Index

A clinical photographs , 669 Abdomino-scrotal hydrocele , 410, 413 diagnosis , 669, 670 Acute epididymitis , 560, 610–612 symptoms , 638, 668 Acute scrotum treatment , 670 acute epididymitis , 610–612 pathophysiology , 665 bacterial epididymitis , 611 Angiomyolipoma causes , 613–614 classifi cation , 157–158 common causes , 601 clinical features , 158 differential diagnosis , 601 diagnosis , 158–159 idiopathic scrotal edema , 612–613 histopathology , 157 orchitis , 610 incidence , 156–157 testicular torsion , 601–608 treatment and prognosis , 159 testicular trauma , 613 Antenatal hydronephrosis torsion of testicular appendices/epididymal antibiotic prophylaxis , 68 appendage bilateral , 67–68 appendix testis , 608 diuretic renography , 67 clinical features , 609 fetal , 65, 66 clinical investigations , 609–610 infants, management of , 67 diagrammatic representation , 608, 609 moderate and mild unilateral , 68 embryology , 609 pathological causes , 66 epididymal appendix , 608 postnatal radiology , 66–67 incidence , 608 risk of , 65–66 treatment , 609–610 severe unilateral , 68 Adrenal rests , 588 severity , 66, 67 AIS . See Androgen insensitivity syndrome (AIS) surgical management , 68–69 Alagille syndrome , 180 systematic review , 66 5-Alpha reductase defi ciency (5ARD) , 638, 656, Tc99m MAG3 , 67 672–673 ultrasonography , 66 Ambiguous genitalia . See Disorder of sex VCUG , 67 development (DSD) vesicoureteral refl ux , 65 Amyand’s hernia , 406, 407 Anti-Müllerian hormone (AMH) effect , 287–291, 530, Anatomical/gonadal sex , 638 531, 538, 643, 648, 654–655, 674, 676, 677 Androgen insensitivity syndrome (AIS) Aphallia , 683 CAIS Aposthia , 683 amenorrhea , 667 Ascent testis , 531, 534 inguinal hernias , 666–669 Aspiration and irrigation procedure , 519–522 receptor-negative type , 666 Autosomal dominant polycystic kidney disease receptor-positive type , 666 (ADPKD), 8, 10, 18, 179 symptoms , 638, 665 Autosomal recessive polycystic kidney disease diagnosis , 665, 667 (ARPKD), 8, 10, 12, 18, 179 gonadectomy , 666–668 hormone replacement , 668 malignant tumors , 665–667 B mild androgen insensitivity syndrome , 670 Bacterial epididymitis , 611 PAIS Bardet-Biedl syndrome , 621–623, 625

© Springer International Publishing Switzerland 2017 687 A.H. Al-Salem, An Illustrated Guide to Pediatric Urology, DOI 10.1007/978-3-319-44182-5 688 Index

Bartter syndrome , 274, 282 Mitchell technique , 357 Beckwith-Wiedemann syndrome (BWS) , 105, 112, 180 modifi ed Bryant’s traction , 357 Bell-clapper deformity , 553, 554, 603 modifi ed Buck’s traction , 357 Berdon syndrome . See Megacystis-microcolon-intestinal MSRE , 358–359 hypoperistalsis syndrome (MMIH syndrome) Mummy wrapping , 357 Bilateral nonpalpable testes , 529–530 pelvic osteotomies , 357 Bilateral vanishing testes , 684 postoperative complications , 360 Bladder prevalence , 350 abnormalities radiological evaluation , 353 agenesis , 32 repair , 352–353 clinical photographs , 22, 27 risk factors , 350 cloacal exstrophy , 22, 25, 26, 28 surgical technique , 355–356 diverticulum , 26, 27, 30, 31 treatment goals , 352 duplication , 32 Bosniak classifi cation , 11 ears , 30, 32 Branchio-oto-renal (BOR) syndrome , 175, 180 megacystis , 32–34 Busse’s hernia , 405, 406 persistent urachus , 26, 28–31 Byer’s fl aps , 464, 465, 471 septation , 32 Bladder exstrophy-epispadias anatomical defects C bladder exstrophy , 339–340 CAH . See Congenital adrenal hyperplasia (CAH) cloacal extrophy , 340 CAIS . See Complete androgen-insensitivity epispadias , 340 syndrome (CAIS) exstrophy variants , 341 Canal of Nuck hydroceles , 412 cloacal exstrophy (see Cloacal exstrophy ) Children’s Oncology Group staging system , 580, 581 congenital abnormalities , 337 Chordee without hypospadias , 454 defi nition , 337 Circumplast circumcision , 488, 489, 494 embryology , 341–342 Clear cell sarcoma of the kidney (CCSK) epispadias clinical features , 140 complete , 342, 343 defi nition , 138 etiology , 343–344 diagnosis , 140 female , 348–349 histopathology , 140–141 glanular , 343, 344 pathophysiology , 139–140 incidence , 342 prognosis , 142 isolated , 342 treatment , 141–142 penile , 344, 345 Clitoromegaly , 39, 40, 625, 652, 658, 684–685 penopubic , 344, 345 Cloacal anomalies prognosis , 349–350 associated anomalies surgical repair, male , 347–351 anorectal malformations , 383 treatment , 345–347 Currarino triad , 383 exstrophy (see Bladder exstrophy ) genital anomalies , 384 incidence , 338 sacrum and spine anomalies , 383 manifestation , 338 tethered cord , 383 musculoskeletal defects , 341 urogenital anomaly , 383 neurologic defects , 341 vaginal/uterine septation , 384 phallus , 339–340 classifi cation staged repair , 338 long common channel , 384 Bladder extrophy short common channel , 384 associated anomalies , 354–355 clinical features classic bladder , 353–354 abdominal distension , 384, 385 cloacal exstrophy, part of , 350–353 Currarino triad , 385 diagnosis , 351 distended vagina , 385 management and evaluation hydrocolpos , 384, 385 antibiotics , 356 hydronephrosis , 385 bladder augmentation , 359 persistent cloaca diagnosis , 384, 385 bladder neck reconstruction , 359 common single perineal opening , 381, 382 cardiac echo , 356 defi nition , 381 CPRE , 357 incidence , 381 early bladder closure , 357 investigations latex precautions , 356 abdominal ultrasonography , 386 Index 689

