9.4.2 Ectopic ureter
In the majority of cases, the upper pole is dysplatic and requires heminephro-ureterectomy. For the exceptional case in which the upper pole has function worth preserving, ureteral re-implantation into the bladder or uereteropyelostomy in combination with partial ureterectomy are therapeutic options.
9.5 REFERENCES Ureterocele
1. Austin PF, Cain MP, Casale AJ, Hiett AK, Rink RC.
Prenatal bladder outlet obstruction secondary to ureterocele. Urology 1998; 52:1132-1135.
Borer JG, Cisek LJ, Atala A, Diamond DA, Retik AB, Peters CA. Pediatric retroperitoneoscopic nephrectomy using 2 mm instrumentation. J Urol 1999; 162: 1725-1729; discussion 1730.
Cain MP, Pope JC, Casale AJ, Adams MC, Keating MA, Rink RC.
Natural history of refluxing distal ureteral stumps after nephrectomy and partial ureterectomy for vesicoureteral reflux. J Urol 1998; 160: 1026-1027.
4. Cendron J, Melin Y, Valayer J.
Traitement simplifie de I'ureterocele chez I'enfant. A propos de 35 cas. Chir Ped 1980; 21: 121-124.
5. Decter RM, Roth DR, Gonzales ET.
Individualized treatment of ureteroceles. J Urol 1989; 142: 535-537.
6. Di Benedetto V, Montfort G.
How prenatal utrasound can change the treatment of ectopic ureterocele in neonates? Eur J Pediatr Surg 1997; 7: 338-340.
7. El Ghoneimi A, Lottmann H, Odet E, Bonnin F, Aigrain Y.
L'ureteropyelostomie dans les duplicites obstructives, une intervention simple et fiable chez I'enfant. Ann Urol (Paris) 1998; 32: 241-246.
8. Husmann D, Strand B, Ewalt D, Clement M, Kramer S, Allen T.
Management of ectopic ureterocele associated with renal duplication: a comparison of partial nephrectomy and endoscopic decompression. J Urol 1999; 162: 1406-1409.
9. Janetschek G, Seibold J, Radmayr C, Bartsch G.
Laparoscopic heminephroureterectomy in pediatric patients. J Urol 1997; 158: 1928-1930.
10. Jayanthi VR, Koff SA.
Long-term outcome of transurethral puncture of ectopic ureteroceles: initial and late problems. J Urol 1999; 162: 1077-1080.
11. Moscovici J, Galinier P, Berrogain N, Juricic M.
Traitement des ureteroceles avec duplicite pyelo-ureterale chez I'enfant. A propos de 64 observations. Ann Urol (Paris) 1999; 33: 369-376.
Montfort G, Ruys JM, Coquet M, Roth K, Louis C, Bocciardi A. Surgical management of duplex ureteroceles. J Ped Surg 1992; 27: 634-638.
Pfister C, Ravasse P, Barret E, Petit T, Mitrofanoff P.
The value of endoscopic treatment for ureteroceles during the neonatal period. J Urol 1998; 159:1006-1009.
14. Rickwood AMK, Reiner I, Jones M, Pournaras C.
Current management of duplex system ureteroceles: experience with 41 patients. BrJ Urol 1992; 70: 196-200.
15. Roy GT, Desai S, Cohen RC.
Ureteroceles in children: an ongoing challenge. Pediatr Surg Int 1997; 12: 44-48.
16. Sherz HC, Kaplan GW, Packer MG, Brock WA.
Ectopic ureteroceles surgical management with preservation of continence. Review of 60 cases. J Urol 1989; 142:538-541.
17. Stephens D.
Coeco-ureteroceles and concepts on the embryology and etiology of ureteroceles. Aust - New Zeal J Surg 1971; 40: 239-248.
Ectopic ureter
1. Carico C, Lebowitz RL.
Incontinence due to an infrasphincteric ectopic ureter: why the delay in diagnosis and what the radiologist can do about it. Pediatr Radiol 1998; 28: 942-949.
