Campbell-Walsh Urology, 11th Edition

PART XV

Pediatric Urology

SECTION C

Upper Urinary Tract Conditions

134

Ectopic Ureter, Ureterocele, and Ureteral Anomalies

Craig A. Peters; Cathy Mendelsohn

Questions

1. All of the following are possible drainage sites for an ectopic ureter in a female except the:
2. fallopian tube.
3. uterus.
4. ovary.
5. vagina.
6. urethra.
7. Inadequate interaction between the ureteral bud and metanephric blastema will most likely lead to which of the following conditions?
8. Dysplasia
9. Hydronephrosis
10. Reflux
11. Ureteral ectopia
12. Multicystic dysplasia
13. The relationship between the upper and lower pole orifices in a complete ureteral duplication is best described by the upper pole:
14. orifice being cephalad and lateral to the lower orifice.
15. ureter joining the lower pole ureter just before entry into the bladder.
16. orifice being caudal and medial to the lower pole orifice.
17. orifice and lower pole orifice being located transversely side by side.
18. ureter joining the bladder neck caudal to the lower pole orifice.
19. The most common site of drainage of an ectopic ureter in a male is:
20. vas deferens.
21. anterior urethra.
22. seminal vesicle.
23. posterior urethra.
24. ampulla of the vas.
25. All of the following contribute to vesicoureteral reflux EXCEPT:
26. lateral ureteral insertion.
27. lax bladder neck.
28. poorly developed trigone.
29. gaping ureteral orifice.
30. short intramural tunnel.
31. The voiding pattern most often seen in a girl with an ectopic ureter is:
32. urge incontinence.
33. stress incontinence.
34. continuous incontinence.
35. interrupted urinary stream.
36. overflow incontinence.
37. Which of the following findings is most likely present on an ultrasound in a patient with an ectopic ureter in a duplicated system?
38. Echogenic parenchyma of the lower pole of the kidney
39. Medially displaced lower pole of the kidney
40. Cystic structure in the bladder
41. Tortuous lower pole ureter
42. Cystic changes in the upper pole of the kidney
43. Ureteroceles can be associated with all of the following EXCEPT:
44. smoking during pregnancy.
45. vesicoureteral reflux.
46. white race.
47. female gender.
48. duplicated kidneys.
49. All of the following can be caused by a ureterocele. Which is the LEAST likely?
50. Bladder outlet obstruction
51. Upper pole obstruction
52. Lower pole reflux
53. Urinary incontinence
54. Contralateral reflux
55. A child known to have a ureterocele based on ultrasound imaging undergoes cystography, but no filling defect is noted. The most likely explanation is:
56. ureterocele eversion.
57. lower pole reflux.
58. ureterocele effacement.
59. ureterocele prolapse.
60. ureterocele disproportion.
61. A girl undergoes open resection of a large ectopic ureterocele. After removal of the catheter, she has high postvoid residuals demonstrated on a sonogram. Which complication is most likely responsible?
62. Persistent reflux
63. Prolapsing residual ureterocele tissue
64. Neurapraxia secondary to bladder retraction
65. Excessive buttressing of deficient detrusor at the bladder neck
66. Residual flap of the ureterocele in the urethra
67. What is the preferred method of endoscopic treatment of a ureterocele?
68. Resection of the roof of the ureterocele
69. Puncture of the ureterocele's urethral extension
70. Puncture of the roof of the ureterocele
71. Transverse incision at the base of the ureterocele
72. Resection of the base of the ureterocele only
73. An adult is evaluated as a possible kidney donor. An excretory urogram demonstrates a round contrast agent-filled area at the bladder base with a thin radiolucent rim around it. What is the most likely diagnosis?
74. Single-system kidney with a ureterocele
75. Marked opacification delay of the kidney
76. Radioopaque stone filing the ureterocele
77. Extension of a ureterocele to the bladder neck and urethra
78. Reflux
79. A white infant is found to have a smooth interlabial mass on the posterior aspect of the urethra. What would be the most appropriate initial management?
80. Chemotherapy
81. Puncture of the mass
82. Topical estrogen cream
