Urology in Childhood

Volume XV "Urology in Childhood" was written in 1956/57 and was the first in the series of the Encyclopedia of Urology to appear. This present volume has been constructed as a supplement and the original intention was to deal only with those subjects in which there have been significant advances during the intervening sixteen years. As the work has proceeded, however, it has become evident that there is no aspect of paediatric urology which has not been developed, and no topic which has not been illuminated by many contributions to the literature. Indeed, there has been such a copious flow of publications devoted to children's urinary tract disease that a full review is no longer possible within the compass of a volume of this size: the decision as to what should be left out has therefore been a matter of the greatest difficulty. The choice has been inevit­ ably arbitrary and many omissions are regretted. The attempt has been made, however, to report the most notable developments of the subject, and perhaps the greatest change in the practice of paediatric urology has been the full integra­ tion with paediatric nephrology. The team approach to infant disease in particular has led to greatly improved results. I have been fortunate to have Dr. T. M.

A. The Nephrological Background to Urology.- I. Development of Renal Function.- 1. Morphological and Biochemical Aspects.- 2. Renal Function before Birth.- 3. The Neonatal Kidney.- 4. Maturation of Renal Function.- II. Metabolic Aspects of Young Children.- 1. Body Size.- 2. Growth.- 3. Diet.- III. Body Fluids.- 1. Water and Electrolyte Physiology.- a) Sodium and Water.- b) Potassium.- c) Hydrogen Ion.- 2. Principles of Parenteral Fluid Therapy.- a) Hypovolaemia.- b) Deficit Replacement.- c) Maintenance Requirements.- d) Abnormal Losses.- IV. Assessment of Renal Function.- 1. General Considerations.- 2. Glomerular Function.- a) Urea.- b) Creatinine.- c) Glomerular Filtration Rate.- d) Individual Kidney Function.- 3. Tubular Function.- a) Measurement of Urine Concentration.- b) Acidification of Urine.- c) Proximal Tubular Function.- B. Acute Renal Failure.- I. Introduction.- II. Pathogenesis of Acute Renal Failure.- 1. Pre-Renal Factors.- 2. Renal Factors.- a) Acute Tubular Necrosis.- b) Renovascular Accidents.- c) Disseminated Intravascular Coagulation.- d) Acute Glomerulonephritis.- e) Pyelonephritis.- 3. Post-Renal Factors.- III. Diagnosis.- IV. Consequences of Acute Renal Failure.- 1. Saline Overload.- 2. Osmolar Disturbances.- 3. Hyperkalaemia.- 4. Acidosis.- 5. Divalent Ions.- 6. Uraemia.- 7. Other Features.- a) Convulsions.- b) Sepsis.- c) Anaemia.- V. Conservative Management of Acute Renal Failure.- VI. Dialysis.- 1. Indications.- 2. Haemodialysis vs Peritoneal Dialysis.- 3. Peritoneal Dialysis.- a) Setting up Dialysis.- b) Dialysis Regime.- c) Complications.- VII. Conclusions.- C. Chronic Renal Failure in Children.- I. Introduction.- II. Epidemiology of Renal Failure in Children.- 1. Mortality.- 2. Aetiology.- III. Pathophysiology of Chronic Renal Failure.- 1. Adaptation to Diminishing GFR.- 2. Uraemia.- 3. Osteodystrophy.- a) Vitamin D.- b) Parathormone.- c) Evolution of Osteodystrophy.- 4. Haematological Consequences.- 5. Growth.- IV. Practical Aspects of Conservative Management.- 1. Elimination of Reversible Causes of Renal Failure.- 2. Dietary Control.- 3. Medical Complications.- 4. Planning for the Future.