- •Preface
- •Acknowledgments
- •Contents
- •1.1 Introduction
- •1.2 Normal Embryology
- •1.3 Abnormalities of the Kidney
- •1.3.1 Renal Agenesis
- •1.3.2 Renal Hypoplasia
- •1.3.3 Supernumerary Kidneys
- •1.3.5 Polycystic Kidney Disease
- •1.3.6 Simple (Solitary) Renal Cyst
- •1.3.7 Renal Fusion and Renal Ectopia
- •1.3.8 Horseshoe Kidney
- •1.3.9 Crossed Fused Renal Ectopia
- •1.4 Abnormalities of the Ureter
- •1.5 Abnormalities of the Bladder
- •1.6 Abnormalities of the Penis and Urethra in Males
- •1.7 Abnormalities of Female External Genitalia
- •Further Reading
- •2.1 Introduction
- •2.2 Pathophysiology
- •2.3 Etiology of Hydronephrosis
- •2.5 Clinical Features
- •2.6 Investigations and Diagnosis
- •2.7 Treatment
- •2.8 Antenatal Hydronephrosis
- •Further Reading
- •3.1 Introduction
- •3.2 Embryology
- •3.3 Pathophysiology
- •3.4 Etiology of PUJ Obstruction
- •3.5 Clinical Features
- •3.6 Diagnosis and Investigations
- •3.7 Management of Newborns with PUJ Obstruction
- •3.8 Treatment
- •3.9 Post-operative Complications and Follow-Up
- •Further Reading
- •4: Renal Tumors in Children
- •4.1 Introduction
- •4.2 Wilms’ Tumor
- •4.2.1 Introduction
- •4.2.2 Etiology
- •4.2.3 Histopathology
- •4.2.4 Nephroblastomatosis
- •4.2.5 Clinical Features
- •4.2.6 Risk Factors for Wilms’ Tumor
- •4.2.7 Staging of Wilms Tumor
- •4.2.8 Investigations
- •4.2.9 Prognosis and Complications of Wilms Tumor
- •4.2.10 Surgical Considerations
- •4.2.11 Surgical Complications
- •4.2.12 Prognosis and Outcome
- •4.2.13 Extrarenal Wilms’ Tumors
- •4.3 Mesoblastic Nephroma
- •4.3.1 Introduction
- •4.3.3 Epidemiology
- •4.3.5 Clinical Features
- •4.3.6 Investigations
- •4.3.7 Treatment and Prognosis
- •4.4 Clear Cell Sarcoma of the Kidney (CCSK)
- •4.4.1 Introduction
- •4.4.2 Pathophysiology
- •4.4.3 Clinical Features
- •4.4.4 Investigations
- •4.4.5 Histopathology
- •4.4.6 Treatment
- •4.4.7 Prognosis
- •4.5 Malignant Rhabdoid Tumor of the Kidney
- •4.5.1 Introduction
- •4.5.2 Etiology and Pathophysiology
- •4.5.3 Histologic Findings
- •4.5.4 Clinical Features
- •4.5.5 Investigations and Diagnosis
- •4.5.6 Treatment and Outcome
- •4.5.7 Mortality/Morbidity
- •4.6 Renal Cell Carcinoma in Children
- •4.6.1 Introduction
- •4.6.2 Histopathology
- •4.6.4 Staging
- •4.6.5 Clinical Features
- •4.6.6 Investigations
- •4.6.7 Management
- •4.6.8 Prognosis
- •4.7 Angiomyolipoma of the Kidney
- •4.7.1 Introduction
- •4.7.2 Histopathology
- •4.7.4 Clinical Features
- •4.7.5 Investigations
- •4.7.6 Treatment and Prognosis
- •4.8 Renal Lymphoma
- •4.8.1 Introduction
- •4.8.2 Etiology and Pathogenesis
- •4.8.3 Diagnosis
- •4.8.4 Clinical Features
- •4.8.5 Treatment and Prognosis
- •4.9 Ossifying Renal Tumor of Infancy
- •4.10 Metanephric Adenoma
- •4.10.1 Introduction
- •4.10.2 Histopathology
- •4.10.3 Diagnosis
- •4.10.4 Clinical Features
- •4.10.5 Treatment
- •4.11 Multilocular Cystic Renal Tumor
- •Further Reading
- •Wilms’ Tumor
- •Mesoblastic Nephroma
- •Renal Cell Carcinoma in Children
- •Angiomyolipoma of the Kidney
- •Renal Lymphoma
- •Ossifying Renal Tumor of Infancy
- •Metanephric Adenoma
- •Multilocular Cystic Renal Tumor
- •5.1 Introduction
- •5.2 Embryology
- •5.4 Histologic Findings
- •5.7 Associated Anomalies
- •5.8 Clinical Features
- •5.9 Investigations
- •5.10 Treatment
- •Further Reading
- •6: Congenital Ureteral Anomalies
- •6.1 Etiology
- •6.2 Clinical Features
- •6.3 Investigations and Diagnosis
- •6.4 Duplex (Duplicated) System
- •6.4.1 Introduction
- •6.4.3 Clinical Features
- •6.4.4 Investigations
- •6.4.5 Treatment and Prognosis
- •6.5 Ectopic Ureter
- •6.5.1 Introduction
- •6.5.3 Clinical Features
- •6.5.4 Diagnosis
- •6.5.5 Surgical Treatment
- •6.6 Ureterocele
- •6.6.1 Introduction
- •6.6.3 Clinical Features
- •6.6.4 Investigations and Diagnosis
- •6.6.5 Treatment
- •6.6.5.1 Surgical Interventions
- •6.8 Mega Ureter
- •Further Reading
- •7: Congenital Megaureter
- •7.1 Introduction
- •7.3 Etiology and Pathophysiology
- •7.4 Clinical Presentation
- •7.5 Investigations and Diagnosis
- •7.6 Treatment and Prognosis
- •7.7 Complications
- •Further Reading
- •8.1 Introduction
- •8.2 Pathophysiology
- •8.4 Etiology of VUR
- •8.5 Clinical Features
- •8.6 Investigations
- •8.7 Management
- •8.7.1 Medical Treatment of VUR
- •8.7.2 Antibiotics Used for Prophylaxis
- •8.7.3 Anticholinergics
- •8.7.4 Surveillance
- •8.8 Surgical Therapy of VUR
- •8.8.1 Indications for Surgical Interventions
- •8.8.2 Indications for Surgical Interventions Based on Age at Diagnosis and the Presence or Absence of Renal Lesions
- •8.8.3 Endoscopic Injection
- •8.8.4 Surgical Management
- •8.9 Mortality/Morbidity
- •Further Reading
- •9: Pediatric Urolithiasis
- •9.1 Introduction
- •9.2 Etiology
- •9.4 Clinical Features
- •9.5 Investigations
- •9.6 Complications of Urolithiasis
- •9.7 Management
- •Further Reading
- •10.1 Introduction
- •10.2 Embryology of Persistent Müllerian Duct Syndrome
- •10.3 Etiology and Inheritance of PMDS
- •10.5 Clinical Features
- •10.6 Treatment
- •10.7 Prognosis
- •Further Reading
- •11.1 Introduction
- •11.2 Physiology and Bladder Function
- •11.2.1 Micturition
- •11.3 Pathophysiological Changes of NBSD
- •11.4 Etiology and Clinical Features
- •11.5 Investigations and Diagnosis
- •11.7 Management
- •11.8 Clean Intermittent Catheterization
- •11.9 Anticholinergics
- •11.10 Botulinum Toxin Type A
- •11.11 Tricyclic Antidepressant Drugs
- •11.12 Surgical Management
- •Further Reading
- •12.1 Introduction
- •12.2 Etiology
- •12.3 Pathophysiology
- •12.4 Clinical Features
- •12.5 Investigations and Diagnosis
- •12.6 Management
- •Further Reading
- •13.1 Introduction
- •13.2 Embryology
- •13.3 Epispadias
- •13.3.1 Introduction
- •13.3.2 Etiology
- •13.3.4 Treatment
- •13.3.6 Female Epispadias
- •13.3.7 Surgical Repair of Female Epispadias
- •13.3.8 Prognosis
- •13.4 Bladder Exstrophy
- •13.4.1 Introduction
- •13.4.2 Associated Anomalies
- •13.4.3 Principles of Surgical Management of Bladder Exstrophy
- •13.4.4 Evaluation and Management
- •13.5 Cloacal Exstrophy
- •13.5.1 Introduction
- •13.5.2 Skeletal Changes in Cloacal Exstrophy
- •13.5.3 Etiology and Pathogenesis
- •13.5.4 Prenatal Diagnosis
- •13.5.5 Associated Anomalies
- •13.5.8 Surgical Reconstruction
- •13.5.9 Management of Urinary Incontinence
- •13.5.10 Prognosis
- •13.5.11 Complications
- •Further Reading
- •14.1 Introduction
- •14.2 Etiology
- •14.3 Clinical Features
- •14.4 Associated Anomalies
- •14.5 Diagnosis
- •14.6 Treatment and Prognosis
- •Further Reading
- •15: Cloacal Anomalies
- •15.1 Introduction
- •15.2 Associated Anomalies
- •15.4 Clinical Features
- •15.5 Investigations
- •Further Reading
- •16: Urachal Remnants
- •16.1 Introduction
- •16.2 Embryology
- •16.4 Clinical Features
- •16.5 Tumors and Urachal Remnants
- •16.6 Management
- •Further Reading
- •17: Inguinal Hernias and Hydroceles
- •17.1 Introduction
- •17.2 Inguinal Hernia
- •17.2.1 Incidence
- •17.2.2 Etiology
- •17.2.3 Clinical Features
- •17.2.4 Variants of Hernia
- •17.2.6 Treatment
- •17.2.7 Complications of Inguinal Herniotomy
- •17.3 Hydrocele
- •17.3.1 Embryology
- •17.3.3 Treatment
- •Further Reading
- •18: Cloacal Exstrophy
- •18.1 Introduction
- •18.2 Etiology and Pathogenesis
- •18.3 Associated Anomalies
