Urology in Childhood

This volume is concerned with the clinical aspects of urology in childhood; the anatomy, physiology and pathology are discussed only where they have a direct bearing upon the clinical problem, and for a detailed description of these aspects, and of operative technique, the reader is referred to other volumes of this series. Emphasis is laid upon the disorders peculiar to infants and children, so that diseases such as tuberculosis, the manifestations of which in the child differ little from those in the adult, receive less attention. Childhood is deemed to cease with the completion of puberty, though illustrations have sometimes been taken from adolescent cases. The personal views expressed in this work are based upon experience at The Hospital for Sick Children, Great Ormond Street, at St. Peter's and St. Paul's Hospitals, and at the Institute of Urology, University of London. I am deeply indebted to all my colleagues and assistants at these institutions for their help and co-operation both in the trea.tment of cases and in the preparation of this volume. I would particularly wish to thank Mr. T. T. TwrSTINGTON HIGGINS for introducing me to the urology of childhood, Drs. M. BODIAN, R. C. B. PUGH and L. L. R. WHITE for their assistance in matters of pathology, and for preparing specimens for illustration, Dr. "\V. W. PAYNE for his advice on biochemistry, Dr.

A. Introduction.- I. The basic requirements for paediatric urology.- II. The nature of genito-urinary disease in children.- III. Urological investigations.- 1. Collection of urine specimens.- 2. Radiological investigations.- 3. Endoscopy.- 4. Cystometry.- IV. Management of surgical cases.- V. Fluid and electrolyte balance in infants.- B. Congenital abnormalities of the upper urinary tract.- I. Renal agenesis.- II. Renal hypoplasia.- 1. Dysplastic kidney with obliterated ureter.- 2. Dysplastic kidney with patent ureter.- 3. The miniature kidney.- III. Cystic disease of the kidneys.- 1. Congenital polycystic disease. Infantile form.- 2. Congenital polycystic disease. Adult form.- 3. Cystic changes in hypoplastic kidneys and accompanying lower urinary obstruction.- 4. Multicystic kidneys.- 5. Simple serous cysts (solitary cysts).- 6. Multi-locular cysts (cyst-adenoma).- 7. Cystic disease with hydronephrosis and pelvi-ureteric obstruction.- 8. Pyelogenic cysts.- IV. Malrotated, fused and ectopic kidneys.- 1. Simple malrotation.- 2. Horse-shoe kidney.- 3. Crossed ectopia.- 4. Pelvic ectopia.- V. Hydronephrosis due to congenital pelvi-ureteric obstruction.- 1. Incidence.- 2. Pathology.- a) Obstruction due to aberrant renal vessels.- b) Kinks and adhesions.- c) High insertion of the ureter.- d) Stenosis.- e) “Unrolled” kidney.- f) Functional disorders.- 3. Clinical features.- 4. Radiological diagnosis.- 5. Treatment and prognosis.- VI. Congenital ureteric stricture, hypoplasia and valves.- VII. Duplications of the ureter.- 1. General.- a) Incidence.- b) Anatomy.- c) Complications.- ?) Recurrent urinary infection.- ?) Pain.- ?) Hydronephrosis.- ?) Hydro-ureter.- ?) Hypoplasia.- 2. Blind-ending duplications.- 3. Supernumerary kidneys.- 4. Caudal bifurcation.- 5. Triplications of the ureter.- VIII. Ectopic ureters.- 1. General.- 2. Urethral ectopia in the female.- 3. Urethral and genital tract ectopia in the male.- 4. Ectopic ureterocele.- a) Anatomy.- b) Symptoms.- c) Diagnosis.- d) Treatment.- 5. Vaginal and vestibular ectopia.- a) Anatomy.- b) Symptoms.- c) Diagnosis.- d) Treatment.- IX. Ureterocele and ureteric prolapse.- X. Retrocaval ureter.- C. Hydro-ureter and mega-ureter.- I. Normal ureteric function.- II. Radiography and other investigations.- III. Vesico-ureteric reflux.- IV. Hydro-ureter in bladder disorders.- V. Hydro-ureter in other reno-ureteral abnormalities.