cystoscopy and vaginoscopy , 386, 387 prevalence , 360, 415 distal loopogram , 386, 387 prognosis , 371 echocardiography , 386 skeletal changes , 363 MRI , 386, 388 surgical reconstruction , 370–371 sacral ratio calculations , 386 surgical repair , 368–370 sacrum plain radiography , 386 urinary incontinence management , 371 spinal ultrasonography , 386 Cobb’s collar , 427 spine plain radiography , 386 Combined 17α-hydroxylase/17,20-lyase management defi ciency , 685 abdominal approach , 387–389 Communicating hydroceles , 411, 412 bowel , 390 Complete androgen-insensitivity colostomy , 387–390 syndrome (CAIS) cystoscopy and vaginoscopy , 390 amenorrhea , 667 dilated sigmoid colon , 389 inguinal hernias , 666–669 goals , 387 receptor-negative type , 666 hydrometrocolpos , 388 receptor-positive type , 666 intermittent catheterization , 390–391 symptoms , 638, 665 laparotomy , 388, 389 Complete Primary Repair of Exstrophy (CPRE) , 357 Mitrofanoff procedure , 390, 391 Congenital adrenal hyperplasia (CAH) prognostic factors , 390 3-beta-hydroxysteroid dehydrogenase PSARVUP , 387–388 defi ciency , 663 stages , 387–388 biochemical defect , 659 surgical treatment , 388 clinical photographs , 636, 637, 660, 661 total urogenital mobilization , 389 diagnosis , 660 Cloacal exstrophy , 532, 535 11-hydroxylase defi ciency , 663 anomalies , 360, 361 21-hydroxylase defi ciency , 659, 660, 662–663 abdominal wall musculature incidence , 660 defi ciency , 418 maternal androgens , 664 bladder augmentation , 418–420 metabolic defects , 660 double and absent appendix , 418 mineralocorticoteroids , 660 gastrointestinal duplications , 418 prenatal diagnosis , 660 lower extremity , 418 salt-wasting nephropathy , 660 short bowel syndrome , 418 symptoms , 638 spinal and skeletal , 417–418 treatment , 664–665 tallipes equinovarus , 418 urogenital sinus , 660–662 upper urinary tract , 418 Congenital anomalies of the kidney and urinary tract urological malformations , 418 (CAKUT), 1–2 anorectal agenesis , 362, 417 Congenital megaureter associated anomalies clinical presentation , 225 cardiovascular and pulmonary , 365 complications , 234 gastrointestinal , 365 dilated ureter , 217 mullerian and testicular , 365 investigations and diagnosis , 225–231 neurospinal , 365 non-refl uxing/non-obstructed megureter spinal and skeletal , 364 primary megaureter , 218, 222, 225 upper urinary tract , 365 secondary megaureter , 219, 222 clinical features , 366–367 obstructed megaureter ileo-cecal region , 415, 416, 418–419 primary megaureter , 219, 220, 222–224 prolapsed bowel , 420 secondary megaureter , 219, 220, 222 prolapsing terminal ileum , 419 refl uxing megaureter ureteric catheters , 420 primary megaureter , 218, 219, 222, 223 complications , 372 secondary megaureter , 219, 222 components , 415, 416 refl uxing/obstructed megaureter embryology , 416–417 primary megaureter , 222, 224–225 etiology , 363, 415 secondary megaureter , 219, 222 genitalia , 415 treatment and prognosis , 231–234 management , 366–368, 419–421 VUR , 218, 234 OEIS complex , 360 Congenital mesoblastic nephroma (CMN) . omphalocele absence , 417 See Mesoblastic nephroma pathogenesis , 363–364, 415 Congenital obstructing posterior urethral membrane prenatal diagnosis , 364 (COPUM), 425 690 Index

Congenital ureteral anomalies fusion , 12, 13 clinical features , 188 horseshoe kidney , 12, 14–15 duplex (duplicated) system hypoplasia , 6–7 abdominal and pelvic ultrasound , 194–195 multicystic kidney , 8–10 bifi d ureters , 191 polycystic kidney disease , 8, 10 classifi cation , 192–193 supernumerary kidneys , 7 clinical features , 193 normal embryology , 2–4 complete ureteral duplication , 189, 191–192 penis and urethra, in males complication , 192 apenia , 33 CT scanning , 192 congenital urethral stricture , 35, 36 CT urography , 195 diphallia , 33–34 incomplete ureteral duplication , 189, 191 epispadias , 36, 39–40 IVU , 193, 194 hypospadias , 35–38 micturating cystourethrography , 195 megalopenis , 34, 35 MR urography , 195, 196 micopenis , 33–34 nuclear scan , 195 posterior urethral valves , 35, 37 scintigraphy , 192 ureter treatment and prognosis , 195–197 duplication of , 18–20 unilateral or bilateral , 189, 190 ectopic ureteral orifi ce , 20 ectopic ureter mega-ureter, obstruction , 21–22, 25, 26 association with , 197 ureteral atresia , 18, 19 bilateral single-system ureteral ectopia , 197 ureterocele , 19, 21 clinical features , 199–200 ureteropelvic junction , 20–23 diagnosis , 200 Coronal hypospadias , 458, 459 duplicated renal collecting system , 197 Cryptorchidism . See Undescended testes embryology and pathophysiology , 198–199 Cystic dysplasia , 179–181, 589, 590 in females , 197, 198 Cystic partially differentiated nephroblastoma (CPDN) , in males , 197, 198 165–168 surgical treatment , 200–202 Cystourethroscopy , 265, 433 Weigert-Meyer rule , 197 etiology , 187–188 investigations and diagnosis , 188–189 D mega ureter , 212–215 Denys-Drash syndrome , 103, 104, 109, 112, ureterocele (see Ureterocele ) 113, 639, 648 VUR , 208–212 Desmopressin (DDAVP) , 127 Congenital urological malformations Detrusor external sphincter dyssynergia (DSD) , 302 bladder Detrusor hyperrefl exia with impaired contractility agenesis , 32 (DHIC), 303 clinical photographs , 22, 27 Detrusor sphincter dyssynergia with detrusor cloacal exstrophy , 22, 25, 26, 28 hyperrefl exia (DSD-DH) , 298, 302–303 diverticulum , 26, 27, 30, 31 Dimercaptosuccinic acid (DMSA) , 33, 82, 183, 195, duplication , 32 205, 242, 255, 256, 263, 316, 329, 433 ears , 30, 32 Diphallia , 33, 34, 36, 683 megacystis , 32–34 Direct inguinal hernia , 405 persistent urachus , 26, 28–31 Disorder of sex development (DSD) septation , 32 Aaronson’s classifi cation , 650 CAKUT , 1–2 AIS , 638 ESKD , 1 aphallia , 683 female external genitalia aposthia , 683 clitoral hypertrophy , 39–40 5ARD , 638, 672–673 distal urethral stenosis , 36–37 aromatase defi ciency , 682–683 female epispadias , 37, 39 aromatase excess syndrome , 683 labial fusion , 37 17β-hydroxysteroid dehydrogenase defi ciency , 682 urethral prolapse , 40–41 bilateral vanishing testes , 684 kidney CAH (see Congenital adrenal hyperplasia (CAH) ) agenesis , 5–6 clinical investigations , 653–655 crossed fused renal ectopia , 15–17 clinical photographs , 635 cysts , 11–12 clitoris, dermoid cyst , 637 dysplasia , 8–10 clitoromegaly , 684–685 ectopic kidney , 12, 13, 17–18 cloacal exstrophy , 636 Index 691