- •18.4 Clinical Features and Management
- •Further Reading
- •19: Posterior Urethral Valve
- •19.1 Introduction
- •19.2 Embryology
- •19.3 Pathophysiology
- •19.5 Clinical Features
- •19.6 Investigations and Diagnosis
- •19.7 Management
- •19.8 Medications Used in Patients with PUV
- •19.10 Long-Term Outcomes
- •19.10.3 Bladder Dysfunction
- •19.10.4 Renal Transplantation
- •19.10.5 Fertility
- •Further Reading
- •20.1 Introduction
- •20.2 Embryology
- •20.4 Clinical Features
- •20.5 Investigations
- •20.6 Treatment
- •20.7 The Müllerian Duct Cyst
- •Further Reading
- •21: Hypospadias
- •21.1 Introduction
- •21.2 Effects of Hypospadias
- •21.3 Embryology
- •21.4 Etiology of Hypospadias
- •21.5 Associated Anomalies
- •21.7 Clinical Features of Hypospadias
- •21.8 Treatment
- •21.9 Urinary Diversion
- •21.10 Postoperative Complications
- •Further Reading
- •22: Male Circumcision
- •22.1 Introduction
- •22.2 Anatomy and Pathophysiology
- •22.3 History of Circumcision
- •22.4 Pain Management
- •22.5 Indications for Circumcision
- •22.6 Contraindications to Circumcision
- •22.7 Surgical Procedure
- •22.8 Complications of Circumcision
- •Further Reading
- •23: Priapism in Children
- •23.1 Introduction
- •23.2 Pathophysiology
- •23.3 Etiology
- •23.5 Clinical Features
- •23.6 Investigations
- •23.7 Management
- •23.8 Prognosis
- •23.9 Priapism and Sickle Cell Disease
- •23.9.1 Introduction
- •23.9.2 Epidemiology
- •23.9.4 Pathophysiology
- •23.9.5 Clinical Features
- •23.9.6 Treatment
- •23.9.7 Prevention of Stuttering Priapism
- •23.9.8 Complications of Priapism and Prognosis
- •Further Reading
- •24.1 Introduction
- •24.2 Embryology and Normal Testicular Development and Descent
- •24.4 Causes of Undescended Testes and Risk Factors
- •24.5 Histopathology
- •24.7 Clinical Features and Diagnosis
- •24.8 Treatment
- •24.8.1 Success of Surgical Treatment
- •24.9 Complications of Orchidopexy
- •24.10 Infertility and Undescended Testes
- •24.11 Undescended Testes and the Risk of Cancer
- •Further Reading
- •25: Varicocele
- •25.1 Introduction
- •25.2 Etiology
- •25.3 Pathophysiology
- •25.4 Grading of Varicoceles
- •25.5 Clinical Features
- •25.6 Diagnosis
- •25.7 Treatment
- •25.8 Postoperative Complications
- •25.9 Prognosis
- •Further Reading
- •26.1 Introduction
- •26.2 Etiology and Risk Factors
- •26.3 Diagnosis
- •26.4 Intermittent Testicular Torsion
- •26.6 Effects of Testicular Torsion
- •26.7 Clinical Features
- •26.8 Treatment
- •26.9.1 Introduction
- •26.9.2 Etiology of Extravaginal Torsion
- •26.9.3 Clinical Features
- •26.9.4 Treatment
- •26.10 Torsion of the Testicular or Epididymal Appendage
- •26.10.1 Introduction
- •26.10.2 Embryology
- •26.10.3 Clinical Features
- •26.10.4 Investigations and Treatment
- •Further Reading
- •27: Testicular Tumors in Children
- •27.1 Introduction
- •27.4 Etiology of Testicular Tumors
- •27.5 Clinical Features
- •27.6 Staging
- •27.6.1 Regional Lymph Node Staging
- •27.7 Investigations
- •27.8 Treatment
- •27.9 Yolk Sac Tumor
- •27.10 Teratoma
- •27.11 Mixed Germ Cell Tumor
- •27.12 Stromal Tumors
- •27.13 Simple Testicular Cyst
- •27.14 Epidermoid Cysts
- •27.15 Testicular Microlithiasis (TM)
- •27.16 Gonadoblastoma
- •27.17 Cystic Dysplasia of the Testes
- •27.18 Leukemia and Lymphoma
- •27.19 Paratesticular Rhabdomyosarcoma
- •27.20 Prognosis and Outcome
- •Further Reading
- •28: Splenogonadal Fusion
- •28.1 Introduction
- •28.2 Etiology
- •28.4 Associated Anomalies
- •28.5 Clinical Features
- •28.6 Investigations
- •28.7 Treatment
- •Further Reading
- •29: Acute Scrotum
- •29.1 Introduction
- •29.2 Torsion of Testes
- •29.2.1 Introduction
- •29.2.3 Etiology
- •29.2.4 Clinical Features
- •29.2.5 Effects of Torsion of Testes
- •29.2.6 Investigations
- •29.2.7 Treatment
- •29.3 Torsion of the Testicular or Epididymal Appendage
- •29.3.1 Introduction
- •29.3.2 Embryology
- •29.3.3 Clinical Features
- •29.3.4 Investigations and Treatment
- •29.4.1 Introduction
- •29.4.2 Etiology
- •29.4.3 Clinical Features
- •29.4.4 Investigations and Treatment
- •29.5 Idiopathic Scrotal Edema
- •29.6 Testicular Trauma
- •29.7 Other Causes of Acute Scrotum
- •29.8 Splenogonadal Fusion
- •Further Reading
- •30.1 Introduction
- •30.2 Imperforate Hymen
- •30.3 Vaginal Atresia
- •30.5 Associated Anomalies
- •30.6 Embryology
- •30.7 Clinical Features
- •30.8 Investigations
- •30.9 Management
- •Further Reading
- •31: Disorders of Sexual Development
- •31.1 Introduction
- •31.2 Embryology
- •31.3 Sexual and Gonadal Differentiation
- •31.5 Evaluation of a Newborn with DSD
- •31.6 Diagnosis and Investigations
- •31.7 Management of Patients with DSD
- •31.8 Surgical Corrections of DSD
- •31.9 Congenital Adrenal Hyperplasia (CAH)
- •31.10 Androgen Insensitivity Syndrome (Testicular Feminization Syndrome)
- •31.13 Gonadal Dysgenesis
- •31.15 Ovotestis Disorders of Sexual Development
- •31.16 Other Rare Disorders of Sexual Development
- •Further Reading
- •Index
Index
A
Abdomino-scrotal hydrocele, 410, 413 Acute epididymitis, 560, 610–612 Acute scrotum
acute epididymitis, 610–612 bacterial epididymitis, 611 causes, 613–614
common causes, 601 differential diagnosis, 601
idiopathic scrotal edema, 612–613 orchitis, 610
testicular torsion, 601–608 testicular trauma, 613
torsion of testicular appendices/epididymal appendage
appendix testis, 608 clinical features, 609
clinical investigations, 609–610 diagrammatic representation, 608, 609 embryology, 609
epididymal appendix, 608 incidence, 608
treatment, 609–610 Adrenal rests, 588
AIS. See Androgen insensitivity syndrome (AIS) Alagille syndrome, 180
5-Alpha reductase deficiency (5ARD), 638, 656, 672–673
Ambiguous genitalia. See Disorder of sex development (DSD)
Amyand’s hernia, 406, 407 Anatomical/gonadal sex, 638 Androgen insensitivity syndrome (AIS)
CAIS
amenorrhea, 667 inguinal hernias, 666–669
receptor-negative type, 666 receptor-positive type, 666 symptoms, 638, 665
diagnosis, 665, 667 gonadectomy, 666–668 hormone replacement, 668 malignant tumors, 665–667
mild androgen insensitivity syndrome, 670 PAIS
clinical photographs, 669 diagnosis, 669, 670 symptoms, 638, 668 treatment, 670
pathophysiology, 665 Angiomyolipoma
classification, 157–158 clinical features, 158 diagnosis, 158–159 histopathology, 157 incidence, 156–157 treatment and prognosis, 159
Antenatal hydronephrosis antibiotic prophylaxis, 68 bilateral, 67–68
diuretic renography, 67 fetal, 65, 66
infants, management of, 67 moderate and mild unilateral, 68 pathological causes, 66 postnatal radiology, 66–67
risk of, 65–66 severe unilateral, 68 severity, 66, 67
surgical management, 68–69 systematic review, 66 Tc99m MAG3, 67 ultrasonography, 66
VCUG, 67 vesicoureteral reflux, 65
Anti-Müllerian hormone (AMH) effect, 287–291, 530, 531, 538, 643, 648, 654–655, 674, 676, 677
Aphallia, 683
Aposthia, 683
Ascent testis, 531, 534
Aspiration and irrigation procedure, 519–522 Autosomal dominant polycystic kidney disease (ADPKD), 8, 10, 18, 179
Autosomal recessive polycystic kidney disease (ARPKD), 8, 10, 12, 18, 179
B
Bacterial epididymitis, 611
Bardet-Biedl syndrome, 621–623, 625
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Bartter syndrome, 274, 282
Beckwith-Wiedemann syndrome (BWS), 105, 112, 180 Bell-clapper deformity, 553, 554, 603