- VI. Infections.- VII. The mega-ureter-megacystis syndrome.- 1. Pathology.- 2. Clinical, radiological and endoscopic findings.- 3. Diagnosis.- 4. Management.- VIII. Simple mega-ureter.- 1. Pathology.- 2. Clinical, radiological and endoscopic features.- 3. Management.- D. Congenital abnormalities of the lower urinary tract.- I. Urachal anomalies.- 1. Urinary fistula at the umbilicus.- a) Pathology.- b) Clinical picture.- c) Treatment.- 2. Urachal cysts.- 3. Urachal diverticulum.- 4. Blind external fistula.- II. Rare vesical malformations.- 1. Agenesis of the bladder.- 2. Duplications of the bladder.- a) Complete reduplication.- b) Incomplete reduplication.- c) Sagittal septum.- d) Frontal septum.- 3. Hour-glass bladder.- 4. Cysts of the trigone.- 5. Trigonal curtains.- 6. Congenital vesico-vaginal fistulae.- III. Vesical diverticula.- 1. Pathology.- 2. Clinical and radiological features.- 3. Treatment.- IV. Bladder neck obstruction and urethral fibro-elastosis.- 1. Definition.- 2. Pathology.- 3. Symptomatology.- 4. Diagnosis.- 5. Treatment.- V. Urethral obstructions.- 1. Congenital obliterations of the urethra.- a) Obliterations at the level of the bladder neck.- b) Obliteration of the membranous urethra.- c) Obliterations of the penile urethra.- 2. Urethral diaphragms and strictures.- 3. Posterior urethral valves.- a) Pathology.- b) Clinical features.- c) Diagnosis.- d) Treatment.- 4. Anterior urethral valves.- 5. Hypertrophy of the verumontanum and other rare obstructions.- VI. Urethral diverticula and duplications.- 1. Congenital urethral diverticula.- 2. Megalo-urethra.- 3. Duplications of the urethra.- VII. Malformations associated with absent abdominal muscles.- 1. Definition.- 2. Urinary tract pathology.- 3. Clinical picture.- 4. Treatment.- VIII. Malformations associated with anal atresia : persistent cloaca.- 1. In the male.- a) Imperforate anus with a perineal fistula extending into the scrotal or penile raphe.- b) Imperforate anus with recto-urethral fistula.- c) Imperforate anus with recto-vesical fistula.- 2. In the female.- 3. Complex malformations.- 4. Upper urinary tract anomalies.- IX. Ectopia vesicae and epispadias.- 1. Ectopia vesicae.- a) Anatomy.- b) Clinical features.- c) Management.- ?) Reconstructive surgery.- ?) Urinary diversion.- 2. Epispadias.- a) Anatomy.- b) Clinical features.- c) Treatment.- 3. Ectopia cloacae.- X. Hypospadias and allied disorders.- 1. Hypospadias.- a) Incidence.- b) Anatomy.- c) Svmptoms.- d) Treatment.- 2. Short urethra.- 3. External urethral fistula.- E. Lower urinary tract obstruction.- I. Introductory.- II. Causes of obstruction.- 1. Intrinsic lesions.- 2. Extrinsic lesions.- 3. Central nervous lesions.- III. Consequences of obstruction.- IV. Symptomatology.- V. Diagnosis and management.- 1. Acute retention.- 2. Chronic retention.- 3. Low grade obstructions.- F. Enuresis.- I. Normal development.- II. Incidence.- III. Symptoms and differential diagnosis.- IV. Aetiological factors.- 1. Faulty training.- 2. Psychological disorders.- 3. Urinary tract disorders.- 4. Bowel disorders.- 5. Neurological disorders.- 6. Polyuria.- 7. Other causes.- V. Management.- 1. Training and conditioning methods of treatment.- 2. Drug treatment.- 3. Surgical treatment.- G. The neurogenic bladder.- I. Congenital spinal cord lesions.- 1. The spinal defect.- a) Spina bifida cystica.- b) Spina bifida occulta.- c) Other bony deformities.- 2. Neurological and orthopaedic signs.- 3. The bladder dysfunction.- a) The natural history.- b) Diagnosis.- c) Treatment.- ?) Treatment of the spinal cord lesion.- ?) General management of the bladder disorder.- ?) Operations designed to increase the urethral resistance.- ?) Operations designed to lower the urethral resistance.