2. Cendron J, Schulman CC.
Ectopie ureterale. In: Urologie pediatrique. Flammarion Medecine Sciences: Paris, 1985, 147-153.
3. El Ghoneimi A, Miranda J, Truong T, Montfort G.
Ectopic ureter with complete ureteric duplication: conservative surgical management. J Pediatr Surg 1996; 31: 467-472.
4. Komatsu K, Niikura S, Maeda Y, Ishiura Y, Yokoyama O, Namiki M.
Single ectopic vaginal ureter diagnosed by computed tomography. Urol Int 1999; 63: 147-150.
5. Plaire JC, Pope JC, Kropp BP, Adams MC, Keating MA, Rink RC, Casale AJ. Management of ectopic ureters: experience with the upper tract approach.
J Urol 1997; 158: 1245-1247.
6. Robert M, Ennouchi JM, Chevallier P, Guiter J, Averous M.
Diagnostic des abouchements ectopiques de I'uretere dans le tractus seminal. Progr Urol 1993; 3: 1028-1033.
7. Smith FL, Ritchie EL, Maizels M, Zaontz MR, Hsueh W, Kaplan WE, Firlit CF.
Surgery for duplex kidneys with ectopic ureters: ipsilateral ureteroureterectomy versus polar nephrectomy. J Urol 1989; 142: 532-534.
8. Sumfest JM, Burns MW, Mitchell ME.
Pseudoureterocele: potential for misdiagnosis of an ectopic ureter as a ureterocele. BrJ Urol 1995; 75: 401-405.
10. INTERSEX
(ABNORMAL SEXUAL DIFFERENTIATION)
10.1 BACKGROUND
Disorders of sexual differentiation most often manifest themselves by the abnormal appearance of the external genitalia as the baby is born, casting doubt on the baby's gender and a lot of anxiety on his/her family. The anatomy and physiology of differentiation of the internal and external sexual organs in the foetus is regulated by factors at three different levels.
Table 14: Factors involved in normal sexual differentiation
Y = Testis determining gene
Chromosomal factors
X = 5cc-reductase gene, androgen receptor gene
Gonadal factors
19 = АМН - receptor gene Testis = TST, АМН
Biochemical (endocrine) factors
TST -DHTST
Wolffian duct External genitalia
Andrenal androgens
TST = testosterone; АМН = anti-Mullerian hormone; DHTST = dihydrotestosterone
With very few exceptions, a normal 46XX or 46XY karyotype leads to normal sex determination and gonadal differentiation. The presence of the so-called SRY gene (sex-determining region Y) on the short arm of the Y chromosome leads to differentiation of the foetal gonad into testis, while the absence of one X chromosome (i.e. 45X0) will lead to early loss of gonadal germ cells and ovarian fibrous degeneration.
The normally functioning foetal testis induces further somatic male differentiation by means of: a) anti-Mullerian hormone (АМН), secreted by the Sertoli cells, which is responsible for Mullerian duct regression; and b) testosterone (TST) secreted by the Leydig cells, which is responsible for the maintenance and male differentiation of the Wolffian ducts and virilization of the urogenital sinus and external genitalia (genital tubercle), after its intracellular conversion to dihydrotestosterone (DHTST) by 5a reductase.
Table 15: Somatic male differentiation via testicular hormones
Testis
Leydig cells
TST
5a
DHTST
Genital tubercle
Male external genitalia
Both АМН and DHTST need the presence of their respective cellular receptors in order to exercise their function. Disorders of АМН or TST (DHTST) biosynthesis or action may lead to intersex states. The fact that the androgen-receptor gene lies on the X chromosome explains the virilization of the female foetus in the AGS.