83. Observation
84. Resection of the mass
85. An 11-year-old child presents with flank pain and hematuria. There is left hydronephrosis to the ureteropelvic junction. There is no ureteral dilation. Diuretic renography shows symmetric uptake in both kidneys and a very delayed washout time with a half-life of 50 minutes. At the time of surgery, a retrograde pyelogram shows a proximal ureteral filling defect. The best course of action is:
86. abandon the procedure and obtain computed tomography (CT) imaging with contrast.
87. perform ureteroscopic biopsy.
88. perform radical nephroureterectomy.
89. perform ureteroscopic excision of the presumed fibroepithelial polyp.
90. proceed with dismembered pyeloplasty and resect a fibroepithelial polyp.
91. An infant is seen with an intravesical ureterocele, no reflux, and an echogenic moderately dilated upper pole that has limited function. The washout curve of the upper pole moiety shows a t_1/2of 10 minutes. The most appropriate treatment option would be:
92. Observation with repeat ultrasound in 6 months
93. Ureterocele excision and common sheath reimplantation
94. Transureteral incision of the ureterocele
95. Prophylactic antibiotics, observation, and repeat ultrasound in 4 months
96. Upper pole partial nephrectomy
97. Which of the following statements regarding duplex kidneys is TRUE?
98. Duplex kidneys are the same size as single-system kidneys.
99. The upper pole moiety is the more likely of the two to have a ureteropelvic junction obstruction.
100. The duplex kidney arises as a consequence of two separate ureteric buds.
101. A duplex kidney results from two separate metanephric blastemal entities arising near the mesonephric duct.
102. The lower pole ureter is less likely to have vesicoureteral reflux.
103. In a child with a functioning nondilated upper pole segment associated with an ectopic ureter, the most efficient therapeutic option(s) (more than one answer may be correct) would be:
104. common sheath ureteral reimplantation.
105. upper to lower ureteropyelostomy.
106. upper to lower distal ureteroureterostomy.
107. upper pole partial nephrectomy.
108. upper pole ureteral reimplantation.
109. Initial endoscopic incision of a ureterocele offers the following advantages EXCEPT:
110. early relief of bladder outlet obstruction.
111. potential for definitive therapy.
112. possible improvement in trigonal deficiency.
113. potential for improved function of the affected renal segment.
114. decompression of a dilated upper pole ureter.
115. What is the most common form of ureteral triplication?
116. All three ureters joining to terminate in a single bladder orifice
117. Three ureters joining to form two ureteral orifices
118. Three ureters draining as three separate orifices
119. One of the three ureters terminating ectopically, the other two draining orthotopically
120. Two ureters draining into three orifices
121. Failure of atrophy of which vessel leads to the formation of a preureteral vena cava?
122. Posterior cardinal vein
123. Subcardinal vein
124. Supracardinal vein
125. Umbilical artery
126. Inferior vitelline vein
127. Which of the following types of ureterocele is associated with the lowest incidence of secondary procedures after transurethral decompression?
128. Ectopic ureterocele
129. Ureterocele in a female patient
130. Intravesical ureterocele
131. Ureterocele associated with a duplicated system
132. Cecoureterocele
133. After the perinatal period, what is the most common method of presentation of a ureterocele?
134. Incontinence
135. Abdominal mass
136. Failure to thrive
137. Stranguria
138. Urinary tract infection
139. A patient with a suspected ectopic ureter due to incontinence has no hydronephrosis on an ultrasonographic study and apparent single systems bilaterally. Which of the following tests is a sensitive method of determining if there is an ectopic ureter and associated renal moiety?
140. Diethylenetriaminepentaacetic acid (DTPA) renal scanning
141. Magnetic resonance imaging (MRI) of the abdomen and pelvis
142. Nuclear voiding cystourethrography
143. Positron emission tomography
144. Intravenous pyelography