combined 17α-hydroxylase/17,20-lyase DYG (The Double Y Glanuloplasty) procedure , 467 defi ciency , 685 Dysgenetic male pseudohermaphroditism (DMP) , defi cient testosterone biosynthesis , 678 673, 676 defi nition , 636 Dysgenetic testis , 343, 531, 533, 646–648, 650, 655, diagnosis , 653–655 673–674, 676, 677 diphallia , 683 gonads disorders , 650, 651 phenotype disorders , 650 E sex chromosomes disorders , 650 Ectopic kidney , 4, 16–18, 72, 181, 355, 622 dysgenetic DSD , 650 Ectopic testicles , 529, 531, 533 EIS , 685 Ectopic ureter embryology , 639–642 association with , 197 gonadal dysgenesis , 673–677 bilateral single-system ureteral ectopia , 197 imperforated hymen , 637 clinical features , 199–200 incidence , 637 diagnosis , 200 isolated 17,20-lyase defi ciency , 685 duplicated renal collecting system , 197 Klinefelter syndrome , 683 embryology and pathophysiology , 198–199 Leydig cell hypoplasia , 685 in females , 197, 198 MGD , 638 in males , 197, 198 micropenis , 683–384 surgical treatment , 200–202 MIS , 638 Weigert-Meyer rule , 197 newborn evaluation , 651–653 Encysted hydroceles , 412 ovarian DSD , 650 Endocrine emergency situation , 635 ovotesticular DSD , 650 End stage renal disease (ESRD) , 2, 7, 241, 425, ovotestis DSD , 679–682 427, 434 PMDS , 670–672 Epididymal appendage PPSH , 685 acute scrotal pain , 566 sex chromosome DSDs , 646–647 appendix testis , 608 sexual and gonadal differentiation , 642–645 clinical features , 567, 609 surgical corrections , 657–659 clinical investigations , 567–568, 609–610 testicular DSD , 650 diagrammatic representation , 566, 608, 609 treatment , 656–657 embryology , 567, 609 triple X syndrome , 683 epididymal appendix , 608 uterus didelphys , 684 incidence , 566, 608 46,XX disorders , 648–649 symptoms , 566 46,XY disorders , 647–648 treatment , 567–568, 609–610 Distal penile hypospadias , 458, 460 Epididymo-orchitis , 199, 554, 555, 559, 601, 610 Distal renal tubular acidosis (dRTA) , 272, 274–275, Estrogen insensitivity syndrome (EIS) , 685 282, 284 European Society for Pediatric Endocrinology (ESPE) , Diuretic nuclear renography , 189 645, 646 Dorsal penile nerve block (DPNB) , 482 Extravaginal torsion Down syndrome , 15, 532 clinical and intraoperative photographs , 556, DSD . See Disorder of sex development (DSD) 557, 562 Duplex (duplicated) system clinical features , 562–565 abdominal and pelvic ultrasound , 194–195 etiology of , 562 bifi d ureters , 191 treatment , 563–566 classifi cation , 192–193 Extravesical detrusorrhaphy , 266, 267 clinical features , 193 complete ureteral duplication , 189, 191–192 complication , 192 F CT scanning , 192 Familial hypomagnesemia with hypercalciuria and CT urography , 195 nephrocalcinosis (FHHNC), 272, 282, 284 incomplete ureteral duplication , 189, 191 Female epispadias IVU , 193, 194 diagnosis , 348 micturating cystourethrography , 195 incidence , 348 MR urography , 195, 196 prognosis , 349 nuclear scan , 195 surgical repair , 349 scintigraphy , 192 Female pseudohermaphrodites . See Congenital treatment and prognosis , 195–197 adrenal hyperplasia (CAH) unilateral or bilateral , 189, 190 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 defi nition , 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 refl ux , 65 MGD , 676 classifi cation , 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-fl ow priapism glomerular fi ltration 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 refl ux , 44, 46 embryology , 410 Hydro-ureteronephrosis , 440 Index 693

Hypercalciuria , 273–274 testosterone injection , 464 Hypoplastic testis , 531, 533 TIP procedure , 467–470, 472 Hypospadias urethroplasty , 467, 468 chordee shape , 451–453 undescended testes , 455–457 chordee without hypospadias , 454 urinary diversion , 472 classifi cation winged prepuce , 452 coronal , 458, 459 Hypovirilization , 570 distal penile , 458, 460 Hypoxanthine-guanine phosphoribosyl transferase glandular , 458 (HPRT) defi ciency , 278, 384 midshaft , 458, 460 penoscrotal , 458, 461 perineal , 458, 462 I proximal penile , 458, 461 Idiopathic hypercalciuria , 273, 274, 279, 283 subcoronal , 458, 459 Idiopathic scrotal edema , 554, 560, 601, 612–613 clinical features Imperforate hymen , 619–621 abnormal prepuce and normal meatus , 464 Incarcerated inguinal hernia , 403, 404, 601, 613, 614 ectopic meatus on surface of penis , 462, 463 Indirect inguinal hernia , 353, 401, 404–405 glanular groove , 462, 463 Inguinal hernias hooded foreskin , 463 Amyand’s hernia , 406, 407 MIP , 463 Busse’s hernia , 405, 406 severe chordee , 463 clinical features , 403–404 cosmetics , 454 complications curvature of the penis , 452, 453 hydrocele formation and recurrence , 410 defi nition , 451 hypesthesia , 410 DSD , 457 iatrogenic cryptorchidism , 410 ectopic urethral meatus , 452 incarceration , 406–408 effects , 455 infertility , 410 embryology , 455–456 metachronous contralateral hernia , 410 etiology , 456 scrotal hematoma , 410 hypospadiologists , 454 strangulation , 408, 409 incidence , 451 testicular atrophy , 410 lack of prepuce , 452 wound infection , 410 mild vesicoureteral refl ux , 457 direct , 405 MIP , 451, 454, 456, 463, 487 etiology , 402–403 postoperative complications incidence , 401–402 deviated penis , 475 indirect , 404–405 hairy urethra , 474–475 Littre’s hernia , 405 immediate , 473 Maydl’s hernia , 406 long-term , 474 Richter’s hernia , 405 meatal stenosis , 474 sliding , 405 urethral diverticula , 474, 475 treatment , 408 urethral strictures , 474 International Classifi cation System for VUR , urethrocutaneous fi stula , 473, 474 246–247 postoperative penis , 454 International Society of Pediatric prostatic utricle , 457 Oncology (SIOP), 103, 107, 113, 121, 124 repair , 454–455 Intersex . See Disorder of sex development (DSD) scrotal transposition , 453 Intratubular germ cell neoplasia (ITGCN) , 572 treatment Intrauterine testicular torsion , 529, 533, 562, Byer’s fl aps , 464, 465 602, 608 glanular hypospadias , 467 Intravenous pyelography (IVP) , 32, 81, 183, 268, 280 HCG , 464 Intravenous urography (IVU) , 58, 60 hormonal therapy , 464, 466 duplex (duplicated) system , 193, 194 LABO technique , 468 ectopic ureter , 200 lateral based fl ap , 468 PUJ obstruction , 81, 83 Mathieu technique , 468 Ischemic priapism . See Low fl ow priapism middle hypospadias , 467 Isolated 17,20-lyase defi ciency , 685 posterior hypospadias , 468, 471 posthypospadias repair , 466, 467 psychological effect , 464, 466 J surgical repair , 464, 465 Juvenile granulosa cell tumors , 572, 576, 587, 588 694 Index

