Malformations

Vesicoureteral Reflux.- A. Definition of Reflux.- B. Anatomy of the Ureterovesical Junction.- I. Contribution of the Ureter and Trigone to the Formation of the Ureterovesical Junction.- II. The Vesical Component of the Ureterovesical Valve.- 1. The Mucosal Layer.- 2. The Inner Longitudinal Muscle Layer.- 3. The Middle Circular Muscle Layer.- 4. The Outer Longitudinal Muscle Layer.- C. Innervation.- D. Physiology of the Ureterovesical Valve.- I. The Effect of Interrupting the Anatomical Continuity between the Ureter and the Trigone.- II. The Effect of Cutting the Nerves that Supply the Trigonal Muscle.- III. The Effect of Active Contraction of the Trigone on the Resistance of the Intravesical Ureter.- IV. The Effect of Active Contraction of the Trigone in Tightening and Occluding the Intravesical Ureter during Voiding.- E. Pathology of the Ureterovesical Junction.- I. Structural Changes at the Ureterovesical Junction.- 1. Conditions in which the Ureteral Hiatus Moves Downward in Relationship to the Ureteral Orifice.- 2. Conditions in which the Ureteral Orifice Moves Upward in Relation to the Ureteral Hiatus.- II. The Effect of Vesical Edema on the Ureterovesical Junction.- III. Congenital Anomalies of the Ureter.- IV. Maturation of the Intravesical Ureter.- V. Concept of the Marginally Competent Ureterovesical Junction.- F. Classification of Reflux by Etiology.- I. Reflux Due to Trigonal Weakness.- II. Reflux Due to Obstruction at or below the Bladder Neck.- III. Reflux Due to Neurogenic Vesical Disease.- IV. Reflux Due to Edema of the Bladder Wall.- V. Reflux Due to Congenital Anomalies.- VI. Reflux Due to Iatrogenic Causes.- G. Etiology of Nonocclusive Ureteral Dilatation (Megaloureter).- I. Factors that Increase the Volume of Urine in the Ureter.- 1. Dilation of the Renal Pelvis and Ureter by Forced Intake of Fluids.- 2. Dilation of the Upper Urinary Tract Due to Diabetes Insipidus.- 3. Dilation of the Pelvis of a Kidney Forced to Increase its Output after Unilateral Nephrectomy.- 4. Reflux as a Cause of Ureteral Overfilling.- II. Emptying Capacity of Various Areas of the Ureter.- III. Reflux and the Ureteropelvic Junction.- IV. Reflux and the Ureterovesical Junction.- H. Incidence of Reflux.- I. High Incidence of Reflux in Children with Urinary Tract Infection.- II. Demonstration of Reflux in Adults with Nonobstructive Pyelonephritis.- I. Effects of Reflux.- I. The Role of Reflux in Obstruction at the Ureteropelvic Junction.- II. The Kidney with Chronic Pyelonephritis.- III. The Atrophic Kidney with Chronic Pyelonephritis.- IV. The Normal Kidney.- V. Reflux as the Cause of Pyelonephritis of Pregnancy.- VI. Role of Reflux in the Perpetuation of Urinary Tract Infection.- VII. Miscellaneous.- J. The Role of the Ureterovesical Junction in the Pathogenesis of Pyelonephritis.- I. Childhood.- II. The Teenage Period.- III. The Adult Period.- IV. Prostatic Period.- K. The Treatment of Reflux and its Complications.- I. Medical Treatment.- II. The Surgical Correction of Reflux.- 1. Types of Operation.- 2. Results of Operation.- 3. Indications for Operation.- a) The Severity of the Reflux.- b) The Presence of Saccules.- c) The Severity of the Clinical Course.- d) The Conditions of the Kidneys.- e) The Age of the Patient.- References.- Anomalies of the Kidney.- I. Agenesis.- a) Bilateral Renal Agenesis.- b) Solitary Pelvic Kidney.- II. Hypoplasia.- a) Unilateral.- b) Bilateral.- III. Cystic Disease.- a) Simple (Solitary) Cysts.- b) Multiple Cysts of the Kidney.- c) Multilocular Cysts.- d) Polycystic Kidney Disease.- e) Peripelvic Cysts.