Answers

1. c. Ovary. An ectopic ureter may drain into any of the structures related to the Wolffian duct and can rupture into the adjoining fallopian tube, uterus, upper vagina, or the urethra.
2. a. Dysplasia.Clinical and experimental observations combine to support the commonly held notion that dysplasia is the product of inadequate ureteric bud-to-blastema interaction. The other conditions may include such an abnormal interaction but are not specifically the result of that interaction.
3. c. Orifice being caudal and medial to the lower pole.The upper pole orifice is caudal and medial to the lower pole orifice because of its later incorporation and migration into the trigonal structure. The lower pole orifice is more cranial and lateral to the caudad medial upper pole orifice.
4. d.Posterior urethra. In the male, the posterior urethra is the most common site of the termination of the ectopic ureter. All other sites except the anterior urethra are possible sites of ectopic ureteral insertion.
5. b. Lax bladder neck.It is owing to the combined effects of the lateral ureteral orifice position, the ureter's shortened submucosal course, the poorly developed trigone, and the abnormal morphology of the ureteral orifice that primary vesicoureteral reflux develops.
6. c. Continuous incontinence. Continuous incontinence in a girl with an otherwise normal voiding pattern after toilet training is the classic symptom of an ectopic ureteral orifice.This may not be obvious in a girl who has not yet been toilet trained, but can occasionally be seen as slow steady dribbling of urine on direct observation.
7. d. Tortuous lower pole ureter.The most obvious imaging sign on ultrasonography of an ectopic ureter is a tortuous dilated ureter due to distal obstruction. This is not always present, but when seen should direct further attention to the distal ureter and bladder to also assess for the presence of a ureterocele, which would appear as a cystic structure in the bladder. The upper pole may be dysplastic, but cystic changes are uncommon. The lower pole is usually normal, but may be hydronephrotic, yet uncommonly echogenic. The lower pole is displaced laterally, not medially.
8. a. Smoking during pregnancy. Ureteroceles occur most frequently in females (4:1 ratio) and almost exclusively in whites.Approximately 10% are bilateral. Eighty percent of all ureteroceles arise from the upper poles of duplicated systems, and approximately 50% will have associated vesicoureteral reflux.
9. a. Bladder outlet obstruction.Ultrasonographic study may show a dilated ureter emanating from a hydronephrotic upper pole. This finding should signal the examiner to image the bladder to determine whether a ureterocele is present. If the lower pole is associated with reflux, or if the ureterocele has caused delayed emptying from the ipsilateral lower pole, this lower pole may likewise be hydronephrotic. Similarly, the ureterocele may impinge on the contralateral ureteral orifice or obstruct the bladder neck and cause hydronephrosis in the opposite kidney, but the latter is uncommon. The upper pole parenchyma drained by the ureterocele will exhibit varying degrees of thickness and echogenicity. Increased echogenicity correlates with dysplastic changes. Reflux may also be seen in the contralateral system if the ureterocele is large enough to distort the trigone and the opposite ureteral submucosal tunnel. In one series, 28% of patients had reflux in the contralateral unit.
10. c. Ureterocele effacement.Voiding cystourethrography can usually demonstrate the size and laterality of the ureterocele as well as the presence or absence of vesicoureteral reflux. If early filling views are not obtained, the ureterocele may efface and the filling defect may not be visible. In some cases the ureterocele will evert and appear as a diverticulum.
11. e. Residual flap of the ureterocele in the urethra.The authors of one study emphasized the need for passing a large catheter antegrade through the bladder neck to ascertain that all mucosal lips that might act as obstructing valves have been removed. In some large ureteroceles, if repair of the maldeveloped trigone is not adequate, a posterior defect at the bladder neck can act as an obstructive valve during voiding.
12. d. Transverse incision at the base of the ureterocele.Our preferred method of incising the ureterocele is similar to that described by Rich and colleagues in 1990, a transverse incision through the full thickness of the ureterocele wall using the cutting current. The incision should be made as distally on the ureterocele and as close to the bladder floor as possible to lessen the chance of postoperative reflux into the ureterocele.
13. a. Single-system kidney with a ureterocele.Excretory urography often demonstrates the characteristic cobra head (or spring-onion) deformity: an area of increased density similar to the head of a cobra with a halo or less dense shadow around it. The halo represents a filling defect, which is the ureterocele wall, and the oval density is contrast material excreted into the ureterocele from the functioning kidney.