Table 16: Effects of inadequate biosynthesis or action of АМН, TST or DHTST
| AMH^ | Retention of Mullerian structures |
| TSU | Inadequate differentiation of Wolffian duct structures (i.e. epididymis, vas deferens, seminal vesicles) |
| DHTSTl | Inadequate differentiation of genital tubercle -> inadequate masculinization of external genitalia (severe hypospadias, microphallus) |
АМН = anti-Mullerian hormone, TST = testosterone, DHTST = dihydrotestosterone
- 1. Phimosis
- 1.1 Background
- 1.2 Diagnosis
- 1.3 Treatment
- 1.4 References
- 2.2 Diagnosis
- 2.3 Treatment
- V Therapy
- 3.3 Treatment
- 3.3.1 Complications
- Tube-onlay
- 15. Duckett jw.
- 4.2 Classification
- 4.2.1 Enuresis
- 4.2.2 Urinary incontinence
- 4.3 Diagnosis
- 4.4 Treatment
- 4.4.1 Nocturnal enuresis (mono-symptomatic)
- 4.4.2 Diurnal enuresis (in children with attention disorders)
- 4.4.3 Urinary incontinence
- 4.4 References
- 14. Madersbacher h, Schultz-Lampel d.
- 5.2.2 Voiding cystourethrography (vcug)
- 5.2.3 Diuresis renography
- 5.2.4 Static renal scintigraphy
- 5.2.5 Intravenous urogram (ivu)
- 5.2.6 Whitaker's test
- 5.3 Treatment
- 5.3.2 Megaureter
- 5.3.3 Ureterocele
- 5.3.4 Retrocaval ureter
- 5.3.5 Bilateral hydronephrosis
- 5.4 References
- 6.3 Treatment
- 6.3.1 Asymptomatic bacteriuria
- 6.3.2 Acute uti without pyelonephritis
- 6.3.3 Pyelonephritis
- 6.3.4 Complicated uti
- 6.3.5 Antibiotic prophylaxis
- 6.4 References
- 7.7.1 Secondary reflux
- 7.2 Classification
- 7.3 Diagnosis
- 7.3.1 Secondary reflux
- 7.4 Treatment
- 7.4.1 Conservative therapy
- 7.4.2 Surgical therapy
- 7.4.3 Endoscopic therapy
- 7.4.4 Open surgery
- 7.4.5 Follow-up
- 7.5 References
- 32. McGladdery sl, Aparicio s, Verrier Jones k, Roberts r, Sacks sh.
- 8.2 Diagnosis
- 8.3 Treatment
- 8.3.1 Conservative treatment
- 8.3.2 Metaphylaxis of paediatric nephrolithiasis
- 8.4 References
- 1. Brandle e, Hautmann r.
- 2. Brandle e, Hautmann r.
- 6. Diamond da, Rickwood am, Lee ph, Johnston jh.
- 19. Kovacevic l, Kovacevic s, Smoljanic z, Peco-Antic a, Kostic n, Gajic m, Kovacevic n, Jovanovic o.
- 20. Kroovand rl.
- 24. Minevich e, Rousseau mb, Wacksman j, Lewis ag, Sheldon ca.
- 9.2 Classification
- 9.2.1 Ectopic ureterocele
- 9.2.2 Orthotopic ureterocele
- 9.2.3 Caecoureterocele
- 9.3 Diagnosis
- 9.3.1 Ureterocele
- 9.3.2 Ectopic ureter
- 9.4 Treatment
- 9.4.1 Ureterocele
- 9.4.2 Ectopic ureter
- 10.2 Classification
- VI. Miscellaneous (? Dysgenetic testes ? teratogenic factors)
- 10.3.2 Late diagnosis and management
- 10.4 Treatment
- 10.4.1 Genitoplasty
- 10.4.2 Indications for the removal of gonads
- 10.5 References
- 11.1.4 Video-urodynamic evaluation
- 11.1.5 Urethral pressure profile (sphincterometry)
- 11.1.6 Electromyography (emg) of the external sphincter
- 11.2 References
- 22. Starr nt.
- 23. Wan j, Greenfield s.
- 26. Zermann dh, Lindner h, Huschke t, Schubert j.
- 12 Abbreviations used in the text