Berdon syndrome. See Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIH syndrome)
Bilateral nonpalpable testes, 529–530 Bilateral vanishing testes, 684 Bladder
abnormalities agenesis, 32
clinical photographs, 22, 27 cloacal exstrophy, 22, 25, 26, 28 diverticulum, 26, 27, 30, 31 duplication, 32
ears, 30, 32 megacystis, 32–34
persistent urachus, 26, 28–31 septation, 32
Bladder exstrophy-epispadias anatomical defects
bladder exstrophy, 339–340 cloacal extrophy, 340 epispadias, 340
exstrophy variants, 341
cloacal exstrophy (see Cloacal exstrophy) congenital abnormalities, 337
definition, 337 embryology, 341–342 epispadias
complete, 342, 343 etiology, 343–344 female, 348–349 glanular, 343, 344 incidence, 342 isolated, 342 penile, 344, 345 penopubic, 344, 345 prognosis, 349–350
surgical repair, male, 347–351 treatment, 345–347
exstrophy (see Bladder exstrophy) incidence, 338
manifestation, 338 musculoskeletal defects, 341 neurologic defects, 341 phallus, 339–340
staged repair, 338 Bladder extrophy
associated anomalies, 354–355 classic bladder, 353–354
cloacal exstrophy, part of, 350–353 diagnosis, 351
management and evaluation antibiotics, 356
bladder augmentation, 359 bladder neck reconstruction, 359 cardiac echo, 356
CPRE, 357
early bladder closure, 357 latex precautions, 356
Mitchell technique, 357 modified Bryant’s traction, 357 modified Buck’s traction, 357 MSRE, 358–359
Mummy wrapping, 357 pelvic osteotomies, 357
postoperative complications, 360 prevalence, 350
radiological evaluation, 353 repair, 352–353
risk factors, 350
surgical technique, 355–356 treatment goals, 352
Bosniak classification, 11
Branchio-oto-renal (BOR) syndrome, 175, 180 Busse’s hernia, 405, 406
Byer’s flaps, 464, 465, 471
C
CAH. See Congenital adrenal hyperplasia (CAH) CAIS. See Complete androgen-insensitivity
syndrome (CAIS) Canal of Nuck hydroceles, 412
Children’s Oncology Group staging system, 580, 581 Chordee without hypospadias, 454
Circumplast circumcision, 488, 489, 494 Clear cell sarcoma of the kidney (CCSK)
clinical features, 140 definition, 138 diagnosis, 140 histopathology, 140–141
pathophysiology, 139–140 prognosis, 142
treatment, 141–142
Clitoromegaly, 39, 40, 625, 652, 658, 684–685 Cloacal anomalies
associated anomalies
anorectal malformations, 383 Currarino triad, 383
genital anomalies, 384
sacrum and spine anomalies, 383 tethered cord, 383
urogenital anomaly, 383 vaginal/uterine septation, 384
classification
long common channel, 384 short common channel, 384
clinical features
abdominal distension, 384, 385 Currarino triad, 385
distended vagina, 385 hydrocolpos, 384, 385 hydronephrosis, 385
persistent cloaca diagnosis, 384, 385 common single perineal opening, 381, 382 definition, 381
incidence, 381 investigations
abdominal ultrasonography, 386
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cystoscopy and vaginoscopy, 386, 387 distal loopogram, 386, 387 echocardiography, 386
MRI, 386, 388
sacral ratio calculations, 386 sacrum plain radiography, 386 spinal ultrasonography, 386 spine plain radiography, 386
management
abdominal approach, 387–389 bowel, 390
colostomy, 387–390
cystoscopy and vaginoscopy, 390 dilated sigmoid colon, 389 goals, 387
hydrometrocolpos, 388
intermittent catheterization, 390–391 laparotomy, 388, 389
Mitrofanoff procedure, 390, 391 prognostic factors, 390 PSARVUP, 387–388
stages, 387–388 surgical treatment, 388
total urogenital mobilization, 389 Cloacal exstrophy, 532, 535
anomalies, 360, 361 abdominal wall musculature
deficiency, 418
bladder augmentation, 418–420 double and absent appendix, 418 gastrointestinal duplications, 418 lower extremity, 418
short bowel syndrome, 418 spinal and skeletal, 417–418 tallipes equinovarus, 418 upper urinary tract, 418 urological malformations, 418
anorectal agenesis, 362, 417 associated anomalies
cardiovascular and pulmonary, 365 gastrointestinal, 365
mullerian and testicular, 365 neurospinal, 365
spinal and skeletal, 364 upper urinary tract, 365
clinical features, 366–367
ileo-cecal region, 415, 416, 418–419 prolapsed bowel, 420
prolapsing terminal ileum, 419 ureteric catheters, 420
complications, 372 components, 415, 416 embryology, 416–417 etiology, 363, 415 genitalia, 415
management, 366–368, 419–421 OEIS complex, 360 omphalocele absence, 417 pathogenesis, 363–364, 415 prenatal diagnosis, 364
prevalence, 360, 415 prognosis, 371 skeletal changes, 363
surgical reconstruction, 370–371 surgical repair, 368–370
urinary incontinence management, 371 Cobb’s collar, 427
Combined 17α-hydroxylase/17,20-lyase deficiency, 685
Communicating hydroceles, 411, 412 Complete androgen-insensitivity
syndrome (CAIS) amenorrhea, 667 inguinal hernias, 666–669
receptor-negative type, 666 receptor-positive type, 666 symptoms, 638, 665
Complete Primary Repair of Exstrophy (CPRE), 357 Congenital adrenal hyperplasia (CAH)
3-beta-hydroxysteroid dehydrogenase deficiency, 663
biochemical defect, 659
clinical photographs, 636, 637, 660, 661 diagnosis, 660
11-hydroxylase deficiency, 663
21-hydroxylase deficiency, 659, 660, 662–663 incidence, 660
maternal androgens, 664 metabolic defects, 660 mineralocorticoteroids, 660 prenatal diagnosis, 660 salt-wasting nephropathy, 660 symptoms, 638
treatment, 664–665 urogenital sinus, 660–662
Congenital anomalies of the kidney and urinary tract (CAKUT), 1–2
Congenital megaureter clinical presentation, 225 complications, 234 dilated ureter, 217
investigations and diagnosis, 225–231 non-refluxing/non-obstructed megureter primary megaureter, 218, 222, 225 secondary megaureter, 219, 222
obstructed megaureter
primary megaureter, 219, 220, 222–224 secondary megaureter, 219, 220, 222
refluxing megaureter
primary megaureter, 218, 219, 222, 223 secondary megaureter, 219, 222
refluxing/obstructed megaureter primary megaureter, 222, 224–225 secondary megaureter, 219, 222
treatment and prognosis, 231–234 VUR, 218, 234
Congenital mesoblastic nephroma (CMN). See Mesoblastic nephroma
Congenital obstructing posterior urethral membrane (COPUM), 425
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Congenital ureteral anomalies clinical features, 188 duplex (duplicated) system
abdominal and pelvic ultrasound, 194–195 bifid ureters, 191
classification, 192–193 clinical features, 193
complete ureteral duplication, 189, 191–192 complication, 192
CT scanning, 192 CT urography, 195
incomplete ureteral duplication, 189, 191 IVU, 193, 194
micturating cystourethrography, 195 MR urography, 195, 196
nuclear scan, 195 scintigraphy, 192
treatment and prognosis, 195–197 unilateral or bilateral, 189, 190
ectopic ureter association with, 197
bilateral single-system ureteral ectopia, 197 clinical features, 199–200
diagnosis, 200
duplicated renal collecting system, 197 embryology and pathophysiology, 198–199 in females, 197, 198
in males, 197, 198
surgical treatment, 200–202 Weigert-Meyer rule, 197
etiology, 187–188
investigations and diagnosis, 188–189 mega ureter, 212–215
ureterocele (see Ureterocele) VUR, 208–212
Congenital urological malformations bladder
agenesis, 32
clinical photographs, 22, 27 cloacal exstrophy, 22, 25, 26, 28 diverticulum, 26, 27, 30, 31 duplication, 32
ears, 30, 32 megacystis, 32–34
persistent urachus, 26, 28–31 septation, 32
CAKUT, 1–2 ESKD, 1