- V. Regular Haemodialysis.- 1. Access to the Circulation.- 2. Equipment and Dialysis Routine.- 3. Medical Problems.- 4. Psychological Problems.- 5. Results.- VI. Transplantation.- 1. Recipient Selection.- 2. Donor Selection.- 3. Technical Aspects.- 4. Immunosuppression and Medical Problems.- 5. Results.- VII. Conclusions.- D. Hypertension in Childhood.- I. Introduction.- II. The Normal Blood Pressure.- III. Saline Dependent Hypertension.- IV. Renin-Angiotensin Hypertension.- 1. Renin-Angiotensin-Aldosterone System.- 2. Renovascular Hypertension.- a) Pathology.- b) Functional Aspects.- c) Investigation.- d) Treatment.- 3. Predominately Unilateral Renal Disease.- 4. Renal Tumours.- V. Corticosteroid Hypertension.- 1. Aldosterone.- 2. Cortisol.- VI. Catecholamine Hypertension.- 1. Physiology.- 2. Pathology.- 3. Functional Aspects and Diagnosis.- 4. Location and Treatment.- VII. Essential Hypertension.- VIII. Consequences of Hypertension.- 1. Cardiac.- 2. Neurological.- 3. Renal.- IX. Hypotensive Therapy.- 1. Indications for Treatment.- 2. Acute Hypertensive Emergencies.- 3. Saline-Dependent Hypertension.- 4. Long Term Therapy.- X. Summary.- E. Glomerular Disease and Haematuria.- I. Introduction.- II. Mechanisms of Glomerular Injury.- III. Methods of Investigation.- 1. Haematuria.- 2. Proteinuria.- 3. Complement.- 4 Renal Biopsy.- a) Background.- b) Equipment.- c) Indications and Precautions.- d) Technique.- e) Post-Operative Care and Complications.- f) Specimen Handling.- g) Normal Appearances and Terminology.- IV. Syndromes of Glomerulonephritis.- 1. The Acute Nephritic Syndrome.- 2. The Nephrotic Syndrome.- 3. Persistent Proteinuria.- 4. Recurrent Haematuria.- 5. Acute and Chronic Renal Failure.- V. Conclusion.- F. Renal Anomalies.- I. Introduction.- II. Absent Kidney.- III. The Small Kidney.- 1. Unilateral Hypoplasia.- 2. Bilateral Oligomeganephronic Hypoplasia.- 3. Segmental Hypoplasia.- 4. Dysplasia.- a) Dysplasia Associated with an Atretic Impermeable Ureter.- b) Dysplasia with Patent Ureter.- c) Dysplasia with Urethral Obstruction.- 5. Renal Vein Thrombosis.- 6. Renal Artery Stenosis.- 7. Medullary Necrosis.- 8. Pyelonephritis.- 9. Obstructive Atrophy.- 10. Post-Irradiation Atrophy.- IV. Multicystic Kidney.- V. Infantile Polycystic Kidney.- VI. Adult Polycystic Kidney.- VII. Medullary Cystic Disease: Nephronophthisis.- VIII. Medullary Sponge Kidney.- IX. Multilocular Cysts (Cystadenoma).- X. Serous Cysts.- XI. Pyelogenic Cysts (Caliceal Diverticula).- XII. Microcystic Disease.- XIII. Cystic Disease and the Multiple Anomaly Syndromes.- XIV. Fused, Ectopic and Dysmorphic Kidneys.- G. Urinary Tract Infection.- I. Introduction.- II. Diagnosis of Infection.- 1. Clinical Features.- 2. Significant Bacteriuria.- 3. Pyuria.- 4. Specimen Collection.- 5. Screening Techniques.- III. Bacteriology.- 1. Infecting Organism.- 2. Recurrences.- 3. Bacterial Variants.- 4. Localisation of Infection.- IV. Epidemiology.- 1. Neonates.- 2. School Children.- V. Immunology of Urinary Tract Infection.- 1. Serum Antibody Response.- 2. Local Antibody Production.- 3. Immunological Factors in Chronicity.- VI. Experimental Observations.- 1. Urineas a Culture Medium.- 2. Haematogenous Pyelonephritis.- 3. Ascending Infection.- VII. Specific Urological Associations.- 1. Vesicoureteric Reflux.- 2. Minor Urological Abnormalities.- 3. Obstructive Uropathy.- 4. Calculus Disease.- 5. Catheterisation.- VIII. Functional Sequelae of Urinary Tract Infection.