K skin bridges , 493, 498 Kallmann’s syndrome , 35, 532 skin chordee , 493, 498 Khitan . See Male circumcision unsatisfactory cosmetic appearance , 493, 497 Kidney urethral fi stula , 493, 499 abnormalities defi nition , 477 agenesis , 5–6 devices used , 479 crossed fused renal ectopia , 15–17 formal surgical circumcision , 478 cysts , 11–12 history dysplasia , 8–10 Africans , 480, 481 ectopic kidney , 12, 13, 17–18 Arabian Peninsula , 480 fusion , 12, 13 Christianity , 481 horseshoe kidney , 12, 14–15 Egypt , 480 hypoplasia , 6–7 Encyclopedia Britannica , 482 multicystic kidney , 8–10 Great Britain , 481 polycystic kidney disease , 8, 10 HIV infection , 482 supernumerary kidneys , 7 Jewish and Islamic faiths , 480 Klinefelter syndrome , 538, 577, 578, 646, 650, 683 Judaism , 480 masturbation , 481 Mediterraneans , 480 L Middle Eastern , 480 Lateral Based Onlay (LABO) technique , 468 Muslim males , 480 Laurence-Moon-Biedl syndrome , 532 Oceania , 480 Lawson Wilkins Pediatric Endocrine Society (LWPES) , Philippines , 481 645, 646 religious signifi cance , 481 Leydig cell hypoplasia , 678, 685 United States , 481 Leydig cell tumor , 569, 570, 575, 587–588 indications Lich-Gregoire repair , 266, 267 balanitis/posthitis , 483 Littre’s hernia , 405 contraindications , 485–486 Low fl ow priapism HPV and cervical cancer management , 485 causes , 502, 504–506, 508 medical , 483 characteristic features , 508 paraphimosis , 483 corpora cavernosa , 507 penile cancer , 485 diagnosis , 508 phimosis , 483 management , 503 STDs management , 484 pain , 508 UTIs management , 484 permanent cavernosal structural damage , 503 pain management , 482 SCD , 513, 514 pathophysiology , 479 treatment , 503, 510–511 plastibel circumcision , 478, 479 venous drainage , 507 prevalence , 477–478 surgical procedure bone cutter , 488, 489 M circumplast , 489, 494 Magnetic resonance urography (MRU) , 16, 62–64, 82, epispadias , 487 189, 192, 196, 200, 205, 257 Gomco clamp , 490, 495 MAGPI (Meatal Advancement Glanduloplasty hypospadias , 487, 488 Incorporated) procedure, 467 interventions , 486 Male circumcision megameatus , 487, 488 anatomy , 479–480 Mogen clamp , 490, 495 benefi ts , 478 open , 486 complications , 479 oral acetaminophen/paracetamol , 486 bleeding , 492–493, 496 plastibel circumcision , 488–494 concealed penis , 493, 497 portable bipolar diathermy , 489, 493 gangrene glans penis , 493, 499 Shang ring , 490, 496 incomplete circumcision , 493, 499 Malignant rhabdoid tumor (MRT) infection , 493 chromosome 22 , 143 meatal stenosis , 493, 496 clinical features , 145–146 pain , 492 defi nition , 143 psychological effects , 494 diagnosis and diagnosis , 146–149 retained plastibel , 493, 498 etiology , 144 sexual effects , 493–494 histologic fi ndings , 144–145 Index 695

mortality/morbidity , 150 Micturating cystourethrogram (MCUG) , 205, 435, 440 pathophysiology , 144 Micturition function , 296, 299 presentation , 144 Midshaft hypospadias , 458, 460, 467, 468 treatment and outcome , 149–150 Mild androgen insensitivity syndrome , 670, 683 Mathieu technique , 468 Mitrofanoff technique , 390, 391, 437, 440 Maydl’s hernia , 406 Mixed germ cell tumors , 571, 574, 575, 587 Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome , Mixed gonadal dysgenesis (MGD) , 638, 646, 647, 651, 621–623 654, 674, 676 MCDK . See Multi cystic dysplastic kidney (MCDK) Mogen clamp , 479, 482, 486, 488, 490, 495 McKusick-Kaufman syndrome , 623, 625 Moorman’s ring . See Cobb’s collar Median umbilical ligament , 2, 4, 393 Müllerian duct cyst , 445, 448–449 Megacystis-microcolon-intestinal hypoperistalsis Mullerian inhibiting substance (MIS) defi ciency , 638 syndrome (MMIH syndrome) Multi cystic dysplastic kidney (MCDK) , 8, 9, 52–54 associated anomalies , 376–378 associated anomalies , 180–181 clinical features , 376 bilateral , 173, 174 defi nition , 373 classifi cation , 179 dilated stomach , 374, 376–378 clinical features , 182 dilated urinary balder , 374, 376–378 complication , 174 etiology , 374–376 embryology , 174–175 hydronephrosis , 373, 374, 376 etiology and pathophysiology , 175 lower contrast enema , 375 histologic fi ndings , 175–178 prenatal diagnosis , 373, 376 investigations , 182–184 prognosis , 377 natural history of , 178–179 treatment noncommunicating cysts , 173, 174 ileostomy , 377, 379 treatment , 183–185 multi-organ transplantation , 377, 378 unilateral , 173, 174 multivisceral transplantation , 378 Multilocular cystic renal tumor (MCRT) vesicostomy , 377 abdominal ultrasound and CT-scan fi ndings , 167 Megameatus with intact prepuce (MIP) , 451, 454, calcifi cation , 168 456, 463, 487 CPDN , 165, 166 Mega ureter, 21, 22, 187, 212–215 cystic nephroma , 165–167 Mesoblastic nephroma differential diagnosis , 166 CBC , 137 gross features , 167 classifi cation , 133–134 microscopic features , 167 clinical features , 136–137 modifi cation , 165 CT-scan , 137, 138 treatment , 168 defi nition , 132 Mummy wrapping , 357 differential diagnosis , 132 epidemiology , 134 histopathology , 134–136 N live function tests , 137 NBSD . See Neurogenic bladder sphincter dysfunction MRI , 137–138 (NBSD) paraneoplastic syndromes , 132 Neurogenic bladder sphincter dysfunction (NBSD) plain radiograph , 137 acquired causes , 295 prognosis , 138 anticholinergics serum electrolyte, BUN and Fesoterodine , 318 creatinine, 137 oxybutynin , 317–318 treatment , 138 Tolterodine L-tartrate , 318 ultrasound , 137 Trospium , 318 Mesonephros , 2, 3, 174, 198, 596 Botulinum A toxin injections , 318 Metanephric adenoma (MA) classifi cation of , 313, 314 clinical features , 164–165 clean intermittent catheterization , 316–317 diagnosis , 164 congenital neural tube defects , 295 histologic analysis , 163–164 conservative management of , 295 histopathology , 164 detrusor muscle , 295 treatment , 165 etiology and clinical features Metanephros , 2–4, 17, 174 advantages , 306 Micropenis , 34–36, 485, 538, 625, 652, 654, 668, anticholinergic drugs , 305 683–685 causes , 304 Micturating cystourethrogram (MCU) , 58–61 cerebral palsy , 307–309 696 Index