female external genitalia clitoral hypertrophy, 39–40
distal urethral stenosis, 36–37 female epispadias, 37, 39 labial fusion, 37
urethral prolapse, 40–41 kidney
agenesis, 5–6
crossed fused renal ectopia, 15–17 cysts, 11–12
dysplasia, 8–10
ectopic kidney, 12, 13, 17–18
fusion, 12, 13
horseshoe kidney, 12, 14–15 hypoplasia, 6–7
multicystic kidney, 8–10 polycystic kidney disease, 8, 10 supernumerary kidneys, 7
normal embryology, 2–4 penis and urethra, in males
apenia, 33
congenital urethral stricture, 35, 36 diphallia, 33–34
epispadias, 36, 39–40 hypospadias, 35–38 megalopenis, 34, 35 micopenis, 33–34
posterior urethral valves, 35, 37 ureter
duplication of, 18–20 ectopic ureteral orifice, 20
mega-ureter, obstruction, 21–22, 25, 26 ureteral atresia, 18, 19
ureterocele, 19, 21 ureteropelvic junction, 20–23
Coronal hypospadias, 458, 459 Cryptorchidism. See Undescended testes Cystic dysplasia, 179–181, 589, 590
Cystic partially differentiated nephroblastoma (CPDN), 165–168
Cystourethroscopy, 265, 433
D
Denys-Drash syndrome, 103, 104, 109, 112, 113, 639, 648
Desmopressin (DDAVP), 127
Detrusor external sphincter dyssynergia (DSD), 302 Detrusor hyperreflexia with impaired contractility
(DHIC), 303
Detrusor sphincter dyssynergia with detrusor hyperreflexia (DSD-DH), 298, 302–303
Dimercaptosuccinic acid (DMSA), 33, 82, 183, 195, 205, 242, 255, 256, 263, 316, 329, 433
Diphallia, 33, 34, 36, 683 Direct inguinal hernia, 405
Disorder of sex development (DSD) Aaronson’s classification, 650 AIS, 638
aphallia, 683 aposthia, 683 5ARD, 638, 672–673
aromatase deficiency, 682–683 aromatase excess syndrome, 683
17β-hydroxysteroid dehydrogenase deficiency, 682 bilateral vanishing testes, 684
CAH (see Congenital adrenal hyperplasia (CAH)) clinical investigations, 653–655
clinical photographs, 635 clitoris, dermoid cyst, 637 clitoromegaly, 684–685 cloacal exstrophy, 636
Index |
691 |
|
|
combined 17α-hydroxylase/17,20-lyase deficiency, 685
deficient testosterone biosynthesis, 678 definition, 636
diagnosis, 653–655 diphallia, 683
gonads disorders, 650, 651 phenotype disorders, 650
sex chromosomes disorders, 650 dysgenetic DSD, 650
EIS, 685 embryology, 639–642
gonadal dysgenesis, 673–677 imperforated hymen, 637 incidence, 637
isolated 17,20-lyase deficiency, 685 Klinefelter syndrome, 683
Leydig cell hypoplasia, 685 MGD, 638
micropenis, 683–384 MIS, 638
newborn evaluation, 651–653 ovarian DSD, 650 ovotesticular DSD, 650 ovotestis DSD, 679–682 PMDS, 670–672
PPSH, 685
sex chromosome DSDs, 646–647
sexual and gonadal differentiation, 642–645 surgical corrections, 657–659
testicular DSD, 650 treatment, 656–657 triple X syndrome, 683 uterus didelphys, 684
46,XX disorders, 648–649 46,XY disorders, 647–648
Distal penile hypospadias, 458, 460
Distal renal tubular acidosis (dRTA), 272, 274–275, 282, 284
Diuretic nuclear renography, 189 Dorsal penile nerve block (DPNB), 482 Down syndrome, 15, 532
DSD. See Disorder of sex development (DSD) Duplex (duplicated) system
abdominal and pelvic ultrasound, 194–195 bifid ureters, 191
classification, 192–193 clinical features, 193
complete ureteral duplication, 189, 191–192 complication, 192
CT scanning, 192 CT urography, 195
incomplete ureteral duplication, 189, 191 IVU, 193, 194
micturating cystourethrography, 195 MR urography, 195, 196
nuclear scan, 195 scintigraphy, 192
treatment and prognosis, 195–197 unilateral or bilateral, 189, 190
DYG (The Double Y Glanuloplasty) procedure, 467 Dysgenetic male pseudohermaphroditism (DMP),
673, 676
Dysgenetic testis, 343, 531, 533, 646–648, 650, 655, 673–674, 676, 677
E
Ectopic kidney, 4, 16–18, 72, 181, 355, 622 Ectopic testicles, 529, 531, 533
Ectopic ureter association with, 197
bilateral single-system ureteral ectopia, 197 clinical features, 199–200
diagnosis, 200
duplicated renal collecting system, 197 embryology and pathophysiology, 198–199 in females, 197, 198
in males, 197, 198
surgical treatment, 200–202 Weigert-Meyer rule, 197
Encysted hydroceles, 412 Endocrine emergency situation, 635
End stage renal disease (ESRD), 2, 7, 241, 425, 427, 434
Epididymal appendage acute scrotal pain, 566 appendix testis, 608 clinical features, 567, 609
clinical investigations, 567–568, 609–610 diagrammatic representation, 566, 608, 609 embryology, 567, 609
epididymal appendix, 608 incidence, 566, 608 symptoms, 566
treatment, 567–568, 609–610 Epididymo-orchitis, 199, 554, 555, 559, 601, 610 Estrogen insensitivity syndrome (EIS), 685
European Society for Pediatric Endocrinology (ESPE), 645, 646
Extravaginal torsion
clinical and intraoperative photographs, 556, 557, 562
clinical features, 562–565 etiology of, 562 treatment, 563–566
Extravesical detrusorrhaphy, 266, 267
F
Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), 272, 282, 284
Female epispadias diagnosis, 348 incidence, 348 prognosis, 349 surgical repair, 349
Female pseudohermaphrodites. See Congenital adrenal hyperplasia (CAH)
Foley’s catheter, 282, 369, 472, 474, 629
692 |
Index |
|
|
Fowler-Stephens principle, 541 |
encysted hydrocele, 412 |
Fraser syndrome, 621, 625 |
noncommunicating, 411–412 |
Frasier syndrome, 639 |
reactive, 412 |
|
treatment, 413 |
|
Hydronephrosis |
G |
abdominal and pelvic ultrasound, 58, 59 |
Gastroschesis, 532, 535 |
abdominal CT-scan, 62, 63 |
Genotypic/chromosomal sex, 637 |
abdominal x-ray, 58, 59 |
Germ-cell tumors |
antegrade/retrograde pyelography, 62, 64 |
ITGCN, 572 |
antenatal hydronephrosis |
mixed forms, 575 |
antibiotic prophylaxis, 68 |
precursor lesions, 574 |
bilateral, 67–68 |
pure forms, 574–575 |
diuretic renography, 67 |
seminomas, 571, 576 |
fetal, 65, 66 |
teratoma, 570–572, 576, 586–587 |
infants, management of, 67 |
yolk sac tumors, 570, 571, 575, 585–586 |
moderate and mild unilateral, 68 |
Glandular hypospadias, 458 |
pathological causes, 66 |
Glenn-Anderson repair, 266, 267 |
postnatal radiology, 66–67 |
Gomco clamp, 479, 482, 486, 488, 490, 495 |
risk of, 65–66 |
Gonadal dysgenesis, 570, 656–657 |
severe unilateral, 68 |
asymmetrical gonads, 674 |
severity, 66, 67 |
definition, 673 |
surgical management, 68–69 |
degree of virilization, 674 |
systematic review, 66 |
DMP, 676 |
Tc99m MAG3, 67 |
dysgenetic testis, 673–674 |
ultrasonography, 66 |
embryology, 674 |
VCUG, 67 |
malignancy risk, 675 |
vesicoureteral reflux, 65 |
MGD, 676 |
classification, 54–57 |
partial gonadal dysgenesis, 675 |
clinical features, 57–58 |
pathogenesis, 674 |
distension and dilation, 43, 44 |
Perrault syndrome, 676 |
diuretic renography, 59, 61 |
pure gonadal dysgenesis, 676–677 |
etiology |
streak gonad, 673 |
antenatal, 54–55 |
treatment, 676 |
causes of, 52, 54 |
Turner syndrome, 675 |
MCDK, 52–54 |
XX gonadal dysgenesis, 676 |
neonates and children, 49–53 |
Gonadoblastomas, 381, 571, 574, 578, 579, 590, |
IVU, 58, 60 |
667, 674–676 |
MCU, 58, 60, 61 |
Granulosa cell tumour, 575 |
megaureter, 44, 47 |
|
MRU, 62–64 |
|
obstruction, 43–44 |
H |
pathophysiology |
Hereditary hypophosphatemic rickets with hypercalciuria |
acute hydronephrosis, 46 |
(HHRH), 274, 275, 282 |
chronic hydronephrosis, 46–49 |
Hermaphroditism, 636, 645, 646, 650, 679 |
functional causes, 45, 48 |
High-flow priapism |
glomerular filtration rate, 46 |
causes, 504, 505 |
hypertension, 47 |
characteristics, 507 |
longstanding, 49 |
diagnosis, 508 |
