O slideshow foi denunciado.
Seu SlideShare está sendo baixado. ×

Ectopic ureter & ureterocoele

Carregando em…3

Confira estes a seguir

1 de 69 Anúncio

Mais Conteúdo rRelacionado

Diapositivos para si (20)

Quem viu também gostou (20)


Semelhante a Ectopic ureter & ureterocoele (20)

Mais de GAURAV NAHAR (20)


Mais recentes (20)

Ectopic ureter & ureterocoele

  1. 1. ECTOPIC URETER & URETEROCELE Gaurav Nahar Deptt.of Urology MMHRC, Madurai
  2. 2. INTRODUCTION Ureteral duplication:  Most common congenital renal abnormality.  Found in 1% population & 10% of children diagnosed with UTIs.  Incomplete ureteral duplication- one common ureter enters bladder, rarely clinically significant.  Complete ureteral duplication- two ureters ipsilaterally enter the bladder.
  3. 3.  Propensity for VUR into lower pole and obstruction of upper pole.  Upper-pole ureter may be ectopic in its insertion into bladder or may end in a ureterocele.  Both conditions are more common in duplicated collecting systems but may also be seen in single systems.
  4. 4. Ectopic ureter & Ureterocele:  Distinct entities, but share many common features.  Same underlying developmental mechanisms.  A continuum of embryologic development.  Similar clinical presentations.  Approached in a similar manner.  Slight variation in management.
  5. 5. DEFINITION Ectopic ureter:  Any ureter, single or duplex, that does not enter trigonal area of bladder.  In a duplex system, inevitably upper pole ureter, because of its budding from mesonephric duct later than lower pole with later incorporation into the developing urogenital sinus.
  6. 6.  In females, entry anywhere from bladder neck to perineum and into vagina, uterus, and even rectum.  May be a/w dilated Gartner duct cyst (Wolffian duct remnant from which ureter buds)→ Rupture → vaginal communication→ incontinence.  In males, entry always above external sphincter or pelvic floor, usually into wolffian structures, including vas deferens, seminal vesicles, or ejaculatory duct.  No incontinence, but infection and pain of affected organs (testicles and epididymis).
  7. 7. Sites of Ectopic ureter
  8. 8. Retrograde injection study of boy with abdominal pain and a ureterocele associated with a hypoplastic right kidney. The intravesical ureterocele (UC) is being injected and demonstrates communication with the right seminal vesicle (arrowhead) and vas deferens (arrows), with the ureter (UR) leading to the dysplastic kidney. At surgical resection, the ureter and vas joined just above the seminal vesicles
  9. 9.  Single-system ectopic ureters & ureteroceles → apparently absent kidney on USG → small, poorly- functioning renal unit on CT Urogram.  Rare B/L single-system ectopic ureters may be a/w hypoplastic bladder & B/L renal abnormalities/dysplasia (apparent bladder agenesis).
  10. 10. Ureterocele:  Cystic dilatation of terminal intravesical ureter.  Intravesical ureterocele- entirely contained within the bladder; may prolapse into urethra during voiding.  Ectopic ureterocele- if any portion is permanently situated at bladder neck or urethra, regardless of the position of orifice(bladder, bladder neck or urethra).  Do not form entirely within urethra, nor do they attach to wolffian ductal structures.  Single or duplex system, and in duplex systems invariably affects upper pole.
  11. 11. Classification of Ureteroceles
  12. 12. A, Sphincterostenotic ectopic ureterocele. B, Cecoureterocele lumen extends distal to the orifice as a long tongue beneath the ureteral submucosa. The orifice communicates with the lumen of the bladder and is large and incompetent.
  13. 13. Churchill’s Functional classification system:  based on impact of ureterocele on upper urinary tract, including all renal units.  1. Only upper pole affected, 2. Entire ipsilateral kidney involved, & 3. Contralateral system also at risk d/t reflux or B.O.O.
  14. 14. Non-obstructive ureterocele with duplication or “Ureterocele disproportion”:  A/w a duplex kidney, but affected upper pole & ureter non-dilated and dysplastic→ not readily detected on most imaging.  Typical ureterocele seen in bladder, but ipsilateral kidney completely normal.
  15. 15. URETEROCELE DISPROPORTION Ureterocele disproportion demonstrated via retrograde pyelography. Note the disparity between the large ureterocele and the thin ureter and nondilated collecting system.