14. b. Puncture of the mass.A ureterocele that extends through the bladder neck and the urethra and presents as a vaginal mass in girls is termed a prolapsing ureterocele. This mass can be distinguished from other interlabial masses (e.g., rhabdomyosarcoma, urethral prolapse, hydrometrocolpos, and periurethral cysts) by virtue of its appearance and location. The prolapsed ureterocele has a smooth round wall, as compared with the grapelike cluster that typifies rhabdomyosarcoma. The color may vary from pink to bright red to the necrotic shades of blue, purple, or brown. The ureterocele usually slides down the posterior wall of the urethra and, hence, the urethra can be demonstrated anterior to the mass and can be catheterized. The short-term goal is to decompress the ureterocele. The prolapsing ureterocele may be manually reduced back into the bladder; however, even if this is successful, the prolapse is likely to recur. Subsequent management is determined by further functional evaluation.
15. e. Proceed with dismembered pyeloplasty and resect a fibroepithelial polyp.This scenario most likely represents a fibroepithelial polyp of the ureteropelvic junction creating or associated with obstruction. The best approach is to proceed with the planned pyeloplasty and identify and resect the polyp thoroughly, followed by performing a conventional dismembered pyeloplasty. At times polyps may be multiple and complex, so this possibility should be looked for.
16. d. Prophylactic antibiotics, observation, and repeat ultrasound in 4 months.In the setting of no reflux and a draining upper pole associated with a ureterocele, the option of observation with prophylactic antibiotics has been seen to permit spontaneous resolution and no surgical intervention. Prophylactic antibiotics are recommended until resolution is demonstrated.
17. c. The duplex kidney arises as a consequence of two separate ureteral buds.Duplication anomalies arise as a consequence of two ureteral buds forming and inducing separate segments of the metanephric blastema. The duplex kidney may be completely normal, although it tends to be longer than normal, but if there is abnormal development, reflux and ureteropelvic junction obstruction occurs, most often in the lower pole, while ectopic ureteral insertion with or without a ureterocele is nearly always associated with the upper pole.
18. b and c.When the upper pole of a duplex system associated with an ectopic ureter demonstrates function, preservation is typically recommended. Two reasonable options exist for this, including proximal ureteropyelostomy which excises most of the usually dilated upper pole ureter, or distal ureteroureterostomy, which permits drainage without any manipulation of the perirenal tissues. There are no data to support one over the other, and both are reasonable options. There is no evidence to indicate that the so called yo-yo phenomenon of urine refluxing into more dilated segments of ureter is a clinically significant concern.
19. c. Possible improvement in trigonal deficiency.Transurethral puncture of a ureterocele offers all of the listed possible, but not certain, advantages, except to improve trigonal deficiency that can be associated with a severe ureterocele. This deficiency, which may lead to persisting reflux and bladder outlet obstruction, may require corrective surgery.
20. a. All three ureters joining to terminate in a single bladder orifice.In the classification used by most investigators, there are four varieties of triplicate ureter. In one variety, all three ureters unite and drain through a single orifice. This appears to be the most common form encountered although all others have been reported.
21. b. Subcardinal vein.If the subcardinal vein in the lumbar portion fails to atrophy and becomes the primary right-sided vein, the ureter is trapped dorsal to it.
22. c. Intravesical ureterocele.Several studies have indicated that intravesical ureteroceles fared better than extravesical ureteroceles with regard to decompression, preservation of upper pole function, newly created reflux, and need for secondary procedures. Nonetheless, the clinical scenario will be the most important indicator of the appropriateness of endoscopic incision for a ureterocele in a particular patient.
23. e. Urinary tract infection.Many ureteroceles are still diagnosed clinically. The most common presentation is that of an infant who has a urinary tract infection or urosepsis. In the early perinatal period, prenatal identification of hydronephrosis is currently the most common means of diagnosis.
24. b. Magnetic resonance imaging (MRI) of the abdomen and pelvis.Occasionally, the renal parenchyma associated with an ectopic ureter is difficult to locate on ultrasound and may be identified only by alternative imaging studies. In such cases in which an ectopic ureter is strongly suspected because of incontinence yet no definite evidence of the upper pole renal segment is found, CT or MRI has demonstrated the small, poorly functioning upper pole segment.