- IV. Fusion Anomalies of the Kidney.- a) Horseshoe Kidney.- b) Unilateral Fused Kidney.- c) Fused Pelvic Kidney.- V. Ectopia of the Kidney.- 1. Introduction.- 2. Classification.- a) Simple Ectopy.- b) Bilateral Ectopia.- c) Crossed Renal Ectopia.- d) Ectopic Pelvic Kidney.- e) Thoracic Kidney (Congenital Superior Ectopia).- VI. Anomalies of Rotation.- 1. Introduction.- 2. Embryology.- 3. Incidence.- 4. Etiology.- 5. Symptoms and Diagnosis.- 6. Treatment.- VII. Anomalies of the Vessels.- 1. Introduction.- 2. Production of Hydronephrosis by Aberrant Vessels.- 3. Incidence.- 4. Symptoms.- 5. Diagnosis.- 6. Treatment.- VIII. Supernumerary Kidney.- 1. Introduction and Incidence.- 2. Pathogenesis.- 3. Symptoms.- 4. Diagnosis.- 5. Treatment.- IX. Cystic Disease of the Renal Pyramids.- 1. Definition and Historical.- 2. Incidence.- 3. Etiology.- 4. Pathology.- 5. Clinical Considerations.- 6. Diagnosis.- 7. Treatment.- 8. Prognosis.- X. Anomalies of the Renal Pelvis.- 1. Incidence.- 2. Embryological Development.- 3. Clinical Considerations.- 4. Differential Diagnosis.- 5. Treatment.- Acknowledgments.- References.- Anomalies of the Ureter.- A. Multiplication, Ectopia and Ureterocele.- I. Ureteral Duplication (and Triplication).- 1. Definitions.- 2. Incidence (Table 1).- 3. Embryology and Physiology.- a) Embryology.- b) Physiology.- 4. Surgical Anatomy.- 5. Associated Anomalies.- 6. Associated Reflux.- 7. Associated Disease.- 8. Clinical Aspects.- a) Symptoms.- b) Diagnosis.- ?) Personal History.- ?) Family History.- ?) Physical Examination.- ?) Laboratory Study of the Urine.- ?) Excretory Urography.- ?) Cystoscopy.- ?) Vaginoscopy and Urethroscopy.- ?) Other Radiographic Techniques.- c) Principles of Management.- ?) Incomplete Ureteral Duplication.- ?) Complete Ureteral Duplication.- ?) Triplication or Quadruplication.- II. Ureteral Ectopia.- 1. Definition and Description.- 2. Sites of Ectopic Orifices.- 3. Incidence.- 4. Associated Anomalies.- 5. Clinical Features.- a) Incontinence.- b) Obstruction and Dilatation.- c) Infection.- 6. Diagnosis.- a) History.- b) Family History.- c) Physical Examination.- d) Urinalysis.- e) Urographie Procedures.- ?) Excretory Urography.- ?) Micturition Urethrocystography.- ?) Vasography.- f) Endoscopy.- 7. Treatment.- III. Ureterocele.- 1. Definition, Description, Types.- 2. Incidence.- 3. Pathogenesis.- 4. Associated Anomalies and Disease.- 5. Clinical Manifestations.- 6. Reflux.- 7. Diagnosis.- a) Urography.- ?) Excretory Urography.- ?) Retrograde Urethrocystography.- b) Endoscopy.- 8. Treatment.- a) Simple Ureterocele.- b) Ectopic Ureterocele.- B. Ureteropelvic Junction Obstruction.- 1. Definition and Description.- 2. Incidence.- 3. Causes.- a) Aberrant Renal Blood Vessels.- b) Adhesions and Kinks.- c) High Insertion of Ureter.- d) Stenosis.- e) Functional Disorders.- f) Vesicoureteral Reflux.- 4. Clinical Aspects.- a) Superimposed Intermittent Acute Obstruction.- b) Recurrent Pyelonephritis with Loin Pain.- c) Renal Calculi.- d) Hematuria.- e) Trauma.- f) Associated Hypertension.- 5. Diagnosis.- 6. Treatment.- C. Unusual Positions of the Ureter.- I. Retrocaval (Post-caval; Circumcaval) Ureter.- 1. Definition.- 2. Incidence.- 3. Embryology.- 4. Clinical Aspects.- a) Symptoms and Signs.- b) Diagnosis.- c) Treatment.- II. Retroiliac Ureter.- III. Herniation of the Ureter.- D. Agenesis, Aplasia, Blind Ending.- I. Agenesis.- II. Aplasia.- III. Blind Ending Ureters.- E. Congenital Dilatation of the Ureter.- I. With Obstruction.- 1. Congenital Ureteral Stricture.- 2. Distal Ureteral Atresia.