  16. 16. PATHOPHYSIOLOGY & EMBRYOLOGY Ureteral bud branch from mesonephric/Wolffian duct Extends into nephrogenic blastema(undifferentiated mesenchyma) Formation of the entire renal collecting system Distal to ureteric bud, mesonephric duct incorporates into UGS U.O. superolaterally moves to its normal position on trigone
  17. 17.  Distal segment of mesonephric duct is carried inferomedially→incorporated into bladder neck.  In male fetus, it also develops into seminal vesicle, vas deferens, and epididymis.  In females, it becomes Gartner duct, located between vagina and urethra.
  18. 18.  In ureteral duplication, two ureteric buds arise from mesonephric duct.  Lower - earlier insertion into UGS & superolateral location of orifice; poor trigonal support & short intramural tunnel → predisposed to VUR.  Upper- inserts later & low on trigone inferomedially → inserts ectopically at bladder neck, ejaculatory duct, seminal vesicle, or vas deferens in males & in Gartner duct in females.
  19. 19.  Ureteral ectopia without duplication result from delayed incorporation of distal ureter into developing bladder.  Ureterocele development- two theories: 1. failure of Chwalle membrane to break down at the distal ureter during development -results in obstruction and saccular dilation. 2. Aberrant signaling from expanding urogenital sinus results in dilation of distal ureter.
  20. 20. EPIDEMIOLOGY  Incidence of ureteral duplication- 1% (autopsy series).  Ureteroceles- 1 per 5000-12000 population; 10% bilateral, 60-80% ectopic, 80% a/w upper-pole ureter of a duplex system. Single system ureteroceles a/w cardiac & genital anomalies.  More common in females.  More common in whites.
  21. 21. PRENATAL IMAGING DETECTION  Majority of ectopic ureters & ureteroceles detected on prenatal USG, even if no specific diagnosis.  Duplex system prenatal Dx difficult, except in dilated upper moiety.  Upper pole “cyst” in a fetus – upper pole hydronephrosis until proven otherwise.  Bladder inspection mandatory to identify ureterocele in all cases- wait for bladder filling.  Character of upper pole parenchyma- thickness & echogenicity.
  22. 22.  A large ectopic ureter may impinge on the bladder appear as intravesical structure, “Pseudoureterocele.”  Careful evaluation of other renal units & bladder.  Ipsilateral lower pole or contralateral dilation suggests reflux or less commonly obstruction from ureterocele or dilated ectopic ureter.  B.O.O. by a ureterocele can manifest as hydronephrosis of all renal units.  Oligohydramnios, contralateral renal dysplasia- rare.  Prenatal intervention or early delivery- no benefit.
  23. 23. CLINICAL PRESENTATION INCIDENTAL:  Significant HN with an ectopic ureter or ureterocele.  During evaluation for cause of general abdominal pain.  Cases of presumed ovarian cysts may be markedly dilated ureters.
  24. 24. INFECTION:  UTI in first few months of life- MC presentation.  Generalised urosepsis d/t infected obstructed system.  Ongoing low-grade fever with periodic spikes.  Purulent discharge from the perineum  Bacterial epididymitis/orchitis- recurrent episodes.
  25. 25. INCONTINENCE:  Caused by an ectopic ureter in a girl, but never in boys.  Persistent low-volume dampness at all time; Child can’t remain dry for even 30-60 min.  Diagnosis difficult before toilet training.  Rare pts.- intermittent leakage through a Gartner duct membrane.  Untreated ureteroceles not a/w incontinence.
  26. 26.  PAIN: Uncommonly a/w acute infection, episodic obstruction of ectopic ureter or bladder pain caused by an obstructing ureterocele.  PROLAPSE: Ureterocele prolapse unusual; smooth, congested, mucosa covered interlabial masses protruding from urethra; non-circumferential, non- lobulated.  LATE PRESENTATION: Infection, abdominal/flank pain, Incontinence, Stone in ureterocele.
  27. 27. Ureterocele stone
  28. 28. DIFFERENTIAL DIAGNOSIS  Multicystic Renal Dysplasia  Pediatric Ureteropelvic Junction Obstruction  Pediatric Urinary Tract Infection  Urethral Anomalies and Urethral Prolapse  Severe Vesicoureteral Reflux  Obstructive megaureter
  29. 29. EVALUATION PHYSICAL EXAMINATION:  May facilitate diagnosis.  Prolapsed ureterocele, ectopic perineal ureteral orifice in a child with H/O continuous dampness  Dilated Gartner duct cyst- rare.  Palpable dilated upper pole of ectopic ureter or ureterocele in a relaxed infant.