- ?) Operations for the diversion of urine.- ?) Appliances for the control of urethral incontinence.- II. Poliomyelitis.- H. Non-tuberculous urinary infections.- I. Incidence.- II. Bacteriology and mode of infection.- III. Acute urinary infections.- 1. Pathology.- a) Acute pyelonephritis.- b) Infected hydronephrosis and pyonephrosis.- 2. Clinical features.- a) In the neonate.- b) In the infant.- c) In the older child.- d) Complicated cases.- 3. Management.- IV. Chronic and recurrent urinary infection.- V. Chronic pyelonephritis.- 1. Pathology.- 2. Clinical features.- 3. Kadiology.- 4. Management.- VI. Pyaemic kidney; renal carbuncle and perinephric abscess.- 1. Pathology.- 2. Clinical features.- 3. Management.- VII. Cystitis.- VIII. Parasitic and fungal infections.- 1. Schistosomiasis (Bilharziasis).- 2. Hydatid cyst.- 3. Myiasis.- 4. Moniliasis.- 5. Actinomycosis.- I. Urogenital tuberculosis.- I. Incidence.- II. Pathology.- III. Clinical features and management.- 1. Cortical lesions.- 2. Early ulcerative lesions of the renal papilla.- 3. Advanced lesions.- 4. Lesions of the male genital tract.- IV. Chemotherapy.- J. Calculous disease.- I. Incidence.- II. Aetiological classification.- 1. Endemic calculi.- 2. Calculi complicating congenital abnormalities of the urinary tract.- 3. Recumbency calculi.- 4. Foreign body calculi.- 5. Cystine calculi.- 6. Xanthine calculi.- 7. Calculi associated with nephrocalcinosis.- a) Hyperchloraemic acidosis, infantile renal acidosis.- b) Oxalate nephrocalcinosis.- c) Hyperparathyroidism.- d) Pyelonephritis, renal hypoplasia and glomerulo-nephritis.- e) Miscellaneous.- 8. Stones in congenital cystic dilatation of the collecting tubules.- 9. Idiopathic calculi.- III. Regional classification.- 1. Renal calculi.- 2. Ureteric calculi.- 3. Vesical calculi.- 4. Urethral calculi.- 5. Preputial calculi.- 6. Calculi outside the urinary tract.- IV. Management of calculus cases.- 1. Diagnosis.- 2. Treatment.- 3. Prevention of recurrence.- K. Neoplastic disease.- I. Neoplasms in infancy and childhood.- II. The kidney.- 1. Nephroblastoma.- a) Incidence.- b) Pathology.- c) Clinical features.- ?) The abdominal tumour.- ?) Abdominal pain.- ?) Haematuria.- ?) Spontaneous rupture.- ?) Hypertension.- ?) Varicocele.- ?) Dystocia.- ?) Metastases.- d) Radiological diagnosis.- e) Treatment.- f) Recurrence.- g) Prognosis.- 2. Adenocarcinoma.- 3. Papillary tumours of the renal pelvis.- 4. Hamartoma and other benign tumours.- 5. Lymphosarcoma and leukaemia.- III. The adrenal gland and retro-peritoneal space.- 1. Neuroblastoma.- 2. Ganglioneuroma.- 3. Pheochromocytoma.- 4. Adrenal cortical tumours.- 5. Adrenal haematoma.- 6. Retro-peritoneal teratoma.- IV. The bladder.- 1. Rhabdomyosarcoma.- 2. Other bladder tumours.- V. The prostate and urethra.- VI. The penis and scrotum.- VII. The epididymis and spermatic cord.- VIII. The testicle.- IX. The ovary.- X. The vagina and uterus.- L. Hypertension.- I. General observations.- II. Renal hypertension.- 1. Pathology.- a) Glomerulonephritis.- b) Chronic pyelonephritis and renal hypoplasia.- c) Hydronephrosis.- d) Vascular lesions.- e) Injury and perinephric haematoma.- f) Tumour.- g) Miscellaneous causes.- 2. Clinical features.- 3. Investigations.- 4. Management.- M. Renal failure and allied disorders.- I. Acute renal failure.- 1. Pathology.- a) Acute tubular necrosis.- b) Acute glomerulonephritis.- c) Bilateral renal venous thrombosis.- d) Suppurative nephritis.- e) Ureteric obstruction.- 2. Clinical picture.- 3. Management.- II. Chronic renal failure.- 1. Aetiology.- 2. Clinical course and biochemical changes.- 3. Bone changes — renal osteodystrophy.- a) Rachitic changes.- b) Osteitis fibrosa changes.- 4. Management.- III. Renal vascular disorders.- 1. Bilateral cortical necrosis.