- 1. Acute Infection.- 2. Chronic Pyelonephritis.- IX. Chronic Pyelonephritis.- 1. Pathological Diagnosis.- 2. Radiological Diagnosis.- 3. Bacteriology.- 4. Natural History of Pyelonephritis.- X. Treatment.- 1. Basic Considerations.- 2. The Acute Infection.- 3. Recurrences.- 4. The Emergence of Resistant Strains.- 5. Maintenance Chemotherapy.- 6. Antibiotic Therapy in Uraemia.- 7. Antibiotic Therapy in Neonates.- 8. Some New Antimicrobial Agents.- 9. Candida.- XI. Conclusion.- H. Localised Inflammatory Lesions.- I. The Kidney.- 1. Renal Carbuncle.- 2. Xanthogranulomatous Pyelonephritis.- 3. Perinephric Abscess.- II. The Bladder.- 1. Acute Haemorrhagic Cystitis.- 2. Eosinophilic Cystitis.- 3. Proliferative and Follicular Cystitis.- 4. Pseudo-Membranous Cystitis.- 5. Malacoplakia.- 6. Bilharziasis.- 7. Granulomatous Cystitis.- 8. Cystitis Due to Cyclophosphamide.- 9. Interstitial Cystitis.- III. The Prostate.- IV. The Urethra.- I. The Female Urethra in Recurrent Infections.- I. Introduction.- II. Clinical Examination.- III. Measurement of Residual Urine.- IV. Endoscopy.- V. Urethral Calibration.- VI. Radiology.- VII. Cystometry and Pressure Flow Measurements.- VIII. Results of Treatment.- IX. Conclusion.- J. Vesico-Ureteric Reflux.- I. Introduction.- II. The Normal Mechanism.- III. The Pathology of Reflux.- 1. Reflux Due to Inflammation.- 2. Short Intra-Vesical Ureter.- 3. Para-Ureteric Saccule.- IV. Reflux and the Ureter.- V. Reflux and Hydronephrosis.- VI. Reflux and Obstructive Nephropathy.- VII. Reflux and Urinary Infection.- VIII. Reflux and Pyelonephritis.- IX. Spontaneous Cessation of Reflux.- X. The Clinical Management of Uncomplicated Reflux.- XI. Operative Technique.- XII. Reflux in Duplex and Ectopic Ureters.- 1. The Bifid Ureter.- 2. Complete Duplications.- XIII. Reflux and Bladder Outflow Obstruction.- XIV. Reflux and Megacystis.- XV. Reflux in Neuropathic Bladder.- XVI. Reflux in Vesical Exstrophy.- XVII. Reflux with Renal Dysplasia.- XVIII. Reflux and Ureteric Obstruction.- K. Ectopic Ureter.- I. Ectopic Ureter with Duplication in the Female.- II. Unilateral Single Ectopic Ureter in the Female.- III. Ectopic Ureter in the Male.- IV. Bilateral Single Ectopic Ureters.- V. Ectopic Ureter with Triplication.- L. Ureteroceles.- I. Orthotopic Ureterocele.- II. Ectopic Ureterocele.- 1. Sex and Side.- 2. The Upper Urinary Tract.- 3. Size and Position.- 4. Tension and Compressibility.- 5. Detrusor Backing.- 6. Prolapsing Ureterocele.- 7. Bladder Outflow Obstruction.- 8. Ruptured Ureterocele.- 9. Treatment.- M. Obstructive Uropathy: The Functional Disorder.- I. Introduction.- II. Urinary Tract Dilatation.- III. Pressure Studies.- IV. Renal Morphology.- V. Renal Function.- 1. Experimental Observations.- 2. Clinical Observations.- a) Technical Considerations.- b) Abnormalities of Function.- c) Prediction of Recovery.- d) Post-Operative Natruresis.- VI. Radiological Studies: The Nephrogram.- VII. Isotope Studies.- VIII. Conclusion.- N. Obstructive Uropathy: The Upper Tract.- I. Hydrocalicosis.- 1. Hydrocalicosis Due to Vascular Obstruction.- 2. Hydrocalicosis Due to Infundibular Stenosis.- 3. Megacalicosis.- 4. Caliceal Diverticulum.- II. Hydronephrosis Due to Pelvi-Ureteric Obstruction.- 1. Intermittent Hydronephrosis.- 2. Hydronephrosis in Neonates.- 3. Hydronephrosis in Mai-Rotated Kidneys.- 4. Hydronephrosis in the Duplex Kidney.- 5. Hydronephrosis with Low Pelvi-Ureteric Obstruction.