  30. 30. Perineal ectopic ureteral orifice (bottom arrow) cannulated with an angiocatheter, situated between the urethral orifice (top arrow) and the vagina, just to the left of midline
  31. 31. A, Gartner duct cyst (bottom right arrow) in newborn with a left multicystic dysplastic kidney. B, Injection of the cyst communicated with the ureter and dysplastic kidney.
  32. 32. ULTRASOUND:  Typical findings-dilated upper pole with ureteral dilation or dilated single system.  Bladder images differentiate ureterocele from ectopic ureter- thin-walled cystic dilation within the bladder, not extending beyond its walls.
  33. 33. Cyst within cyst appearance
  34. 34. Ultrasound demonstrating dilated upper pole (UP) and lower pole (LP) associated with a ureterocele. The upper pole has evident renal parenchyma. The lower pole is dilated because of compression of the dilated upper pole ureter on the lower pole system, creating a partial obstruction.
  35. 35. Ultrasound image of dilated upper pole (UP) associated with a ureterocele, demonstrating limited renal parenchyma
  36. 36. Intravesical ureterocele Vs. Dilated Ectopic ureter
  37. 37. MRI:  Provides most detailed imaging.  Currently reseved for patients with distorted, complex anatomy.  Added advantage- functional information.
  38. 38. RENAL FUNCTION- NUCLEAR IMAGING:  Gold standard for renal functional assessment- DMSA.  Prime role- function of affected upper pole, also status of other renal moieties, if lower pole reflux of HN of any unit.  To assess drainage function in ureteroceles in which Observation is planned- Diuretic renal scan replaces DMSA- provides both funvtional & drainage information. IVU:  Less useful baseline study.  Functional assessment only qualitative.  Ureterocele- a "cobra head" or "spring onion" configuration at bladder level.
  39. 39. DMSA SCAN
  40. 40. Ureterocele
  41. 41. VOIDING CYSTOURETHROGRAM (VCUG):  Most definitive test for bladder,distal ureters &urethra.  Obligatory to define baseline situation before any intervention.  Omitted in emergency TUI for ureterocele producing BOO, urosepsis or B/L upper tract obstruction.  Duplicated collecting systems with lower-pole reflux & nonrefluxing upper pole, give appearance of a "drooping lily“.
  42. 42. VCUG of ureterocele Early filling phase
  43. 43. VCUG of duplex system ureterocele with reflux into lower moiety ureterocele Reflux into lower moiety Everting ureterocele
  44. 44. Voiding cystourethrogram image of a cecoureterocele where the ureterocele (black arrow) is attached to the urethra (white arrow) and the lumen extends into the urethra
  45. 45. Drooping Lily sign
  46. 46. REFLUX:  Reflux of ipsilateral lower pole – 50%.  Contralateral reflux in 25% of cases, and  Reflux into ureterocele in 10% of cases.  In an ectopic ureter, ipsilateral lower pole reflux is unlikely to resolve spontaneously.
  47. 47. ENDOSCOPIC EVALUATION:  Assess character of urethra, bladder neck and trigone relative to ureterocele or ectopic ureter.  Location of other ureteral orifices should be documented.  Orifice of affected ureter should be sought but may not be identified.  Urethra is examined carefully for orifice if not seen in bladder.  Appearance of ureterocele will vary with bladder filling; start with little filling and slowly increase bladder volume.  Lowest portion – best site for incision.  Retrograde contrast can confirm ureterocele disproportion & unusual connections with genital ducts.
  48. 48. CLINICAL MANAGEMENT  Before intervention, obtain maximum information about pts’ altered anatomy & physiology.  No criteria to decide how much upper pole renal function in worth preseving. MANAGEMENT GOALS: 1. Preservation of renal function; 2. Elimination of infection, obstruction, and reflux; 3. Maintenance of urinary continence; and 4. Minimizing surgical morbidity.
  49. 49. ACUTE DECOMPRESSION: Indications:  Ureterocele producing BOO or severe B/L upper tract obstruction.  Severe urosepsis.  Sepsis not responding to appropriate therapy. Methods:  For ureteroceles- Transurethral Incision (TUI).  For ectopic ureters- end ureterostomy near bladder.
  50. 50. DEFINITIVE SURGICAL OPTIONS:  For Ectopic ureter- common sheath reimplantation or ureteroureterostomy, either low/distal or high proximal near the renal pelvis.  For Ureterocele- TUI, ureterocele excision and common sheath reimplantation or ureteroureterostomy.