Neurogenic bladder sphincter dysfunction (NBSD) (cont .) Pelviureteric junction (PUJ) obstruction clean intermittent catheterization , 305 abdominal CT-scan , 82, 89 detrusor arefl exia , 303 abdominal MRI , 82, 85 detrusor hyperrefl exia , 302 abdominal radiograph , 81 detrusor instability , 303 abdominal ultrasound , 81, 82 DHIC , 303 clinical features , 76–80 DSD , 302 defi nition , 71 DSD-DH , 302–303 diagnosis , 71 myelomeningocele , 304, 305 diagrammatic representation , 71, 72 neurogenic dysfunction , 304 diuretic renography , 82, 83, 85, 86 occult spinal dysraphism , 306–307 embryology , 73 outfl ow obstruction , 303 etiology overactive bladder , 303 primary causes , 74, 75 peripheral neuropathy , 304 secondary causes , 74–77 sacral agenesis , 307, 308 follow-up , 97–98 sacral cord injury , 304 incidence , 71 surgical procedures , 306 intermittent UPJ obstruction , 81 traumatic injuries, spine , 309 intravenous urography , 81, 83 urinary retention , 303 intrinsic, causes of , 71–72 investigations and diagnosis , 310–313 newborns, management of , 88–89 management , 314–316 pathophysiology , 73–74 medical management , 296 post-operative complications , 97–98 pathophysiological changes of , 301–302 prenatal evaluation , 79–80 risk factors , 296 pressure fl ow studies , 87–88 surgical management , 320–321 retrograde/antegrade pyelography , 86–87 tricyclic antidepressant drugs , 318–320 serum electrolytes, BUN and creatinine , 81 urinary bladder (see Urinary bladder ) treatment urinary sphincter , 295 asymptomatic patients , 90–92 urodynamic assessment , 295, 296 balloon dilatations , 95–97 Neuronal nicotinic acetylcholine receptor (ηAChR) , 375 endopyeloplasty: , 95 Noncommunicating hydroceles , 411–412 endoscopic treatment , 94–95 Nonischemic priapism . See High-fl ow priapism laparoscopic pyeloplasty , 93–94 Non-refl uxing/non-obstructed megureter , 217, 222 open surgical therapy , 91–93 Noonan syndrome , 532 patients selection , 89 ureterocalicostomy , 95 urine analysis and culture , 81 O urological anomalies , 72 Obstructed megaureter , 217, 218, 221–222, 231 VCUG , 86 Occult spinal dysraphism , 306–307 Penis at 12 , 638 OEIS complex . See Cloacal exstrophy Penoscrotal hypospadias , 38, 446, 458, 461, 647 Oligomeganephronia , 6–7, 18 Perineal hypospadias , 38, 446, 458, 462, 532 Omphaloceles , 365, 532, 535 Perrault syndrome , 673, 676 Open antirefl ux surgery , 265, 266, 268 Persistent Müllerian duct syndrome (PMDS) , 670–672 Orchidopexy , 527, 529, 538, 539, 541 AMH , 287 Orchitis , 610 classifi cation , 291 Ovotestis DSD , 679–682 clinical features of , 287, 291–292 embryology of , 287–290 etiology and inheritance , 290–291 P prognosis , 293 PAIS . See Partial androgen insensitivity treatment , 292–293 syndrome (PAIS) Phenotypic sex , 636, 638, 642, 645, 650, 674 Pampiniform venous plexus , 545 Phosphoribosyl pyrophosphate synthetase superactivity Partial androgen insensitivity syndrome (PAIS) (PRPSS), 277–278, 284 clinical photographs , 669 Plastibel circumcision , 478, 479, 482, 486, 488–494, 498 diagnosis , 669, 670 Politano-Leadbetter procedure , 266–267 symptoms , 638, 668 Polycystic kidney disease (PCKD) , 8, 10, 179, 182–183 treatment , 670 Pontine micturition center (PMC) , 297–298, 301 Partial androgen resistance , 656 Posterior sagittal anorectovaginourethroplasty Partial gonadal dysgenesis , 647, 648, 651, 655, 673, 675 (PSARVUP), 382, 383, 387, 388 Patent urachuss , 395–397 Posterior urethral valve (PUV) Index 697