- 3. Congenital Ureteral Valves (or Folds).- II. Without Obstruction.- F. Twists, Kinks, Congenital Diverticula, Blockage by Vessels.- I. Spiral Twist or Torsion of Ureter.- II. Ureteral Kinks.- III. Diverticulum of the Ureter.- IV. Blockage of the Ureter by Blood Vessels.- References.- Congenital Abnormalities of the Bladder.- A. Development of the Bladder.- I. The Cloaca.- II. The Genital Tubercle.- III. Separation of the Bladder from the Intestinal Tract.- IV. Formation of the Trigone.- V. Regression of the Allantoic Duct.- VI. Development of the Bladder Musculature.- VII. Fetal Descent of the Urinary Tract.- VIII. Summary of Important Early Developmental Events.- B. Congenital Abnormalities of the Bladder.- I. Agenesis of the Bladder.- 1. Incidence.- 2. Pathology.- 3. Treatment.- II. Urachal Abnormalities.- 1. Incidence.- 2. Pathology.- 3. Classification.- 4. Clinical features.- a) Congenital Patent Urachus.- b) Vesico-urachal Diverticulum.- c) Umbilical Cyst and Sinus.- d) Alternating Urachal Sinus.- 5. Treatment.- III. Duplication of the Bladder.- 1. Classification.- a) Complete Duplication.- b) Incomplete Duplication.- c) Sagittal Septum.- d) Frontal Septum.- e) Multiseptate Bladder.- f) Hourglass Bladder.- 2. Pathology.- 3. Clinical Features.- 4. Diagnosis.- 5. Treatment.- IV. Bladder Mucosal Redundancy (Trigonal Curtains).- 1. Clinical Features.- 2. Treatment.- V. Congenital Cyst of the Bladder.- 1. Clinical Features.- 2. Pathology.- 3. Diagnosis and Treatment.- VI. Congenital Bladder Diverticulum.- 1. Incidence.- 2. Etiology.- 3. Pathology.- 4. Diagnosis.- 5. Treatment.- VII. The Exstrophy-epispadias Complex.- 1. Pathology.- 2. Pseudo-exstrophy of the Bladder.- 3. Classical Exstrophy of the Bladder.- a) Pathology.- b) Anatomy.- c) Clinical Features.- d) Treatment.- ?) Urinary Diversion.- ?) Functional Closure of Exstrophy.- 4. Cloacal Exstrophy.- a) Pathology.- b) Anatomy and Clinical Features.- c) Treatment.- 5. Variants of Exstrophy.- a) Superior Vesical Fissure.- b) Duplicate Exstrophy.- c) Inferior Vesical Fissure.- d) Incomplete Cloacal Exstrophy.- 6. Epispadias.- a) Pathology.- b) Description.- ?) Balanic Type.- ?) Penile Type.- ?) Penopubic Type.- c) Clinical Features.- d) Treatment.- References.- Anomalies of the Bladder Neck.- Anomalies of the Bladder Neck.- Symptoms.- Diagnosis.- The Normal Bladder Neck.- Technique.- Cinefluorographic Appearance of Normal Bladder Outlets During Micturition.- Dilatation of the Urethra Distal to Wide Bladder Necks.- Bladder Neck Dysfunction.- Errors in Diagnosis and Management.- The Roentgen Appearance of the Normal Male Bladder Neck.- Bladder Neck Dysfunction in the Male.- Bladder Neck Dysfunction Accompanying Posterior Urethral Valves.- Summary.- References.- Anomalies of the Urethra.- I. Introduction.- II. Congenital Absence of the Urethra.- 1. Complete Absence of the Urethra.- 2. Obliteration at the Level of the Bladder Neck.- 3. Obliteration at the Level of the Membranous Urethra.- 4. Obliteration of the Penile Urethra.- III. Meatal Stenosis.- IV. Distal Urethral Stenosis.- V. Congenital Urethral Stricture.- VI. Congenital Valves of the Female Urethra.- VII. Congenital Hypertrophy of the Verumontanum.- VIII. Anterior Urethral Valves.- IX. Diverticula of the Anterior Urethra.- 1. Incidence.- 2. Etiology and Pathology.- 3. Clinical Presentation and Diagnosis.- 4. Treatment and Prognosis.- X. Double Urethra and Accessory Urethra.- 1. Complete Double Urethra.- 2. Incomplete Dorsal Accessory Urethra-blind.- 3. Incomplete Dorsal Accessory Urethra-Communicating with the Urethra.