  51. 51. OBSERVATIONAL MANAGEMENT:  Non-operative management of ureteroceles meeting certain criteria, in carefully selected pt.& parental education- 1. no obstruction of ipsilateral lower pole or contralateral kidney, 2. limited reflux to lower pole (grade III or less), 3. no function of upper pole, or 4. no obstruction on diuretic renography.  Potential for later unpredictable acute presentation.
  52. 52. TOTAL RECONSTRUCTION:  Upper pole nephrectomy with ureterocele excision and reimplantation of lower pole ureter is definitive but extensive operation performed with two incisions.  Ideal candidate- older child with a massive ureterocele and no function of an upper pole with significant lower pole reflux.
  53. 53. UPPER POLE PARTIAL OR HEMI- NEPHRECTOMY:  Preferred treatment when no function in the upper pole.  Open surgery conventional laparoscopy, Robotic laparoscopy, Laparoendoscopic single-site surgery(LESS) nephrectomy.  Results in ureteroceles with/without lower pole reflux: resolution- 20%, New reflux- 15-50%, secondary surgery rate- 40-50%.
  54. 54. Upper pole Partial nephrectomy
  55. 55. Surgical management of the refluxing ureteral stump. A It is difficult to completely separate the distal 2 to 3 cm of upper pole ureter from lower pole ureter. The ectopic ureter is excised to this point. B The outer wall of ectopic ureter is excised to the bladder level. C A transfixing suture obliterates its lumen, with care being taken not to injure the orthotopic ureter.
  56. 56.  COMPLICATIONS OF UPPER POLAR NEPHRECTOMY: 1. Loss of lower pole function, 2. Postoperative upper pole urinoma 3. IVC laceration, 4. Duodenal perforation, 5. Total nephrectomy, 6. Peritoneal tears.
  57. 57. LOWER TRACT RECONSTRUCTION:  A definitive reconstruction at bladder is suitable for both ectopic ureter and ureterocele.  Advantage: relieving obstruction as well as correcting reflux.  Disadvantages: potential for injury to bladder neck and vagina, complexity of the procedure.  If clinically significant reflux persists after other procedures, lower tract reconstruction may be necessary.
  58. 58.  Results:Very good.  Persisting reflux- 5-10%, more common when ureteral tapering done.
  59. 59. PYELOURETEROSTOMY & URETEROURETEROSTOMY:  When upper pole of an ectopic ureter or ureterocele is preserved owing to function or surgeon preference.  Anastomosis between upper pole ureter & lower pole ureter in an end-to-side fashion. Proximal & distal approaches used.  Proximal anastomoses preferable to a distal ureteroureterostomy with a dilated upper pole, because the latter may result in more urinary stasis .
  60. 60. TRANSURETHRAL INCISION (TUI):  Transverse incision through full thickness of ureterocele wall using cutting current, as distally & close to the bladder floor as possible.  Bugbee electrode, angled-tip wire, Cold knife, resectoscope with Collins hot knife, Laser incision.  Deep incision to incise thick wall, see for urine-jet or inner urothelium.  Ectopic ureterocele:Longitudinal incision from intravesical into urethral portion, or two incisions.
  61. 61.  No catheter required.  Follow-up USG after 4-6 weeks to assess degree of decompression.  VCUG at 2-3 months to determine status of lower pole reflux.  Risk of reoperation high with extravesical ureteroceles & lower pole reflux (persisting or new).
  62. 62. TEMPORARY END URETEROSTOMY FOR ECTOPIC URETER:  Ectopic ureter in infant with sepsis or massive dilation.  Advantage- Acute decompression to manage sepsis and permit later assessment(in 4 mths or 6 mths age) of any function in affected renal unit before definitive management.
  63. 63. CLINICAL DECISION MAKING ECTOPIC URETERS: Duplex System Single System Preservation or Excision (based on function & Surgeon preference) Lower pole reflux No reflux Proximal or distal uretero- ureterostomy Reflux Common sheath reimplantation or lower pole reimplantation with distal upper to lower ureteroureterostomy Massively dilated ureter Temporary end ureterostomy
  65. 65. URETEROCELE:  TUI reasonable to offer before more complex reconstructions, specially young infants.  May make a subsequent surgical procedure less complex by decompressing a dilated upper pole ureter. Reimplantation may be much more effective and not require excisional tapering.  Older child with a massive upper pole, removal & definitive surgery perform at diagnosis.
  66. 66. THANK YOU !!!