Guidelines on Paediatric Urology S. Tekgül (chair), H.S. Dogan, P. Hoebeke, R. Kocvara, J.M. Nijman, Chr. Radmayr, R. Stein European Society for Paediatric Urology © European Association of Urology 2014 TABLE OF CONTENTS PAGE 1. METHODOLOGY 8 1.1 Introduction 8 1.2 Data ideication and evidence sources 8 1.3 Level of evidence and grade of recommendation 8 1.4 Publication history 9 1.4.1 Summary of updated and new information 9 1.5 Potential conflict of interest statement 9 1.6 References 9 2. PHIMOSIS 10 2.1 Background 10 2.2 Diagnosis 10 2.3 Treatment 10 2.4 Conclusions and recommendations on phimosis 11 2.5 References 11 3. CRYPTORCHIDISM 13 3.1 Background 13 3.2 Diagnosis 13 3.3 Treatment 13 3.3.1 Medical therapy 13 3.3.2 Surgery 13 3.4 Prognosis 14 3.5 Recommendations for cryptorchidism 14 3.6 References 14 4. HYDROCELE 16 4.1 Background 16 4.2 Diagnosis 16 4.3 Treatment 16 4.4 Recommendations for the management of hydrocele 17 4.5 References 17 5. ACUTE SCROTUM IN CHILDREN 17 5.1 Background 17 5.2 Diagnosis 18 5.3 Treatment 18 5.3.1 Epididymitis 18 5.3.2 Testicular torsion 18 5.3.3 Surgical treatment 19 5.4 Prognosis 19 5.4.1 Fertility 19 5.4.2 Subfertility 19 5.4.3 Androgen levels 19 5.4.4 Testicular cancer 19 5.4.5 Nitric oxide 19 5.5 Perinatal torsion 19 5.6 Recommendations for the treatment of acute scrotum in children 20 5.7 References 20 6. HYPOSPADIAS 23 6.1 Background 23 6.1.1 Risk factors 23 6.2 Diagnosis 24 6.3 Treatment 24 6.3.1 Age at surgery 24 6.3.2 Penile curvature 25 6.3.3 Preservation of the well-vascularized urethral plate 25 2 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 6.3.4 Re-do hypospadias repairs 25 6.3.5 Urethral reconstruction 26 6.3.6 Urine drainage and wound dressing 26 6.3.7 Outcome 26 6.4 Conclusions and recommendations for the management of hypospadias 26 6.5 References 27 7. CONGENITAL PENILE CURVATURE 30 7.1 Background 30 7.2 Diagnosis 30 7.3 Treatment 30 7.4 References 30 8. VARICOCELE IN CHILDREN AND ADOLESCENTS 31 8.1 Background 31 8.2 Diagnosis 31 8.3 Therapy 31 8.4 Conclusions and recommendations 32 8.5 References 32 9. URINARY TRACT INFECTIONS IN CHILDREN 34 9.1 Introduction 34 9.2 Classification 34 9.2.1 Classification according to site 34 9.2.2 Classification according to episode 34 9.2.3 Classification according to severity 35 9.2.4 Classification according to symptoms 35 9.2.5 Classification according to complicating factors 35 9.3 Diagnosis 35 9.3.1 Medical history 35 9.3.2 Clinical signs and symptoms 35 9.3.3 Physical examination 35 9.4 Urine sampling, analysis and culture 35 9.4.1 Urine sampling 35 9.4.2 Urinalysis 36 9.4.3 Urine culture 37 9.5 Therapy 37 9.5.1 Administration route 37 9.5.2 Duration of therapy 38 9.5.3 Antimicrobial agents 39 9.5.4 Chemoprophylaxis 40 9.6 Monitoring of UTI 41 9.7 Imaging 41 9.7.1 Ultrasound 41 9.7.2 Radionuclide scanning 41 9.7.3 Voiding cystourethrography 41 9.8 Bladder and bowel dysfunction 41 9.9 Conclusions