medical treatment for specific stone types 75 15.2.1 Calcium stones 76 15.2.2 Uric acid stones 77 15.2.3 Cystine stones 77 15.2.4 Infection stones (struvite stones) 77 15.3 Clinical presentation 78 15.4 Diagnosis 78 15.4.1 Imaging 78 15.4.2 Metabolic evaluation 78 15.5 Management 80 15.5.1 Extracorporeal shock wave lithotripsy (SWL) 80 15.5.2 Percutaneous nephrolithotomy 80 15.5.3 Ureterorenoscopy 81 15.5.4 Open stone surgery 81 15.6 Conclusions and recommendations 82 15.7 References 83 16. OBSTRUCTIVE PATHOLOGY OF RENAL DUPLICATION: URETEROCELE AND ECTOPIC URETER 87 16.1 Background 87 16.1.1 Ureterocele 88 16.1.2 Ectopic ureter 88 16.2 Definition and classification 88 16.2.1 Ureterocele 88 16.2.1.1 Ectopic (extravesical) ureterocele 88 16.2.1.2 Orthotopic (intravesical) ureterocele 88 16.2.2 Ectopic ureter 88 16.3 Diagnosis 89 16.3.1 Ureterocele 89 16.3.2 Ectopic ureter 89 16.4 Treatment 89 16.4.1 Ureterocele 89 16.4.1.1 Early treatment 89 16.4.1.2 Re-evaluation 89 16.4.2 Ectopic ureter 90 16.5 Conclusions and recommendations for obstructive pathology of renal duplication: ureterocele and ectopic ureter 91 16.6 References 91 17. DISORDERS OF SEX DEVELOPMENT 94 17.1 Background 94 17.2 Micropenis 94 17.2.1 Background 94 17.2.2 Diagnosis 95 17.2.3 Treatment 95 17.3 The neonatal emergency 95 17.3.1 Family history and clinical examination 95 17.3.2 Choice of laboratory investigations 96 17.4 Gender assignment 96 17.5 Role of the paediatric urologist 96 17.5.1 Diagnosis 97 17.51.1 Clinical examination 97 17.5.1.2 Investigations 97 17.6 Management 97 17.6.1 Feminising surgery 98 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 5 17.6.2 Masculinising surgery 98 17.7 Guidelines for the treatment of disorders of sex development 98 17.8 References 99 18. POSTERIOR URETHRAL VALVES 100 18.1 Background 100 18.2 Classification 100 18.2.1 Urethral valve 100 18.3 Diagnosis 100 18.4 Treatment 101 18.4.1 Antenatal treatment 101 18.4.2 Postnatal treatment 101 18.5 Summary 104 18.6 Conclusions and recommendations posterior urethral valves 104 18.7 References 104 19. PAEDIATRIC UROLOGICAL TRAUMA 106 19.1 Background 106 19.2 Paediatric renal trauma 106 19.2.1 Diagnosis 107 19.2.1.1 Haematuria 107 19.2.1.2 Blood pressure 107 19.2.1.2 Choice of imaging method 107 19.2.2 Treatment 108 19.2.3 Guidelines for the diagnosis and treatment of paediatric renal trauma 108 19.3 Paediatric ureteral trauma 108 19.3.1 Diagnosis 108 19.3.2 Treatment 108 19.3.3 Guidelines for the diagnosis and treatment of paediatric ureteral trauma 109 19.4 Paediatric bladder injuries 109 19.4.1 Diagnosis 109 19.4.2 Treatment 109 19.4.2.1 Intraperitoneal injuries 109 19.4.2.2 Extraperitoneal injuries 109 19.4.3 Guidelines for the diagnosis and treatment of paediatric bladder injuries 110 19.5 Paediatric urethral injuries 110 19.5.1 Diagnosis 110 19.5.2 Treatment 110 19.5.3 Guidelines for the diagnosis and treatment of paediatric trauma 111 19.6 References 111 20.