obstruction, 45–46 |
genitourinary trauma, 507 |
persistent hydronephrosis, 48–49 |
SCD, 513–515 |
urinary stasis, 47, 49 |
treatment, 502, 511–512 |
prenatally diagnosis, 44 |
Horseshoe kidney, 12, 14–15, 18, 26, 72, 104, 112, 181, |
renal urography, 61–62 |
355, 365, 383, 418, 622 |
severity, 45 |
Human papilloma virus (HPV), 478, 485 |
SFU grade, 44–45 |
Hydrocele, 614, 615 |
signs and symptoms, 43 |
abdomino-scrotal, 413 |
treatment, 63, 65 |
canal of Nuck, 412 |
ureterovesical junction, 44, 45, 47 |
communicating, 411, 412 |
vesicoureteral reflux, 44, 46 |
embryology, 410 |
Hydro-ureteronephrosis, 440 |
Index |
693 |
|
|
Hypercalciuria, 273–274 Hypoplastic testis, 531, 533 Hypospadias
chordee shape, 451–453
chordee without hypospadias, 454 classification
coronal, 458, 459 distal penile, 458, 460 glandular, 458 midshaft, 458, 460 penoscrotal, 458, 461 perineal, 458, 462
proximal penile, 458, 461 subcoronal, 458, 459
clinical features
abnormal prepuce and normal meatus, 464 ectopic meatus on surface of penis, 462, 463 glanular groove, 462, 463
hooded foreskin, 463 MIP, 463
severe chordee, 463 cosmetics, 454
curvature of the penis, 452, 453 definition, 451
DSD, 457
ectopic urethral meatus, 452 effects, 455
embryology, 455–456 etiology, 456 hypospadiologists, 454 incidence, 451
lack of prepuce, 452
mild vesicoureteral reflux, 457 MIP, 451, 454, 456, 463, 487 postoperative complications
deviated penis, 475 hairy urethra, 474–475 immediate, 473 long-term, 474
meatal stenosis, 474 urethral diverticula, 474, 475 urethral strictures, 474
urethrocutaneous fistula, 473, 474 postoperative penis, 454
prostatic utricle, 457 repair, 454–455
scrotal transposition, 453 treatment
Byer’s flaps, 464, 465 glanular hypospadias, 467 HCG, 464
hormonal therapy, 464, 466 LABO technique, 468 lateral based flap, 468 Mathieu technique, 468 middle hypospadias, 467
posterior hypospadias, 468, 471 posthypospadias repair, 466, 467 psychological effect, 464, 466 surgical repair, 464, 465
testosterone injection, 464 TIP procedure, 467–470, 472 urethroplasty, 467, 468
undescended testes, 455–457 urinary diversion, 472 winged prepuce, 452
Hypovirilization, 570
Hypoxanthine-guanine phosphoribosyl transferase (HPRT) deficiency, 278, 384
I
Idiopathic hypercalciuria, 273, 274, 279, 283 Idiopathic scrotal edema, 554, 560, 601, 612–613 Imperforate hymen, 619–621
Incarcerated inguinal hernia, 403, 404, 601, 613, 614 Indirect inguinal hernia, 353, 401, 404–405
Inguinal hernias
Amyand’s hernia, 406, 407 Busse’s hernia, 405, 406 clinical features, 403–404 complications
hydrocele formation and recurrence, 410 hypesthesia, 410
iatrogenic cryptorchidism, 410 incarceration, 406–408 infertility, 410
metachronous contralateral hernia, 410 scrotal hematoma, 410
strangulation, 408, 409 testicular atrophy, 410 wound infection, 410
direct, 405 etiology, 402–403 incidence, 401–402 indirect, 404–405 Littre’s hernia, 405 Maydl’s hernia, 406
Richter’s hernia, 405 sliding, 405 treatment, 408
International Classification System for VUR, 246–247
International Society of Pediatric
Oncology (SIOP), 103, 107, 113, 121, 124 Intersex. See Disorder of sex development (DSD) Intratubular germ cell neoplasia (ITGCN), 572 Intrauterine testicular torsion, 529, 533, 562,
602, 608
Intravenous pyelography (IVP), 32, 81, 183, 268, 280 Intravenous urography (IVU), 58, 60
duplex (duplicated) system, 193, 194 ectopic ureter, 200
PUJ obstruction, 81, 83
Ischemic priapism. See Low flow priapism Isolated 17,20-lyase deficiency, 685
J
Juvenile granulosa cell tumors, 572, 576, 587, 588
694 |
Index |
|
|
K
Kallmann’s syndrome, 35, 532 Khitan. See Male circumcision Kidney
abnormalities agenesis, 5–6
crossed fused renal ectopia, 15–17 cysts, 11–12
dysplasia, 8–10
ectopic kidney, 12, 13, 17–18 fusion, 12, 13
horseshoe kidney, 12, 14–15 hypoplasia, 6–7
multicystic kidney, 8–10 polycystic kidney disease, 8, 10 supernumerary kidneys, 7
Klinefelter syndrome, 538, 577, 578, 646, 650, 683
L
Lateral Based Onlay (LABO) technique, 468 Laurence-Moon-Biedl syndrome, 532
Lawson Wilkins Pediatric Endocrine Society (LWPES), 645, 646
Leydig cell hypoplasia, 678, 685
Leydig cell tumor, 569, 570, 575, 587–588 Lich-Gregoire repair, 266, 267
Littre’s hernia, 405 Low flow priapism
causes, 502, 504–506, 508 characteristic features, 508 corpora cavernosa, 507 diagnosis, 508 management, 503
pain, 508
permanent cavernosal structural damage, 503 SCD, 513, 514
treatment, 503, 510–511 venous drainage, 507
M
Magnetic resonance urography (MRU), 16, 62–64, 82, 189, 192, 196, 200, 205, 257
MAGPI (Meatal Advancement Glanduloplasty Incorporated) procedure, 467
Male circumcision anatomy, 479–480 benefits, 478 complications, 479
bleeding, 492–493, 496 concealed penis, 493, 497 gangrene glans penis, 493, 499 incomplete circumcision, 493, 499 infection, 493
meatal stenosis, 493, 496 pain, 492
psychological effects, 494 retained plastibel, 493, 498 sexual effects, 493–494
skin bridges, 493, 498 skin chordee, 493, 498
unsatisfactory cosmetic appearance, 493, 497 urethral fistula, 493, 499
definition, 477 devices used, 479
formal surgical circumcision, 478 history
Africans, 480, 481 Arabian Peninsula, 480 Christianity, 481 Egypt, 480
Encyclopedia Britannica, 482 Great Britain, 481
HIV infection, 482
Jewish and Islamic faiths, 480 Judaism, 480
masturbation, 481 Mediterraneans, 480 Middle Eastern, 480 Muslim males, 480 Oceania, 480 Philippines, 481
religious significance, 481 United States, 481
indications balanitis/posthitis, 483
contraindications, 485–486
HPV and cervical cancer management, 485 medical, 483
paraphimosis, 483 penile cancer, 485 phimosis, 483
STDs management, 484 UTIs management, 484
pain management, 482 pathophysiology, 479
plastibel circumcision, 478, 479 prevalence, 477–478
surgical procedure
bone cutter, 488, 489 circumplast, 489, 494 epispadias, 487
Gomco clamp, 490, 495 hypospadias, 487, 488 interventions, 486 megameatus, 487, 488 Mogen clamp, 490, 495 open, 486
oral acetaminophen/paracetamol, 486 plastibel circumcision, 488–494 portable bipolar diathermy, 489, 493 Shang ring, 490, 496
Malignant rhabdoid tumor (MRT) chromosome 22, 143
clinical features, 145–146 definition, 143
diagnosis and diagnosis, 146–149 etiology, 144
histologic findings, 144–145
Index |
695 |
|
|
mortality/morbidity, 150 pathophysiology, 144 presentation, 144
treatment and outcome, 149–150 Mathieu technique, 468
Maydl’s hernia, 406 Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome,
621–623
MCDK. See Multi cystic dysplastic kidney (MCDK) McKusick-Kaufman syndrome, 623, 625
Median umbilical ligament, 2, 4, 393 Megacystis-microcolon-intestinal hypoperistalsis
syndrome (MMIH syndrome) associated anomalies, 376–378 clinical features, 376
definition, 373
dilated stomach, 374, 376–378 dilated urinary balder, 374, 376–378 etiology, 374–376
hydronephrosis, 373, 374, 376 lower contrast enema, 375 prenatal diagnosis, 373, 376 prognosis, 377
treatment
ileostomy, 377, 379
multi-organ transplantation, 377, 378 multivisceral transplantation, 378 vesicostomy, 377
Megameatus with intact prepuce (MIP), 451, 454, 456, 463, 487
Mega ureter, 21, 22, 187, 212–215 Mesoblastic nephroma
CBC, 137 classification, 133–134
