This document discusses ectopic ureters and ureteroceles, which are abnormalities involving the insertion of the ureter into the urinary tract. It describes the normal embryonic development of the urinary system and presents theories on how abnormalities may arise. Clinical features like infection, incontinence, and pain are outlined. Evaluation methods like ultrasound, voiding cystourethrogram, and MRI are presented. The document concludes with a discussion of management goals and surgical techniques for these conditions.

1. Ectopic Ureter
&
Ureterocele
- Abhishek Pandey

2. Introduction
• Ectopic Ureter – Ureter not entering the trigone.
• Ureterocele – Cystic dilatation of distal ureter.
• Associated with single / duplex systems
• Distinct clinical entities.
• Share common developmental and clinical features.
• Ureteroceles do not form entirely in urethra or
wolffian structures.

3. UGS
Development

4. Embryology – Classic Theory
Origin of Trigone
Fusion of bilateral Common Nephric Ducts (CND)
[Mesodermal origin]

5. Revised Model of Ureteral Maturation
Apoptosis of CND after its insertion in UGS
Formation of Loop of distal ureter & CND
Apoptosis and incorporation of the loop into UGS
Separation of distal ureter & mesonephric duct

6. CND Remodelling
Whole mounts of urogenital tract at various stages of development

7. Weigert-Meyer Rule
• Duplex ureteral orifices → Ectopic ureter/Ureterocele
of upper-pole → caudal and medial to lower-pole

8. Mackie-Stephens Theory
• Final position of Ureteral orifice depends on origin
position of Ureteral bud on Wolffian duct

10. Embryological origin of Ureterocele
• Chwalla membrane – two-cell thick layer over
ureteral orifice → Apoptosis → patent connection.
Failure of rupture of Chwalla membrane
Distal Ureteral Expansion
[Ureterocele]

11. Ectopic Orifices in Mullerian Structures
• No direct insertion into
mullerian derivatives
• Gartner duct – Wolffian
duct remnant – associated
with ectopic ureter
• Antero-lateral vaginal wall

12. Location of ectopic ureteral orifices - Males

13. Location of ectopic ureteral orifices - Females

14. Ureterocele American Academy
of Pediatrics (AAP) Classification
• Intravesical –
– Entirely within bladder
– Above the bladder neck
• Ectopic / Extravesical – Some part
is permanently
– At bladder neck or
– In urethra

15. Stephens classification
• Stenotic – Narrow orifice; within bladder
• Sphincteric – Wide orifice; distal to internal sphincter
• Sphinctero-stenotic – Narrow orifice; distal to IUS
• Cecoureterocele – blind cavity of ureterocele tunnelling
into submucosa beyond the orifice.
• Blind – Ureterocele without corresponding renal tissue
• Non-obstructed –

16. Stenotic Ureterocele
Sphinctero-stenotic Ureterocele Cecoureterocele

17. Ureterocele Disproportion
• Nonobstructive
Ureterocele with
duplication
• Affected upper system
non-dilated & dysplastic

18. Churchill’s classification
• Based on renal tissue / units at risk of damage from
obstruction or high-grade reflux
• Grade 1: ureterocele segment only affected.
• Grade 2: both segments of one kidney affected.
• Grade 3: both kidneys affected.

19. Presentation
• Prenatal Detection – Majority – Prenatal USG
– Upper polar cyst
– Hydroureteronephrosis
– Ureterocele
Ureterocele
Lower polar HDN + HUN + Ureterocele

20. • Infection – in obstructed system.
– Febrile UTI & Urosepsis
– Low-grade fever episodes with negative Urine C/S
– Recurrent epididymitis with negative Urine C/S
• Incontinence –
– Girls with ectopic ureter opening in mullerian structures
– Continuous leakage
– Untreated ureterocele patients do not have incontinence

21. • Pain – acute infection or episodic obstruction
• Prolapse – Ureterocele prolapse
– Smooth
– Congested
– Mucosa-covered
– Intralabial mass

22. Evaluation
Gartner duct cyst with Ectopic ureter
Perineal Ectopic ureteral orifice

23. USG
Dilated upper pole
Dilated upper & lower (compression) pole
Ureterocele

24. MCU
Filling Phase Voiding Phase

25. Ureterocele prolapsing into urethra with lower pole reflux & “drooping lily” appearance of
the lower pole, displaced by the dilated upper pole.

26. Pseudoureterocele
• Dilated ectopic ureter
– Thicker wall than ureterocele.
– Lumen extends well beyond bladder wall.

27. IVP
Cobra-head – Ureterocele
Ectopic ureter opening in urethra

28. CECT
Ectopic Ureteral orifice in Prostatic urethra Volume rendered image

29. MRI
Right MCDK draining in obstructed
hemivagina, with dilated hemiuterus
Occult left duplex upper pole

30. Functional imaging
DMSA – Non-functional left upper pole
DMSA – Left upper polar function after
decompression of ureterocele

31. Cystourethroscopy
Ectopic bladder neck ureteral orifice
Ureterocele just above bladder neck

32. Management
• Preservation of Renal Function
• Elimination of Infection
• Relieving Obstruction
• Correction of Reflux
• Maintenance of Urinary Continence

33. THANK YOU