- 6. Hydronephrosis Due to Ureteric Polyps.- III. Mid-Ureteric Obstructions.- 1. Functional Obstructions.- 2. Peristaltic Disorders in the Bifid Ureter.- 3. Congenital Stricture: Atretic Segment.- 4. Retrocaval Ureter.- 5. Retro-Iliac Ureter.- 6. Ureteric Polyps and Tumours.- 7. Extrinsic Obstructions.- IV. Lower Ureteric Obstructions.- O. Obstructive Uropathy: The Bladder.- I. Introduction.- 1. Urethral Obstruction.- 2. Occult Neuropathic Bladder.- 3. Incomplete Prune Belly Syndrome.- 4. Mega-Ureter-Megacystis Syndrome.- 5. Atonic Bladder.- 6. Polyuric Bladder.- II. Bladder Neck Obstruction.- III. Bladder Neck Resection.- IV. Vesical Diverticula.- P. Obstructive Uropathy: The Urethra.- I. Introduction.- II. Pathology of Urethral Valves.- III. Presentation.- IV. Diagnosis.- V. The Infant Emergency.- VI. Operation for Urethral Valve Ablation.- VII. Post-Operative Management.- VIII. Persistent Upper Tract Disturbance.- 1. The Functionless Kidney.- 2. Reflux.- 3. Uretero-Vesical Obstruction.- IX. Cutaneous Ureterostomy and Vesicostomy.- X. Re-Modelling and Re-Implantation of the Ureters.- XI. The Results of Treatment.- XII. Other Urethral Anomalies.- 1. Posterior Urethral Diverticula.- a) Cystic Dilatation of the Prostatic Utricle.- b) The Vagina in Male Intersex.- c) Ectopic Ureter.- d) Rectal Stump.- 2. Anterior Urethral Diverticula.- a) Wide Mouthed Diverticula.- b) Narrow Necked Diverticula.- c) Traumatic Diverticula.- d) Megalo-Urethra.- 3. Urethral Duplications.- 4. Urethral Atresia in the Female.- Q. The Prune Belly Syndrome.- I. Introduction.- II. Urinary Tract Pathology.- III. The Abdominal Muscles and Other Anomalies.- IV. Clinical Presentation.- 1. Complete Urethral Obstruction: Neonatal Presentation.- 2. Infant Emergencies.- 3. Late Presentations.- V. Incomplete Syndromes.- R. Urinary Incontinence.- I. Introduction.- II. Enuresis.- III. Small Capacity Bladder.- IV. The Lazy Bladder Syndrome.- V. Giggle Incontinence.- VI. Wide Bladder Neck Anomaly.- VII. Behaviour Disorders and Incontinence.- VIII. Infection and Incontinence.- IX. Obstruction and Incontinence.- X. Neuropathy and Incontinence.- XI. Post-Traumatic and Iatrogenic Incontinence.- XII. Epispadias and Incontinence.- XIII. Ectopic Ureter and Incontinence.- XIV. Implantation of Electronic Stimulators.- S. Neuropathic Bladder.- I. Introduction.- II. Classification.- III. Causes of Neuropathic Bladder.- 1. Spina Bifida Occulta.- 2. Spinal Dysraphism.- 3. Sacral Agenesis.- 4. Myelomeningocele.- 5. Spinal Cord Tumours.- 6. Osteomyelitis of the Vertebral Bodies.- 7. Traumatic Paraplegia.- 8. Infections.- 9. Transverse Myelitis.- 10. Operative Trauma.- 11. Subclinical Neuropathic Bladder.- IV. Complications of Neuropathic Bladder.- V. Investigations of Neuropathic Bladder.- 1. Urinalysis.- 2. Excretory Urography.- 3. Cystography.- 4. Pressure Studies.- 5. Electromyography.- 6. Isotope Renography.- 7. Cystoscopy.- VI. Treatment of the Neuropathic Bladder.- 1. Bladder Expression.- 2. Drug Treatment.- 3. Catheter Drainage.- 4. Urethral Plication.- 5. Pudendal Neurectomy.- 6. Bladder Neck Resection.- 7. Resection of the External Sphincter.- 8. Continence Restoring Procedures.- 9. Intestinal Cystoplasties.- 10. Electrical Stimulation of the Bladder and its Sphincters.- 11. Reflux Prevention.- 12. Appliances.- 13. Urinary Diversion.- a) Vesicostomy.- b) Cutaneous Ureterostomy.- c) Ileal Conduit.- d) Colonic Conduit.- VII. Defunctioned Neuropathic Bladder.