clinical features, 136–137 CT-scan, 137, 138 definition, 132 differential diagnosis, 132 epidemiology, 134 histopathology, 134–136 live function tests, 137 MRI, 137–138
paraneoplastic syndromes, 132 plain radiograph, 137 prognosis, 138
serum electrolyte, BUN and creatinine, 137
treatment, 138 ultrasound, 137
Mesonephros, 2, 3, 174, 198, 596 Metanephric adenoma (MA)
clinical features, 164–165 diagnosis, 164
histologic analysis, 163–164 histopathology, 164 treatment, 165
Metanephros, 2–4, 17, 174
Micropenis, 34–36, 485, 538, 625, 652, 654, 668, 683–685
Micturating cystourethrogram (MCU), 58–61
Micturating cystourethrogram (MCUG), 205, 435, 440 Micturition function, 296, 299
Midshaft hypospadias, 458, 460, 467, 468 Mild androgen insensitivity syndrome, 670, 683 Mitrofanoff technique, 390, 391, 437, 440 Mixed germ cell tumors, 571, 574, 575, 587
Mixed gonadal dysgenesis (MGD), 638, 646, 647, 651, 654, 674, 676
Mogen clamp, 479, 482, 486, 488, 490, 495 Moorman’s ring. See Cobb’s collar Müllerian duct cyst, 445, 448–449
Mullerian inhibiting substance (MIS) deficiency, 638 Multi cystic dysplastic kidney (MCDK), 8, 9, 52–54
associated anomalies, 180–181 bilateral, 173, 174 classification, 179
clinical features, 182 complication, 174 embryology, 174–175
etiology and pathophysiology, 175 histologic findings, 175–178 investigations, 182–184
natural history of, 178–179 noncommunicating cysts, 173, 174 treatment, 183–185
unilateral, 173, 174
Multilocular cystic renal tumor (MCRT) abdominal ultrasound and CT-scan findings, 167 calcification, 168
CPDN, 165, 166
cystic nephroma, 165–167 differential diagnosis, 166 gross features, 167 microscopic features, 167 modification, 165 treatment, 168
Mummy wrapping, 357
N
NBSD. See Neurogenic bladder sphincter dysfunction (NBSD)
Neurogenic bladder sphincter dysfunction (NBSD) acquired causes, 295
anticholinergics Fesoterodine, 318 oxybutynin, 317–318 Tolterodine L-tartrate, 318 Trospium, 318
Botulinum A toxin injections, 318 classification of, 313, 314
clean intermittent catheterization, 316–317 congenital neural tube defects, 295 conservative management of, 295 detrusor muscle, 295
etiology and clinical features advantages, 306 anticholinergic drugs, 305 causes, 304
cerebral palsy, 307–309
696 |
Index |
|
|
Neurogenic bladder sphincter dysfunction (NBSD) (cont.) clean intermittent catheterization, 305
detrusor areflexia, 303 detrusor hyperreflexia, 302 detrusor instability, 303 DHIC, 303
DSD, 302 DSD-DH, 302–303
myelomeningocele, 304, 305 neurogenic dysfunction, 304 occult spinal dysraphism, 306–307 outflow obstruction, 303 overactive bladder, 303
peripheral neuropathy, 304 sacral agenesis, 307, 308 sacral cord injury, 304 surgical procedures, 306 traumatic injuries, spine, 309 urinary retention, 303
investigations and diagnosis, 310–313 management, 314–316
medical management, 296 pathophysiological changes of, 301–302 risk factors, 296
surgical management, 320–321 tricyclic antidepressant drugs, 318–320 urinary bladder (see Urinary bladder) urinary sphincter, 295
urodynamic assessment, 295, 296
Neuronal nicotinic acetylcholine receptor (ηAChR), 375 Noncommunicating hydroceles, 411–412
Nonischemic priapism. See High-flow priapism Non-refluxing/non-obstructed megureter, 217, 222 Noonan syndrome, 532
O
Obstructed megaureter, 217, 218, 221–222, 231 Occult spinal dysraphism, 306–307
OEIS complex. See Cloacal exstrophy Oligomeganephronia, 6–7, 18 Omphaloceles, 365, 532, 535
Open antireflux surgery, 265, 266, 268 Orchidopexy, 527, 529, 538, 539, 541 Orchitis, 610
Ovotestis DSD, 679–682
P
PAIS. See Partial androgen insensitivity syndrome (PAIS)
Pampiniform venous plexus, 545
Partial androgen insensitivity syndrome (PAIS) clinical photographs, 669
diagnosis, 669, 670 symptoms, 638, 668 treatment, 670
Partial androgen resistance, 656
Partial gonadal dysgenesis, 647, 648, 651, 655, 673, 675 Patent urachuss, 395–397
Pelviureteric junction (PUJ) obstruction abdominal CT-scan, 82, 89 abdominal MRI, 82, 85
abdominal radiograph, 81 abdominal ultrasound, 81, 82 clinical features, 76–80 definition, 71
diagnosis, 71
diagrammatic representation, 71, 72 diuretic renography, 82, 83, 85, 86 embryology, 73
etiology
primary causes, 74, 75 secondary causes, 74–77
follow-up, 97–98 incidence, 71
intermittent UPJ obstruction, 81 intravenous urography, 81, 83 intrinsic, causes of, 71–72 newborns, management of, 88–89 pathophysiology, 73–74 post-operative complications, 97–98 prenatal evaluation, 79–80
pressure flow studies, 87–88 retrograde/antegrade pyelography, 86–87 serum electrolytes, BUN and creatinine, 81 treatment
asymptomatic patients, 90–92 balloon dilatations, 95–97 endopyeloplasty:, 95 endoscopic treatment, 94–95 laparoscopic pyeloplasty, 93–94 open surgical therapy, 91–93 patients selection, 89 ureterocalicostomy, 95
urine analysis and culture, 81 urological anomalies, 72 VCUG, 86
Penis at 12, 638
Penoscrotal hypospadias, 38, 446, 458, 461, 647 Perineal hypospadias, 38, 446, 458, 462, 532 Perrault syndrome, 673, 676
Persistent Müllerian duct syndrome (PMDS), 670–672 AMH, 287
classification, 291
clinical features of, 287, 291–292 embryology of, 287–290 etiology and inheritance, 290–291 prognosis, 293
treatment, 292–293
Phenotypic sex, 636, 638, 642, 645, 650, 674 Phosphoribosyl pyrophosphate synthetase superactivity
(PRPSS), 277–278, 284
Plastibel circumcision, 478, 479, 482, 486, 488–494, 498 Politano-Leadbetter procedure, 266–267
Polycystic kidney disease (PCKD), 8, 10, 179, 182–183 Pontine micturition center (PMC), 297–298, 301 Posterior sagittal anorectovaginourethroplasty
(PSARVUP), 382, 383, 387, 388 Posterior urethral valve (PUV)
Index |
697 |
|
|
classification, 426 type I, 427 type II, 427 type III, 427
definition, 423
delayed presentation, 427 embryology, 424–425 ESRD, 425, 427, 434 fertility, 441
follow-ups, 438–439 hydro-ureteronephrosis, 440 incidence, 423, 427 investigations and diagnosis
antenatal ultrasound, 428, 430–431 chest radiographs, 428
computed tomography, 432 cystourethroscopy, 433 IVU, 431–432
MRI, 432
nuclear imaging, 432–433
plain abdominal radiographs, 428
renal and bladder ultrasonography, 428, 429 serum electrolytes, 428
urethral valves, 429 urodynamic studies, 433 VCUG, 429–433
long-term outcomes, 439–440 management
aims, 433–434
augmentation cystoplasty, 437 bladder, 435
continent appendicovesicostomy, 437 cutaneous ureterostomies, 436 cystoscopy, 435
endoscopic treatments, 434 multidisciplinary team, 433 neonatal, 434–435
postnatal primary valve ablation, 436 prenatal intervention, 434
primary valve resection, 435 refluxing ureterostomy, 437 renal dysfunction, 434 surgical treatment, 436 urinary diversion, 434 vesicostomy, 436
medications, 437–438 pathophysiology
bladder dysfunction, 426 bladder emptying, 425 congenital obstruction, urinary
tract, 425 hydronephrosis, 426 protective mechanisms, 426 renal deterioration, 425 renal maldevelopment, 425 vesicoureteral reflux, 426
physical findings, 428 prenatal diagnosis, 427 prognosis, 438–439 pulmonary hypoplasia, 428
PUV, 440
renal transplantation, 441 vesico-ureteric reflux, 440 Postpubertal orchiopexy, 570
Potter classification of renal dysplasia, 179
Prader–Willi syndrome, 35, 532 Priapism
acute priapism, 508 causes, 505
in childhood and adolescence, 501 clinical features, 508–509