and recommendations for UTI in children 42 9.10 References 43 10. DAYTIME LOWER URINARY TRACT CONDITIONS 47 10.1 Background 47 10.2 Definition 48 10.2.1 Filling-phase dysfunctions 48 10.2.2 Voiding-phase (emptying) dysfunctions 48 10.3 Diagnosis 48 10.4 Treatment 49 10.4.1 Standard therapy 49 10.4.2 Specific interventions 49 10.5 Recommendations for the treatment of daytime lower urinary tract conditions 49 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 3 10.6 References 49 11. MONOSYMPTOMATIC ENURESIS 51 11.1 Background 51 11.2 Definition 51 11.3 Diagnosis 51 11.4 Treatment 52 11.4.1 Supportive treatment measures 52 11.4.2 Alarm treatment 52 11.4.3 Medication 52 11.5 Guidelines for the treatment of monosymptomatic enuresis 53 11.6 References 53 12. MANAGEMENT OF NEUROGENIC BLADDER IN CHILDREN 53 12.1 Background 53 12.2 Definition 54 12.3 Classification 54 12.4 Urodynamic studies 55 12.4.1 Method of urodynamic study 55 12.4.2 Uroflowmetry 55 12.4.3 Cystometry 55 12.5 Management 56 12.5.1 Investigations 56 12.5.2 Early management with intermittent catheterisation 56 12.5.3 Medical therapy 56 12.5.3.1 Botulinum toxin injections 57 12.5.4 Management of bowel incontinence 57 12.5.5 Urinary tract infection 57 12.5.6 Sexuality 57 12.5.7 Bladder augmentation 57 12.5.8 Bladder outlet procedures 58 12.5.9 Continent stoma 58 12.5.10 Total bladder replacement 58 12.5.11 Lifelong follow-up of neurogenic bladder patients 58 12.6 References 58 13. DILATATION OF THE UPPER URINARY TRACT (URETEROPELVIC JUNCTION AND URETEROVESICAL JUNCTION OBSTRUCTION) 64 13.1 Background 64 13.2 Diagnosis 64 13.2.1 Antenatal ultrasound 64 13.2.2 Postnatal ultrasound 64 13.2.3 Voiding cystourethrogram (VCUG) 64 13.2.4 Diuretic renography 65 13.3 Treatment 65 13.3.1 Prenatal management 65 13.3.2 UPJ obstruction 65 13.3.3 Megaureter 66 13.3.3.1 Conservative management 66 13.3.3.2 Surgical management 66 13.4 Conclusion 66 13.5 Conclusions and recommendations for UPJ-, UVJ-obstruction 66 13.6 References 66 14. VESICOURETERIC REFLUX IN CHILDREN 67 14.1 Methodology 67 14.2 Background 67 14.3 Diagnostic work-up 68 14.3.1 Infants presenting because of prenatally diagnosed hydronephrosis 69 14.3.2 Siblings and offspring of reflux patients 70 4 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 14.3.3 Recommendations for paediatric screening of VUR 70 14.3.4 Children with febrile urinary tract infections 70 14.3.5 Children with lower urinary tract symptoms and vesicoureteric reflux 70 14.4 Treatment 70 14.4.1 Conservative therapy 71 14.4.1.1 Follow-up 71 14.4.1.2 Continuous antibiotic prophylaxis (CAP) 71 14.4.2 Surgical treatment 71 14.4.2.1 Subureteric injection of bulking materials 71 14.4.2.2 Open surgical techniques 72 14.4.2.3 Laparoscopy 72 14.5 Recommendations for the management of vesicoureteric reflux in childhood 72 14.6 References 74 15. URINARY STONE DISEASE 78 15.1 Background 78 15.2 Stone formation mechanisms, diagnosis