- 4. Incomplete Ventral Accessory Urethra-blind.- 5. Double Urethra in the Female.- XI. Posterior Urethral Valves.- 1. Incidence.- 2. Etiology and Pathology.- 3. Clinical Presentation.- 4. Diagnosis.- 5. Treatment.- 6. Prognosis.- References.- Anomalies of the Male Genitalia.- A. The Undescended Testicle.- I. Causes.- II. Associated Conditions.- III. Differential Diagnosis.- IV. Method of Examination.- V. Medical Treatment.- VI. Surgical Treatment.- 1. Indications.- 2. Surgical Anatomy.- 3. Surgical Principles.- 4. Technique.- 5. Special Situations.- 6. Other Methods.- 7. Results.- B. Other Abnormalities of the Testicle.- Anorchism and Hypoplasia.- C. Appendages of the Testicle, Epididymis and Cord.- D. Anomalies of the Tunics of the Testicle and Scrotum.- E. Epididymis.- F. Vas Deferens.- G. Prostate.- H. Seminal Vesicles.- References.- Hypospadias.- Embryology and Morphology.- Incidence.- Female Hypospadias.- Therapeutic Principles.- Techniques for Correction of Chordec.- Position of New Meatus.- Basic Types of Urethroplasty.- General Results.- Personal Experiences.- Complications.- References.- Anomalies of the Female Genitalia.- I. Anomalies of the Vulva and Vagina.- 1. Reduplication of the Vulva.- 2. Masculinization of the Vulva.- 3. Imperforate Hymen.- 4. Polyps of the Hymen.- 5. Transverse Vaginal Septum.- II. Anomalies of the Mullerian Ducts.- 1. Congenital Absence of the Vagina and Uterus.- 2. Maldevelopment of the Vagina and Uterus.- a) Obstructive Maldevelopment of the Vagina and Uterus.- b) Non-obstructive Maldevelopment of the Vagina and Uterus.- III. Anomalies of the Ovary.- 1. Congenital Absence of the Ovaries.- 2. Supernumerary Ovaries.- 3. Accessory Ovaries.- 4. Displaced Ovaries.- References.- The Intersex States.- I. Criteria of Sex.- 1. The Sex Chromatin and Sex Chromosomes.- 2. Gonadal Structure.- 3. Morphology of External Genitalia.- 4. Morphology of the Internal Genitalia.- 5. Hormonal Status.- 6. Gender Role.- II. The Definition and Classification of Hermaphroditism.- III. Ovarian Agenesis and Dysgenesis.- 1. The Pathology of Gonadal Agenesis.- 2. The Diagnosis in Newborn Infants.- 3. The Diagnosis in Adolescence.- 4. Endocrine Findings.- IV. True Hermaphroditism.- 1. General Considerations.- 2. Criteria for Diagnosis.- 3. Classification of True Hermaphroditism.- 4. Clinical Features.- 5. Sex Chromosome Complements.- 6. Principles of Treatment.- V. Klinefelter's Syndrome.- 1. Clinical Characteristics.- 2. Laboratory Findings.- 3. Pathological and Cytogenetic Findings.- 4. Treatment.- VI. Double X Males.- VII. Multiple X Syndromes.- VIII. Female Hermaphroditism Due to Congential Adrenal Hyperplasia.- 1. Clinical Considerations.- 2. Adrenal Pathology.- 3. Ovary.- 4. Developmental Anomalies of the Genital Tubercle and Urogenital Sinus Derivatives.- 5. Hormonal Changes.- 6. Pathogenesis of Virilizing Adrenal Hyperplasia.- 7. Diagnosis.- 8. Treatment.- IX. Female Hermaphroditism without Progressive Masculinization.- 1. Classification.- 2. Maternal Ingestion of Androgen.- 3. Maternal Androgen Tumor.- 4. Idiopathic.- 5. Special or Non-Specific Female Hermaphroditism.- X. Male Hermaphroditism.- 1. General Considerations.- 2. Classification.- 3. Masculinizing Male Hermaphrodites.- 4. Asymmetrical Gonadal Differentiation.- 5. Feminizing Male Hermaphrodites (Testicular Feminization Syndrome).- 6. Sex Chromosomes in Male Hermaphroditism.- XI. Differential Diagnosis of Infants with Ambiguous Genitalia.- XII. The Treatment of Hermaphroditism.- 1. General Considerations.- 2. The Construction of Female External Genitalia.- References.- Author Index.