clinical investigations, 509–510 complications, 524
definition, 501
early intervention, 501, 503 erectile dysfunction, 501 etiology, 505–507
high flow priapism (see High flow priapism) low flow priapism (see Low flow priapism) medications, 505
nocturnal episodes, 501 pathophysiology, 503–505 prognosis, 512, 524
SCD (see Sickle cell disease (SCD)) stuttering priapism, 508
treatment, 510–512
Primary hyperoxaluria (PH), 275, 279, 285 Primary megaureter
non-refluxing unobstructed, 225, 232 obstructed, 221, 232
refluxing, 232 symptoms, 218 unobstructed, 221
Pronephros, 3, 174, 198 Prostatic utricular cyst
classification, 445–446 clinical features, 446 CT-scan, 447, 448 definition, 443 embryology, 445 function, 443 hypospadias, 444 investigations, 446–447 location, 443
MRI, 448
müllerian duct cyst, 448–449 Prune belly syndrome, 444 surgical treatment, 447–449 VCUG, 446–447
Proximal penile hypospadias, 458, 461
Prune belly syndrome, 30, 56, 224, 397, 444, 532, 533, 538
PSARVUP. See Posterior sagittal anorectovaginourethroplasty (PSARVUP)
Pseudovaginal perineoscrotal hypospadias (PPSH), 685
Pure gonadal dysgenesis, 578, 590, 650, 651, 653, 673, 676–677
PUV. See Posterior urethral valve (PUV)
698 |
Index |
|
|
R
Radionuclide scan, 183, 555, 606 Reactive hydroceles, 412
Refluxing megaureter, 217, 222, 224 Refluxing/obstructed megaureter, 217 Renal cell carcinoma (RCC)
classification, 153 clinical features, 154–155 diagnosis, 155 histological evidence, 150 histopathology, 151–152 incidence, 151 management, 155–156 prognosis, 156
staging, 153–154
Renal coloboma syndrome (RCS), 175, 180–181 Renal lymphoma
clinical features, 161–162 definition, 159
diagnosis, 160–161 etiology, 159–160 pathogenesis, 159–160 treatment and prognosis, 162
Renal transplantation, 424, 425, 434, 441 Renal tumors
age at presentation, 102 angiomyolipoma
classification, 157–158 clinical features, 158 diagnosis, 158–159 histopathology, 157 incidence, 156–157 treatment and prognosis, 159
CCSK
clinical features, 140 definition, 138 diagnosis, 140 histopathology, 140–141
pathophysiology, 139–140 prognosis, 142
treatment, 141–142 lymphoma
clinical features, 161–162 definition, 159
diagnosis, 160–161 etiology, 159–160 pathogenesis, 159–160 treatment and prognosis, 162
MCRT
abdominal ultrasound and CT-scan findings, 167 calcification, 168
CPDN, 165, 166
cystic nephroma, 165–167 differential diagnosis, 166 gross features, 167 microscopic features, 167 modification, 165 treatment, 168
mesoblastic nephroma CBC, 137
classification, 133–134 clinical features, 136–137 CT-scan, 137, 138 definition, 132 differential diagnosis, 132 epidemiology, 134 histopathology, 134–136 live function tests, 137 MRI, 137–138
paraneoplastic syndromes, 132 plain radiograph, 137 prognosis, 138
serum electrolyte, BUN and creatinine, 137 treatment, 138
ultrasound, 137 metanephric adenoma, 163–165
clinical features, 164–165 diagnosis, 164
histologic analysis, 163–164 histopathology, 164 treatment, 165
MRT
chromosome 22, 143 clinical features, 145–146 definition, 143
diagnosis and diagnosis, 146–149 etiology, 144
histologic findings, 144–145 mortality/morbidity, 150 pathophysiology, 144 presentation, 144
treatment and outcome, 149–150 ossifying, 162–163
RCC
classification, 153 clinical features, 154–155 diagnosis, 155 histological evidence, 150 histopathology, 151–152 incidence, 151 management, 155–156 prognosis, 156
staging, 153–154
Wilms’ tumor (see Wilms’ tumor) Retractile testicles, 529, 531, 533, 534 Retrograde pyelography, 62, 81, 86, 183
Retroperitoneal lymph node dissection (RPLND), 573, 591, 592
Richter’s hernia, 405
S
SCD. See Sickle cell disease (SCD) Schönlein-Henoch purpura, 601, 613, 614 Secondary hyperoxaluria, 276, 284 Secondary megaureter
causes of, 218–223, 226 nonrefluxing/nonobstructed megaureter, 222–223 obstructed megaureter, 221
refluxing megaureter, 222
Index |
699 |
|
|
Seminomas, 542, 543, 570–572, 574, 576, 579, 582, 585, 665, 675, 676, 681
Sertoli cell tumors, 571–575, 588–589 Sex cord/Gonadal stromal tumors
adrenal rests, 588 granulosa cell tumour, 575
incompletely differentiated, 575 juvenile granulosa cell tumors, 576, 588
Leydig cell tumor, 569, 570, 575, 587–588 mixed types, 575, 589
sertoli cell tumors, 575, 588–589 Thecoma Fibroma Group, 575
Sexually transmitted diseases (STDs), 484 Shang ring, 488, 491, 496
Sickle cell anemia, 504–505, 517 Sickle cell disease (SCD)
acute severe priapism, 515 clinical features, 517–518
clinical photographs, 501, 502, 513 complication, 501, 503 epidemiology, 514
high-flow priapism, 513–515 low flow priapism, 513, 514 mean age, 501 pathophysiology, 515–517 prepubertal period, 501
stuttering priapism, 501, 515, 521–524 treatment, 519–522
Sliding inguinal hernia, 405
Snodgrass urethroplasty. See Tubularized incised plate (TIP) repair
Society for Fetal Urology (SFU) grade, 44–45, 78–79 Spigelian hernia, 532, 534
Splenogonadal fusion causes, 613–614
clinical features, 596–598
clinical intraoperative photograph, 595, 596, 616 clinical investigations, 598
continuous splenogonadal fusion, 595–597, 616 definition, 595
diagnosis, 595, 596, 616
discontinuous splenogonadal fusion, 595–597, 616 etiology, 596
intra-operative frozen section biopsy, 595 male-to-female ratio, 615 malformations, 597
SGFLD, 597 symptoms, 595
treatment, 596, 598–599, 616
Splenogonadal fusion limb defect syndrome (SGFLD), 597
Steroidogenic factor 1 (SF-1), 639 Subcoronal hypospadias, 455, 458, 459 Suprapubic urinary diversion (cistofix), 472
T
Teratoma, 570–572, 576, 586–587
Testicular feminization syndrome, 528, 578, 665–670 Testicular microlithiasis (TM), 577, 589–590
Testicular torsion
acute scrotal pain, 553, 554 bell-clapper deformity, 553, 554 bilateral testicular torsion, 553 causes, 553, 602
clinical features, 558–560 clinical investigations, 606, 607
degree of torsion, 553, 557–558, 605–606 diagnosis, 554–556
duration of torsion, 558 etiology, 554–555
extravaginal torsion (see Extravaginal torsion) clinical features, 605
diagrammatic representation, 602 etiology, 603–604
in neonates, 603 incidence, 554
intermittent testicular torsion, 556 intrauterine testicular torsion, 602 intravaginal torsion, 556, 558
in adolescents, 602 clinical features, 604–605
diagrammatic representation, 602 etiology, 603, 604
incidence, 602 risk factors, 554–555
treatment, 560–562, 606–608 Testicular tumors
adjuvant therapy, 573 alpha-fetoprotein (AFP), 573 benign, 574
bimodal age distribution, 569 carcinoid, 575
Children’s Oncology Group staging system, 580, 581 clinical features, 579–580
clinical investigations, 582–583 collecting ducts and rete tumors, 575 cystic dysplasia, 590
distant metastatic staging, 581 epidermoid cysts, 589 etiology of, 576–579 germ-cell tumors
ITGCN, 572 mixed forms, 575
precursor lesions, 574 pure forms, 574–575 seminomas, 571, 576
teratoma, 570–572, 576, 586–587
yolk sac tumors, 570, 571, 575, 585–586 gonadal dysgenesis, 570
gonadoblastomas, 571, 574, 590 haematopoietic tumors, 575 hypovirilization, 570
incidence of, 569 inguinal orchiectomy, 572
intergroup staging system, 581 intratubular germ cell neoplasia, 570 leukemia and lymphoma, 590–591 malignant, 574
mesenchymal tumors, 575
700 |
Index |
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Testicular tumors (cont.)