- 2. Venous thrombosis with haemorrhagic infarction.- a) Pathology.- b) Clinical features.- c) Treatment.- 3. Venous obstruction with thrombosis of inferior vena cava.- 4. Arterial occlusion.- IV. Polyuria and the renal tubular disorders.- 1. Diabetes insipidus.- 2. Diabetes insipidus renalis.- 3. Primary polydipsia.- 4. Hyperchloraemic renal acidosis.- a) Infantile form (Lightwood’s syndrome).- b) Adult form (Albright’s syndrome).- 5. Lignac Fanconi disease (De Toni’s syndrome).- 6. Idiopathic hypercalcaemia.- V. Haematuria and nephritis.- 1. General considerations.- 2. Surgical causes of haematuria.- 3. Haemorrhagic diseases.- 4. Nephritis.- 5. Medicational haematuria.- 6. Unexplained haematuria.- N. The male genital tract.- I. Congenital abnormalities of the penis.- 1. Micropenis.- 2. Congenital absence of the penis.- 3. Diphallus.- 4. Pre-penile scrotum.- 5. Torsion of the penis.- 6. Webbing of the penis.- 7. Cysts of the penile raphe.- II. Phimosis and paraphimosis.- 1. The normal development.- 2. Phimosis.- 3. Paraphimosis.- 4. Routine circumcision.- III. Skin lesions.- IV. Meatal ulcer and stenosis.- V. Genital tract infections.- 1. Urethritis.- 2. Prostatitis and prostatic abscess.- 3. Epididymitis.- 4. Orchitis.- VI. Urethral injuries, stricture and fistula.- 1. Anterior urethral injuries.- 2. Posterior urethral injuries.- 3. Urethral stricture.- 4. Urethral fistula.- VII. Priapism.- VIII. Undescended testicles.- 1. Embryology.- 2. Incidence.- 3. Surgical anatomy.- 4. Histology.- 5. Function.- 6. Cause.- 7. Complications.- 8. Clinical picture.- 9. Management.- IX. Other testicular anomalies.- 1. Anorchia and monorchia.- 2. Hypoplasia.- 3. Polyorchism.- 4. Rare forms of testicular ectopia.- X. Hydrocele.- 1. Hydrocele of the tunica vaginalis.- a) Irreducible.- b) Reducible.- 2. Hydrocele of the cord, or of the canal of Nuck.- 3. Hydrocele with undescended testis.- 4. Meconium hydrocele.- XI. Varicocele.- XII. Torsion of the spermatic cord and of the testicular appendages.- 1. Torsion of the spermatic cord.- 2. Torsion of the testicular appendages.- XIII. Scrotal oedema and gangrene.- O. The female genital tract and urethra.- I. The clitoris and labia.- 1. Ligated clitoris.- 2. Labial fusion.- 3. Ammonia dermatitis.- 4. Labial thickening.- II. The external urinary meatus.- 1. Stenosis.- 2. Hypospadias.- 3. Covered meatus.- III. Urethral prolapse.- IV. Urethro-trigonitis.- V. Vulvo-vaginitis.- 1. Gonococcal vulvo-vaginitis.- 2. Non-gonococcal vulvo-vaginitis.- VI. Urethral injuries and fistulae.- VII. Hydrocolpos and haematocolpos.- VIII. Congenital absence of the vagina.- P. Intersex states and endocrine disorders.- I. Normal development.- 1. The genetic basis.- 2. Endocrine factors.- 3. The anatomical growth changes.- II. Female pseudohermaphroditism.- 1. Congenital adreno-cortical hyperplasia.- a) Anatomy.- b) Clinical progress.- c) Laboratory findings.- d) Diagnosis.- e) Management.- 2. Other forms of female pseudohermaphroditism.- III. Male pseudohermaphroditism.- 1. Male pseudohermaphrodites with external genitalia of equivocal sexuality.- 2. Male pseudohermaphrodites with purely feminine external genitalia.- IV. True hermaphroditism.- 1. Anatomy.- 2. Clinical features.- 3. Diagnosis.- 4. Management.- V. The practical approach to intersex.- VI. Gonadal agenesis.- VII. Hypogonadism in the male.- 1. Pituitary deficiency.- 2. Testicular deficiency.- 3. Klinefelter’s syndrome.- 4. Familial hypogonadism.- VIII. Sexual precocity.- 1. Constitutional precocious puberty.- 2. Precocity due to hypothalamic lesions.- 3. Albright’s syndrome.- 4. Iso-sexual precocity in the male.- 5. Iso-sexual precocity in the female.- 6. Heterosexual precocity in the male.- 7. Heterosexual precocity in the female.- References.- Author Index.