of causative factors and medical treatment for specific stone types 78 15.2.1 Calcium stones 79 15.2.2 Uric acid stones 80 15.2.3 Cystine stones 80 15.2.4 Infection stones (struvite stones) 80 15.3 Clinical presentation 81 15.4 Diagnosis 81 15.4.1 Imaging 81 15.4.2 Metabolic evaluation 81 15.5 Management 83 15.5.1 Extracorporeal shock wave lithotripsy (SWL) 83 15.5.2 Percutaneous nephrolithotomy 83 15.5.3 Ureterorenoscopy 84 15.5.4 Open stone surgery 84 15.6 Conclusions and recommendations 85 15.7 References 86 16. OBSTRUCTIVE PATHOLOGY OF RENAL DUPLICATION: URETEROCELE AND ECTOPIC URETER 90 16.1 Background 90 16.1.1 Ureterocele 91 16.1.2 Ectopic ureter 91 16.2 Definition and classification 91 16.2.1 Ureterocele 91 16.2.1.1 Ectopic (extravesical) ureterocele 91 16.2.1.2 Orthotopic (intravesical) ureterocele 91 16.2.2 Ectopic ureter 91 16.3 Diagnosis 92 16.3.1 Ureterocele 92 16.3.2 Ectopic ureter 92 16.4 Treatment 92 16.4.1 Ureterocele 92 16.4.1.1 Early treatment 92 16.4.1.2 Re-evaluation 92 16.4.2 Ectopic ureter 93 16.5 Conclusions and recommendations for obstructive pathology of renal duplication: ureterocele and ectopic ureter 94 16.6 References 94 17. DISORDERS OF SEX DEVELOPMENT 97 17.1 Background 97 17.2 Micropenis 97 17.2.1 Background 97 17.2.2 Diagnosis 98 PAEDIATRIC UROLOGY - UPDATE MARCH 2013 5 17.2.3 Treatment 98 17.3 The neonatal emergency 98 17.3.1 Family history and clinical examination 98 17.3.2 Choice of laboratory investigations 99 17.4 Gender assignment 99 17.5 Role of the paediatric urologist 99 17.5.1 Diagnosis 100 17.5.1.1 Clinical examination 100 17.5.1.2 Investigations 100 17.6 Management 100 17.6.1 Feminising surgery 101 17.6.2 Masculinising surgery 101 17.7 Guidelines for the treatment of disorders of sex development 101 17.8 References 102 18. POSTERIOR URETHRAL VALVES 103 18.1 Background 103 18.2 Classification 103 18.2.1 Urethral valve 103 18.3 Diagnosis 104 18.4 Treatment 104 18.4.1 Antenatal treatment 104 18.5 Summary 107 18.6 Conclusions and recommendations posterior urethral valves 107 18.7 References 107 19. PAEDIATRIC UROLOGICAL TRAUMA 110 19.1 Background 110 19.2 Paediatric renal trauma 110 19.2.1 Diagnosis 110 19.2.1.1 Haematuria 110 19.2.1.2 Blood pressure 110 19.2.1.3 Choice of imaging method 110 19.2.2 Treatment 111 19.2.3 Guidelines for the diagnosis and treatment of paediatric renal trauma 111 19.3 Paediatric ureteral trauma 111 19.3.1 Diagnosis 111 19.3.2 Treatment 111 19.3.3 Guidelines for the diagnosis and treatment of paediatric ureteral trauma 112 19.4 Paediatric bladder injuries 112 19.4.1 Diagnosis 112 19.4.2 Treatment 112 19.4.2.1 Intraperitoneal injuries 112 19.4.2.2 Extraperitoneal injuries 113 19.4.3 Guidelines for the diagnosis and treatment of paediatric bladder injuries 113 19.5 Paediatric urethral injuries 113 19.5.1 Diagnosis 113 19.5.2 Treatment 113 19.5.3 Guidelines for the diagnosis and treatment of paediatric trauma 114 19.6 References 114 20.