classifi cation , 426 PUV , 440 type I , 427 renal transplantation , 441 type II , 427 vesico-ureteric refl ux , 440 type III , 427 Postpubertal orchiopexy , 570 defi nition , 423 Potter classifi cation of renal delayed presentation , 427 dysplasia, 179 embryology , 424–425 Prader–Willi syndrome , 35, 532 ESRD , 425, 427, 434 Priapism fertility , 441 acute priapism , 508 follow-ups , 438–439 causes , 505 hydro-ureteronephrosis , 440 in childhood and adolescence , 501 incidence , 423, 427 clinical features , 508–509 investigations and diagnosis clinical investigations , 509–510 antenatal ultrasound , 428, 430–431 complications , 524 chest radiographs , 428 defi nition , 501 computed tomography , 432 early intervention , 501, 503 cystourethroscopy , 433 erectile dysfunction , 501 IVU , 431–432 etiology , 505–507 MRI , 432 high fl ow priapism (see High fl ow priapism ) nuclear imaging , 432–433 low fl ow priapism (see Low fl ow priapism ) plain abdominal radiographs , 428 medications , 505 renal and bladder ultrasonography , 428, 429 nocturnal episodes , 501 serum electrolytes , 428 pathophysiology , 503–505 urethral valves , 429 prognosis , 512, 524 urodynamic studies , 433 SCD (see Sickle cell disease (SCD) ) VCUG , 429–433 stuttering priapism , 508 long-term outcomes , 439–440 treatment , 510–512 management Primary hyperoxaluria (PH) , 275, 279, 285 aims , 433–434 Primary megaureter augmentation cystoplasty , 437 non-refl uxing unobstructed , 225, 232 bladder , 435 obstructed , 221, 232 continent appendicovesicostomy , 437 refl uxing , 232 cutaneous ureterostomies , 436 symptoms , 218 cystoscopy , 435 unobstructed , 221 endoscopic treatments , 434 Pronephros , 3, 174, 198 multidisciplinary team , 433 Prostatic utricular cyst neonatal , 434–435 classifi cation , 445–446 postnatal primary valve ablation , 436 clinical features , 446 prenatal intervention , 434 CT-scan , 447, 448 primary valve resection , 435 defi nition , 443 refl uxing ureterostomy , 437 embryology , 445 renal dysfunction , 434 function , 443 surgical treatment , 436 hypospadias , 444 urinary diversion , 434 investigations , 446–447 vesicostomy , 436 location , 443 medications , 437–438 MRI , 448 pathophysiology müllerian duct cyst , 448–449 bladder dysfunction , 426 Prune belly syndrome , 444 bladder emptying , 425 surgical treatment , 447–449 congenital obstruction, urinary VCUG , 446–447 tract, 425 Proximal penile hypospadias , 458, 461 hydronephrosis , 426 Prune belly syndrome , 30, 56, 224, 397, 444, 532, protective mechanisms , 426 533, 538 renal deterioration , 425 PSARVUP . See Posterior sagittal renal maldevelopment , 425 anorectovaginourethroplasty (PSARVUP) vesicoureteral refl ux , 426 Pseudovaginal perineoscrotal physical fi ndings , 428 hypospadias (PPSH) , 685 prenatal diagnosis , 427 Pure gonadal dysgenesis , 578, 590, 650, 651, 653, prognosis , 438–439 673, 676–677 pulmonary hypoplasia , 428 P U V . See Posterior urethral valve (PUV) 698 Index

R classifi cation , 133–134 Radionuclide scan , 183, 555, 606 clinical features , 136–137 Reactive hydroceles , 412 CT-scan , 137, 138 R e fl uxing megaureter , 217, 222, 224 defi nition , 132 R e fl uxing/obstructed megaureter , 217 differential diagnosis , 132 Renal cell carcinoma (RCC) epidemiology , 134 classifi cation , 153 histopathology , 134–136 clinical features , 154–155 live function tests , 137 diagnosis , 155 MRI , 137–138 histological evidence , 150 paraneoplastic syndromes , 132 histopathology , 151–152 plain radiograph , 137 incidence , 151 prognosis , 138 management , 155–156 serum electrolyte, BUN and creatinine , 137 prognosis , 156 treatment , 138 staging , 153–154 ultrasound , 137 Renal coloboma syndrome (RCS) , 175, 180–181 metanephric adenoma , 163–165 Renal lymphoma clinical features , 164–165 clinical features , 161–162 diagnosis , 164 defi nition , 159 histologic analysis , 163–164 diagnosis , 160–161 histopathology , 164 etiology , 159–160 treatment , 165 pathogenesis , 159–160 MRT treatment and prognosis , 162 chromosome 22 , 143 Renal transplantation , 424, 425, 434, 441 clinical features , 145–146 Renal tumors defi nition , 143 age at presentation , 102 diagnosis and diagnosis , 146–149 angiomyolipoma etiology , 144 classifi cation , 157–158 histologic fi ndings , 144–145 clinical features , 158 mortality/morbidity , 150 diagnosis , 158–159 pathophysiology , 144 histopathology , 157 presentation , 144 incidence , 156–157 treatment and outcome , 149–150 treatment and prognosis , 159 ossifying , 162–163 CCSK RCC clinical features , 140 classifi cation , 153 defi nition , 138 clinical features , 154–155 diagnosis , 140 diagnosis , 155 histopathology , 140–141 histological evidence , 150 pathophysiology , 139–140 histopathology , 151–152 prognosis , 142 incidence , 151 treatment , 141–142 management , 155–156 lymphoma prognosis , 156 clinical features , 161–162 staging , 153–154 defi nition , 159 Wilms’ tumor (see Wilms’ tumor ) diagnosis , 160–161 Retractile testicles , 529, 531, 533, 534 etiology , 159–160 Retrograde pyelography , 62, 81, 86, 183 pathogenesis , 159–160 Retroperitoneal lymph node dissection (RPLND) , 573, treatment and prognosis , 162 591, 592 MCRT Richter’s hernia , 405 abdominal ultrasound and CT-scan fi ndings , 167 calcifi cation , 168 CPDN , 165, 166 S cystic nephroma , 165–167 SCD . See Sickle cell disease (SCD) differential diagnosis , 166 Schönlein-Henoch purpura , 601, 613, 614 gross features , 167 Secondary hyperoxaluria , 276, 284 microscopic features , 167 Secondary megaureter modifi cation , 165 causes of , 218–223, 226 treatment , 168 nonrefl uxing/nonobstructed megaureter , 222–223 mesoblastic nephroma obstructed megaureter , 221 CBC , 137 refl uxing megaureter , 222 Index 699