mixed germ cell tumors, 571, 574, 575, 587 ovarian epithelial types tumors, 575 paratesticular rhabdomyosarcoma, 591–592 paratesticular structure tumors, 575 pediatric prepubertal testicular tumors, 571 postpubertal orchiopexy, 570
prevalence of, 569
primary testicular tumors, 574 primary tumor staging, 581 prognosis and outcomes, 592
regional lymph node staging, 581–582 retroperitoneal lymph node dissection, 573 secondary testicular tumors, 574, 575 serum tumor marker staging, 581
sex cord/Gonadal stromal tumors adrenal rests, 588
granulosa cell tumour, 575 incompletely differentiated, 575 juvenile granulosa cell tumors, 576, 588 Leydig cell tumor, 569, 570, 575,
587–588
mixed types, 575, 589
sertoli cell tumors, 575, 588–589 Thecoma Fibroma Group, 575
simple testicular cyst, 589 symptoms, 571
testis-sparing approach, 572, 573 TM, 589–590
TNM staging system, 580–581 transscrotal biopsy, 572 treatment, 583–585
Torsion of testicular appendices. See Epididymal appendage
Transurethral urinary diversion (Foley’s catheter), 472 Triple X syndrome, 683
True undescended testicles, 528, 529, 533 Tubularized incised plate (TIP) repair, 454,
467–469, 471, 472 Tuli. See Male circumcision
U
Undescended testes
American Urological Association guidelines, 528–529
ascent testis, 531, 534
bilateral nonpalpable testes, 529–530 birth defect, 527
cancer risk, 542–543 causes, 528, 531–532
clinical features and diagnosis, 537–538 clinical photograph, 527
cloacal exstrophy, 532, 535 complications, 529
congenital adrenal hyperplasia, 529 definition, 527
down syndrome, 532 dysgenetic testis, 531, 533 ectopic testicles, 529, 531, 533
embryology, 530
epididymal abnormality, 536, 537 gastroschesis, 532, 535 histopathology, 532–533 hypoplastic testis, 531, 533 incidence of, 527
infertility, 541–542 inguinal hernia, 529 Kallmann’s syndrome, 532
Laurence-Moon-Biedl syndrome, 532 Noonan syndrome, 532
normal testicular development, 530 omphaloceles, 532, 535 penoscrotal/perineal hypospadias, 532 Prader–Willi syndrome, 532 prevalence, 527, 528
prune belly syndrome, 532, 533 retractile testicles, 529, 531, 534 spermatogenesis, 531
spigelian hernia, 532, 534 symptoms, 529
testicular descent, 530–531 testicular feminization
syndrome, 528 treatment
hormonal treatment, 538–539 length-increasing maneuvers, 541 orchidopexy, 527, 529, 538, 541 surgical treatment, 539–541
true undescended testicles, 529, 533 vanished testis, 531, 533–534
Urachal cyst, 28, 30, 393, 395, 397–399 Urachal remnants
classification inflammation, 396
patent urachuss, 395, 396 urachal cyst, 395 urachal-umbilical sinus, 395, 396
vesicourachal diverticulum, 395, 396 clinical features
asymptomatic patients, 396 patent urachus, 396, 397 umbilical-urachal sinus, 397 urachal cyst, 397–399 vesicourachal diverticulum, 397
clinical problems, 393 embryology, 393–395 management, 399–400
median umbilical ligament, 393 retzius, 393
tumors, 398–399 Urachal-umbilical sinus, 395, 396 Ureter
abnormalities duplication of, 18–20
ectopic ureteral orifice, 20 mega-ureter, obstruction, 21–22, 25, 26 ureteral atresia, 18, 19
ureterocele, 19, 21 ureteropelvic junction, 20–23
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Ureterocele classification, 203 clinical features, 204
congenital saccular dilatation, 202 ectopic ureterocele, 203 incidence of, 203
investigations and diagnosis, 204–206 surgical interventions, 203
treatment, 205–208
bladder neck reconstruction, 207 conservative treatment, 206, 208 single system ureterocele, 208 surgical interventions, 208
upper pole hemi-nephrectomy, 205–207 ureterocele puncture, 205, 207 uretero-ureterostomy, 207–208
Urinary bladder
micturition function, 301
central and peripheral nervous systems, 296 parasympathetic nervous system, 298, 300 PMC, 297, 298
pudendal nerve, 296 somatic nerve, 299, 300 spinal reflux, 297
sympathetic nervous system, 298, 300 unobstructed urinary flow and bladder emptying,
296 storage function
central and peripheral nervous systems, 296 control of, 299
low-pressure reservoir, 296
Urinary diversion, 196, 303, 319–321, 339, 348, 349, 351, 357, 359, 368, 371, 372, 390, 391, 434, 437, 440, 441, 472
Urinary tract infection (UTI) antibiotics
oral treatment, 330–332 parenteral treatment, 332, 333 prophylaxis, 333, 334
clinical features, 325–327 diagnosis of, 324 etiology, 324–325 incidence of, 323–324
investigations and diagnosis, 327–330 pathophysiology, 325
urological abnormalities, 324 Urolithiasis
chemical composition, 273 classification of, 278 clinical features, 278–280 complications of, 282 cystinuria, 276–277, 284 drugs, 272
fluid intake, 272 hypercalciuria
Bartter syndrome, 274 causes of, 274 definition, 273
dent disease, 274 dRTA, 274–275
FHHNC, 274 gastrostomy tube-fed, 273
genitourinary anomalies, 273 HHRH, 275
idiopathic, 273 hyperoxaluria
primary hyperoxaluria, 275 secondary hyperoxaluria, 276
hyperuricosuria, 277–278 hypocitraturia, 276 investigations
abdominal CT-scan, 280 metabolic, 281–282 radiopaque stones, 280, 281 ultrasonography, 280
management of, 282–285
renal transplant complication, 272 risk factors for, 272, 273
urinary calcium increases, 272 urinary stones developments, 272
Uterus didelphys, 6, 684
UTI. See Urinary tract infection (UTI) Utricular cyst. See Prostatic utricular cyst
V
VACTERL association, 15, 16, 181, 423 Vaginal atresia
associated anomalies, 622 Bardet-Biedl syndrome, 621 classification, 621–622 clinical features, 623–625
congenital developmental defect, 621 embryology, 622–623
Fraser syndrome, 621 investigations, 625–628 MRKH, 621
surgical management, 628–633 syndromes, 621
Valves unilateral reflux dysplasia (VURD) syndrome, 424, 439
Vanished testis, 531, 533–534 Varicocele, 614, 615
causes, 545
clinical features, 548 diagnosis, 548–549
diagrammatic representation, 545 embolization therapy, 549 etiology, 546
grading, 547–548 incidence, 545, 546 medical treatment, 549 pathophysiology, 546–547
postoperative complications, 550 prognosis, 551
situs inversus, 546 surgical treatment, 550 treatment indications, 549
unilateral right-side varicocele, 546 Varicocelectomy, 550, 551
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Ventriculoperitoneal shunt, 403, 408, 409 Vesicourachal diverticulum, 395, 397 Vesicoureteral reflux (VUR)
antibiotics, prophylaxis, 261 anticholinergics, 261–262
atrophic kidneys secondary, 238, 239 clinical features, 250–252, 254 congenital megaureter, 218, 234 congenital ureteral anomalies, 208–212 diagnosis of, 238
etiology of, 247–249, 251–253 incidence of, 238 investigations
abdominal and pelvic CT-scan and MRU, 257, 258
abdominal ultrasound, 255 DMSA renal scan:, 256
non-contaminated urine specimen, 254 radionuclide cystography, 256 urodynamic studies, 256–257
VCUG, 254–256
medical treatment of, 259–260
micturating cystourethrogram, 237, 239, 240 mortality/morbidity, 268–269 pathophysiology, 240–243
prevalence of, 237, 238 primary, 238, 243–244 renal lesions, 239, 240
secondary, 238–239, 245, 246 surgical therapy of
endoscopic injection, 263–266
indications, surgical interventions, 262–263 surgical management, 266–268
surveillance, 262
Vessico-intestinal fissure. See Cloacal exstrophy Voiding cystourethrogram (VCUG)
congenital ureteral anomalies, 189 ectopic ureter, 200
UTI, 329–330
Voiding cystourethrography (VCUG), 67 congenital megaureter, 228–230 MCDK, 183
PUJ obstruction, 86
VUR, 244, 246–249, 254, 255 VUR. See Vesicoureteral reflux (VUR)
W
Waardenburg syndrome type 1, 181
Weigert-Meyer rule, 188, 197
Whitaker test, 88, 189, 230, 231
Williams’ syndrome, 181
Wilms’ tumor
abdominal ultrasound, 115, 117 anaplasia, 103
cases of, 102–103 chemotherapy, 104
chest CT-scan, 118, 120–122 chest radiograph, 115, 116 chromosome 11, 104 clinical features, 110–112 clinical photograph, 102 congenital anomalies, 104 elements, 103
etiology, 104–105 extrarenal, 131 favorable, 103 histopathology, 105–108
intravenous urography, 118, 119 MRI scanning, 118–120 multimodality therapy, 103 nephroblastomatosis, 108–110
plain abdominal radiograph, 114–115 prognosis
and complications, 121–123 and outcome, 130–131
rhabdomyoid differentiation, 103 risk factors, 112–113
staging, 113–114
surgical complications, 130 surgical considerations
aspiration cytology, 124
Bilateral Wilms’ tumor, 124, 126, 127 desmopressin, 127
in European, 124 hypothesis, 127
inoperable tumors, 124, 125 intracaval tumor extension, 124 lung metastasis, 127, 128
in North America, 123–124 NWTSG, 124–125 radiotherapy, 123
stage I-IV anaplasia, 127 surgical management, 128–130
thoracic CT-scan, 115, 117, 118 Wilms tumor (WT-1) gene, 639
X
45 X/ 46 XY mixed gonadal digenesis, 657
Y
Yolk sac tumors, 293, 570–576, 579, 581–587