- VIII. Neuropathic Anal Canal.- IX. Sexual Function Associated with the Neuropathic Bladder.- X. A Practical Guide to the Management of the Congenital Neuropathic Bladder.- T. Epispadias and Exstrophy.- I. Introduction.- II. Cloacal Exstrophy.- III. Vesical Exstrophy.- 1. The Musculo-Skeletal Anomaly.- 2. The Ano-Rectal Defect.- 3. The Male Genital Defect.- 4. The Female Genital Defect.- 5. The Urinary Tract Defect.- 6. Functional Reconstruction.- 7. Urinary Diversion.- IV. Female Epispadias.- V. Male Epispadias.- VI. Split Symphysis Variants.- U. Urolithiasis.- I. Introduction.- II. Endemic Calculi.- III. Infective Calculi.- 1. Incidence.- 2. Infecting Organisms.- 3. Composition of Calculi.- 4. Site of Calculi.- 5. Effects on the Urinary Tract.- 6. Presentation and Progress.- 7. Treatment.- IV. Metabolic Calculi.- 1. Disorders of Calcium Metabolism.- a) Hypercalcaemia.- b) Hypercalciuria.- c) Defects of Urinary Acidification.- 2. Disorders of Oxalate Metabolism.- 3. Disorders of Purine Metabolism.- a) Uric Acid Calculi.- b) Xanthinuria.- 4. Disorders of Amino-Acid Metabolism.- a) Cystinuria.- b) Glycinuria.- 5. Screening Investigations for Metabolic Stones.- V. Neoplastic Disease: The Kidney.- I. Introduction.- II. Incidence.- III. Pathology of Nephroblastoma.- 1. Histology and Origin of Neoplasia.- 2. Grading of Tumour.- 3. Staging of Tumour.- 4. Growth Rate.- 5. Bilateral Disease.- 6. Nephroblastomatosis.- IV. Pathology of Other Renal Tumours.- 1. Neonatal Tumours.- 2. Rhabdomyosarcoma.- 3. Leiomyosarcoma.- 4. Angiomyolipoma.- 5. Haemangioma.- 6. Haemangiopericytoma.- 7. Adenocarcinoma.- 8. Lymphosarcoma and Leukaemia.- 9. Cystadenoma.- V. Clinical Presentation.- VI. Diagnosis.- VII. Treatment.- 1. Surgery.- 2. Radiotherapy.- 3. Chemotherapy.- 4. Treatment Regimes.- VIII. Results.- W. Neoplastic Disease: The Lower Urinary Tract.- I. Rhabdomyosarcoma.- 1. Polypoid Rhabdomyosarcoma of the Bladder.- 2. Solid Rhabdomyosarcoma of Bladder Base and Prostate.- II. Transitional Cell Tumours.- III. Leukaemia.- IV. Hamartomata and Benign Tumours.- 1. Neurofibromatosis.- 2. Myoma.- 3. Simple and Fibromatosis Polyps.- 4. Haemangioma.- 5. Pheochromocytoma.- X. Congenital Adrenal Hyperplasia.- I. Introduction.- II. Pathogenesis.- 1. 21-Hydroxylase Defect.- 2. 11-Hydroxylase Defect.- 3. Lipoid Hyperplasia.- 4. 3?-Hydroxy dehydrogenase Defect.- 5. 17?-Hydroxylase Defect.- III. Cause of Virilisation.- IV. Aetiology of Salt-Losing Defect.- V. Clinical Features.- 1. Females.- 2. Males.- VI. Salt-Losing Syndrome.- VII. Genetics.- VIII. Diagnosis.- IX. Diagnostic Tests.- X. Treatment.- XI. Treatment of the Salt-Losing Syndrome.- 1. Emergency Treatment.- 2. Maintenance Treatment.- Y. Sex Chromosome Anomalies and Intersex States.- I. The Normal Chromosomes.- II. Chromosome Abnormalities.- III. Sex Chromatin.- IV. The Identification of Chromosomes.- 1. Autoradiography.- 2. Fluorescence Studies.- 3. Other Chromosome Banding Techniques.- V. Errors of Sexual Development.- VI. Errors of Sex Determination.- 1. Complete Sex Inversions.- 2. Incomplete Sex Inversions: True Hermaphrodites.- VII. Errors of Sex Determining Mechanism.- 1. Turner’s Syndrome (XO sex chromosomes; 45,X chromosome complement).- 2. Klinefelter’s Syndrome.- 3. 47, XYY Males.- 4. 47, XXX Females.- VIII. Errors of Sex Differentiation.- 1. Female Pseudohermaphrodites.- 2. Male Pseudohermaphrodites.- IX. Sex Assignment.- Appendix: Z. U...