Seminomas , 542, 543, 570–572, 574, 576, 579, Testicular torsion 582, 585, 665, 675, 676, 681 acute scrotal pain , 553, 554 Sertoli cell tumors , 571–575, 588–589 bell-clapper deformity , 553, 554 Sex cord/Gonadal stromal tumors bilateral testicular torsion , 553 adrenal rests , 588 causes , 553, 602 granulosa cell tumour , 575 clinical features , 558–560 incompletely differentiated , 575 clinical investigations , 606, 607 juvenile granulosa cell tumors , 576, 588 degree of torsion , 553, 557–558, 605–606 Leydig cell tumor , 569, 570, 575, 587–588 diagnosis , 554–556 mixed types , 575, 589 duration of torsion , 558 sertoli cell tumors , 575, 588–589 etiology , 554–555 Thecoma Fibroma Group , 575 extravaginal torsion (see Extravaginal torsion ) Sexually transmitted diseases (STDs) , 484 clinical features , 605 Shang ring , 488, 491, 496 diagrammatic representation , 602 Sickle cell anemia , 504–505, 517 etiology , 603–604 Sickle cell disease (SCD) in neonates , 603 acute severe priapism , 515 incidence , 554 clinical features , 517–518 intermittent testicular torsion , 556 clinical photographs , 501, 502, 513 intrauterine testicular torsion , 602 complication , 501, 503 intravaginal torsion , 556, 558 epidemiology , 514 in adolescents , 602 high-fl ow priapism , 513–515 clinical features , 604–605 low fl ow priapism , 513, 514 diagrammatic representation , 602 mean age , 501 etiology , 603, 604 pathophysiology , 515–517 incidence , 602 prepubertal period , 501 risk factors , 554–555 stuttering priapism , 501, 515, 521–524 treatment , 560–562, 606–608 treatment , 519–522 Testicular tumors Sliding inguinal hernia , 405 adjuvant therapy , 573 Snodgrass urethroplasty . See Tubularized incised plate alpha-fetoprotein (AFP) , 573 (TIP) repair benign , 574 Society for Fetal Urology (SFU) grade , 44–45, 78–79 bimodal age distribution , 569 Spigelian hernia , 532, 534 carcinoid , 575 Splenogonadal fusion Children’s Oncology Group staging system , 580, 581 causes , 613–614 clinical features , 579–580 clinical features , 596–598 clinical investigations , 582–583 clinical intraoperative photograph , 595, 596, 616 collecting ducts and rete tumors , 575 clinical investigations , 598 cystic dysplasia , 590 continuous splenogonadal fusion , 595–597, 616 distant metastatic staging , 581 defi nition , 595 epidermoid cysts , 589 diagnosis , 595, 596, 616 etiology of , 576–579 discontinuous splenogonadal fusion , 595–597, 616 germ-cell tumors etiology , 596 ITGCN , 572 intra-operative frozen section biopsy , 595 mixed forms , 575 male-to-female ratio , 615 precursor lesions , 574 malformations , 597 pure forms , 574–575 SGFLD , 597 seminomas , 571, 576 symptoms , 595 teratoma , 570–572, 576, 586–587 treatment , 596, 598–599, 616 yolk sac tumors , 570, 571, 575, 585–586 Splenogonadal fusion limb defect syndrome (SGFLD) , gonadal dysgenesis , 570 597 gonadoblastomas , 571, 574, 590 Steroidogenic factor 1 (SF-1), 639 haematopoietic tumors , 575 Subcoronal hypospadias , 455, 458, 459 hypovirilization , 570 Suprapubic urinary diversion (cistofi x) , 472 incidence of , 569 inguinal orchiectomy , 572 intergroup staging system , 581 T intratubular germ cell neoplasia , 570 Teratoma , 570–572, 576, 586–587 leukemia and lymphoma , 590–591 Testicular feminization syndrome , 528, 578, 665–670 malignant , 574 Testicular microlithiasis (TM) , 577, 589–590 mesenchymal tumors , 575 700 Index

Testicular tumors (cont .) embryology , 530 mixed germ cell tumors , 571, 574, 575, 587 epididymal abnormality , 536, 537 ovarian epithelial types tumors , 575 gastroschesis , 532, 535 paratesticular rhabdomyosarcoma , 591–592 histopathology , 532–533 paratesticular structure tumors , 575 hypoplastic testis , 531, 533 pediatric prepubertal testicular tumors , 571 incidence of , 527 postpubertal orchiopexy , 570 infertility , 541–542 prevalence of , 569 inguinal hernia , 529 primary testicular tumors , 574 Kallmann’s syndrome , 532 primary tumor staging , 581 Laurence-Moon-Biedl syndrome , 532 prognosis and outcomes , 592 Noonan syndrome , 532 regional lymph node staging , 581–582 normal testicular development , 530 retroperitoneal lymph node dissection , 573 omphaloceles , 532, 535 secondary testicular tumors , 574, 575 penoscrotal/perineal hypospadias , 532 serum tumor marker staging , 581 Prader–Willi syndrome , 532 sex cord/Gonadal stromal tumors prevalence , 527, 528 adrenal rests , 588 prune belly syndrome , 532, 533 granulosa cell tumour , 575 retractile testicles , 529, 531, 534 incompletely differentiated , 575 spermatogenesis , 531 juvenile granulosa cell tumors , 576, 588 spigelian hernia , 532, 534 Leydig cell tumor , 569, 570, 575, symptoms , 529 587–588 testicular descent , 530–531 mixed types , 575, 589 testicular feminization sertoli cell tumors , 575, 588–589 syndrome, 528 Thecoma Fibroma Group , 575 treatment simple testicular cyst , 589 hormonal treatment , 538–539 symptoms , 571 length-increasing maneuvers , 541 testis-sparing approach , 572, 573 orchidopexy , 527, 529, 538, 541 TM , 589–590 surgical treatment , 539–541 TNM staging system , 580–581 true undescended testicles , 529, 533 transscrotal biopsy , 572 vanished testis , 531, 533–534 treatment , 583–585 Urachal cyst , 28, 30, 393, 395, 397–399 Torsion of testicular appendices . See Epididymal Urachal remnants appendage classifi cation Transurethral urinary diversion (Foley’s catheter) , 472 infl ammation , 396 Triple X syndrome , 683 patent urachuss , 395, 396 True undescended testicles , 528, 529, 533 urachal cyst , 395 Tubularized incised plate (TIP) repair , 454, urachal-umbilical sinus , 395, 396 467–469, 471, 472 vesicourachal diverticulum , 395, 396 Tuli . See Male circumcision clinical features asymptomatic patients , 396 patent urachus , 396, 397 U umbilical-urachal sinus , 397 Undescended testes urachal cyst , 397–399 American Urological Association vesicourachal diverticulum , 397 guidelines, 528–529 clinical problems , 393 ascent testis , 531, 534 embryology , 393–395 bilateral nonpalpable testes , 529–530 management , 399–400 birth defect , 527 median umbilical ligament , 393 cancer risk , 542–543 retzius , 393 causes , 528, 531–532 tumors , 398–399 clinical features and diagnosis , 537–538 Urachal-umbilical sinus , 395, 396 clinical photograph , 527 Ureter cloacal exstrophy , 532, 535 abnormalities complications , 529 duplication of , 18–20 congenital adrenal hyperplasia , 529 ectopic ureteral orifi ce , 20 defi nition , 527 mega-ureter, obstruction , 21–22, 25, 26 down syndrome , 532 ureteral atresia , 18, 19 dysgenetic testis , 531, 533 ureterocele , 19, 21 ectopic testicles , 529, 531, 533 ureteropelvic junction , 20–23 Index 701