Chapter review

1. An ectopic ureter in a duplex system inevitably drains the upper pole.
2. In females, the ectopic ureter may enter from the bladder neck to the perineum or into the vagina, uterus, or rectum.
3. In males, the ectopic ureter always enters the urogenital system above the external sphincter and may enter wolffian duct structures, such as the vas deferens, seminal vesicles, and ejaculatory duct.
4. The orifice of a cecoureterocele is within the bladder; however, the ureterocele may extend beyond the bladder neck into the urethra.
5. The ectopic ureter inserts into the wolffian duct structure and not directly into a müllerian structure. Therefore, in the female, for an ectopic ureter to enter the vagina, cervix, or uterus, it requires a rupture into those structures.
6. The Weigert-Meyer rule states that an ectopic ureter or ureterocele is associated with the upper pole and is located caudal and medial to the lower pole ureteral orifice.
7. A young boy presenting with epididymitis might have an ectopic ureter.
8. A toilet-trained girl with verified continuous urinary leakage should be evaluated for an ectopic ureter.
9. A ureterocele or ectopic ureter associated with a patulous bladder neck may be complicated by incontinence. Cecoureteroceles are at particular risk for this.
10. On endoscopy, ureteroceles vary in their appearance with bladder filling.
11. An obstructed ureterocele may be treated endoscopically by multiple punctures or by a transverse incision. Both techniques have similar rates of success in decompression. In a transurethral incision of the ureterocele, the incision is made transversely as close to the bladder floor as possible. This may prevent subsequent reflux.
12. In patients with an ectopic ureter who present with sepsis and have massive ureteral dilatation, a temporary end ureterostomy may be the best management.
13. When the upper pole of the kidney is removed for an ectopic ureter, the residual stump is rarely problematic.
14. The separation of duplex ureters distally in the intravesical dissection should be discouraged because it may injure the common blood supply.
15. Conditions that routinely affect the single-system kidney generally affect the lower pole of a duplex system, such as ureteropelvic junction obstruction and vesical ureteral reflux. Conditions that affect the upper pole are more likely due to abnormal ureteral formation, such as ectopia and ureterocele.
16. On a voiding cystourethrogram, if early filling views are not obtained, a ureterocele may efface and the filling defect may not be visible.
17. Fibroepithelial polyps most commonly occur at the ureteropelvic junction but may occur anywhere in the ureter.
18. Correction of the circumcaval ureter requires ureteral division and relocation ventral to the vena cava.
19. When the upper pole of a duplex system associated with an ectopic ureter demonstrates function, preservation of renal tissue is typically recommended. Two reasonable options exist for this, including proximal ureteropyelostomy, which excises most of the usually dilated upper pole ureter, or distal ureteroureterostomy, which permits drainage without any manipulation of the perirenal tissues. No data exist to support one versus the other, and both are reasonable options.