Ureterocele FHHNC , 274 classifi cation , 203 gastrostomy tube-fed , 273 clinical features , 204 genitourinary anomalies , 273 congenital saccular dilatation , 202 HHRH , 275 ectopic ureterocele , 203 idiopathic , 273 incidence of , 203 hyperoxaluria investigations and diagnosis , 204–206 primary hyperoxaluria , 275 surgical interventions , 203 secondary hyperoxaluria , 276 treatment , 205–208 hyperuricosuria , 277–278 bladder neck reconstruction , 207 hypocitraturia , 276 conservative treatment , 206, 208 investigations single system ureterocele , 208 abdominal CT-scan , 280 surgical interventions , 208 metabolic , 281–282 upper pole hemi-nephrectomy , 205–207 radiopaque stones , 280, 281 ureterocele puncture , 205, 207 ultrasonography , 280 uretero-ureterostomy , 207–208 management of , 282–285 Urinary bladder renal transplant complication , 272 micturition function , 301 risk factors for , 272, 273 central and peripheral nervous systems , 296 urinary calcium increases , 272 parasympathetic nervous system , 298, 300 urinary stones developments , 272 PMC , 297, 298 Uterus didelphys , 6, 684 pudendal nerve , 296 UTI . See Urinary tract infection (UTI) somatic nerve , 299, 300 Utricular cyst . See Prostatic utricular cyst spinal refl ux , 297 sympathetic nervous system , 298, 300 unobstructed urinary fl ow and bladder emptying , V 296 VACTERL association , 15, 16, 181, 423 storage function Vaginal atresia central and peripheral nervous systems , 296 associated anomalies , 622 control of , 299 Bardet-Biedl syndrome , 621 low-pressure reservoir , 296 classifi cation , 621–622 Urinary diversion , 196, 303, 319–321, 339, 348, 349, clinical features , 623–625 351, 357, 359, 368, 371, 372, 390, 391, 434, congenital developmental defect , 621 437, 440, 441, 472 embryology , 622–623 Urinary tract infection (UTI) Fraser syndrome , 621 antibiotics investigations , 625–628 oral treatment , 330–332 MRKH , 621 parenteral treatment , 332, 333 surgical management , 628–633 prophylaxis , 333, 334 syndromes , 621 clinical features , 325–327 Valves unilateral refl ux dysplasia (VURD) diagnosis of , 324 syndrome, 424, 439 etiology , 324–325 Vanished testis , 531, 533–534 incidence of , 323–324 Varicocele , 614, 615 investigations and diagnosis , 327–330 causes , 545 pathophysiology , 325 clinical features , 548 urological abnormalities , 324 diagnosis , 548–549 Urolithiasis diagrammatic representation , 545 chemical composition , 273 embolization therapy , 549 classifi cation of , 278 etiology , 546 clinical features , 278–280 grading , 547–548 complications of , 282 incidence , 545, 546 cystinuria , 276–277, 284 medical treatment , 549 drugs , 272 pathophysiology , 546–547 fl uid intake , 272 postoperative complications , 550 hypercalciuria prognosis , 551 Bartter syndrome , 274 situs inversus , 546 causes of , 274 surgical treatment , 550 defi nition , 273 treatment indications , 549 dent disease , 274 unilateral right-side varicocele , 546 dRTA , 274–275 Varicocelectomy , 550, 551 702 Index

Ventriculoperitoneal shunt , 403, 408, 409 Wilms’ tumor Vesicourachal diverticulum , 395, 397 abdominal ultrasound , 115, 117 Vesicoureteral refl ux (VUR) anaplasia , 103 antibiotics, prophylaxis , 261 cases of , 102–103 anticholinergics , 261–262 chemotherapy , 104 atrophic kidneys secondary , 238, 239 chest CT-scan , 118, 120–122 clinical features , 250–252, 254 chest radiograph , 115, 116 congenital megaureter , 218, 234 chromosome 11 , 104 congenital ureteral anomalies , 208–212 clinical features , 110–112 diagnosis of , 238 clinical photograph , 102 etiology of , 247–249, 251–253 congenital anomalies , 104 incidence of , 238 elements , 103 investigations etiology , 104–105 abdominal and pelvic CT-scan and extrarenal , 131 MRU , 257, 258 favorable , 103 abdominal ultrasound , 255 histopathology , 105–108 DMSA renal scan: , 256 intravenous urography , 118, 119 non-contaminated urine specimen , 254 MRI scanning , 118–120 radionuclide cystography , 256 multimodality therapy , 103 urodynamic studies , 256–257 nephroblastomatosis , 108–110 VCUG , 254–256 plain abdominal radiograph , 114–115 medical treatment of , 259–260 prognosis micturating cystourethrogram , 237, 239, 240 and complications , 121–123 mortality/morbidity , 268–269 and outcome , 130–131 pathophysiology , 240–243 rhabdomyoid differentiation , 103 prevalence of , 237, 238 risk factors , 112–113 primary , 238, 243–244 staging , 113–114 renal lesions , 239, 240 surgical complications , 130 secondary , 238–239, 245, 246 surgical considerations surgical therapy of aspiration cytology , 124 endoscopic injection , 263–266 Bilateral Wilms’ tumor , 124, 126, 127 indications, surgical interventions , 262–263 desmopressin , 127 surgical management , 266–268 in European , 124 surveillance , 262 hypothesis , 127 Vessico-intestinal fi ssure . See Cloacal exstrophy inoperable tumors , 124, 125 Voiding cystourethrogram (VCUG) intracaval tumor extension , 124 congenital ureteral anomalies , 189 lung metastasis , 127, 128 ectopic ureter , 200 in North America , 123–124 UTI , 329–330 NWTSG , 124–125 Voiding cystourethrography (VCUG) , 67 radiotherapy , 123 congenital megaureter , 228–230 stage I-IV anaplasia , 127 MCDK , 183 surgical management , 128–130 PUJ obstruction , 86 thoracic CT-scan , 115, 117, 118 VUR , 244, 246–249, 254, 255 Wilms tumor (WT - 1 ) gene , 639 VUR . See Vesicoureteral refl ux (VUR)

X W 45 X/ 46 XY mixed gonadal digenesis , 657 Waardenburg syndrome type 1 , 181 Weigert-Meyer rule , 188, 197 Whitaker test , 88, 189, 230, 231 Y Williams’ syndrome , 181 Yolk sac tumors , 293, 570–576, 579, 581–587