•
Two Unusual Conditions Simulating Ectopic Ureterocele
Pedia tric Radiology
Unilateral Hydrometrocolpos with Ipsilateral Renal Agenesis or Hypoplasia, and Ectopic Ureteral Opening into a Seminal Vesicle 1 Tudor J. Sutton, M.D., F.R.C.P.(C) and Noelle Gauthier, M.D. The cl inical and radiologic findings in two infants are presented, one with unilateral hydrometrocolpos and ipsilateral renal agenesis or hypoplasia and the other with ureteral duplication with one of the ureters opening into a cystically dilated seminal vesicle. The excretory urograms of both infants suggested ectopic ureterocele. Further radiologic investigation, including lateral views of the bladder, led to the correct preoperative diagnosis. Both conditions are relat ively rare , and their appearance simulating ectopic ureterocele is even more uncommon . INDEX TERMS: Genitourinary tract, abnormalities. Kidneys, abnormalities. Seminal vesicle • Ureters, abnormalities. Vagina , abnormalities Radiology 117:381-384, November 1975
•
•
DIAGNOSIS of ectopic ureterocele was entertained in two patients after the initial clinical and radiologic work-up. Further investigation led to the diagnosis of two unusual conditions.
A
At cystoscopy no right ureteral orifice was found, implying homolateral renal agenesis. However, rare cases of ectopic atretic nonduplicated ureter opening into a hydrometrocolpos and associated with a hypoplastic nonfunctioning kidney have been described (8).
CASE REPORTS CASE I: N. S., a 6-month-old girl, had been admitted to another hospital for urinary tract infection with pyuria. Excretory urography revealed a right-sided bladder filling defect without apparent function of the right kidney, suggesting the presence of a mass obstructing the ureteral orifice. In view of the complete ureteral duplication on the left, the mass was felt to represent an ectopic ureterocele (Fig. 1).
The infant was admitted to the Hopital des Enfants-Malades. A firm, cystic, rounded pelvic mass was palpated. A renal scintigram revealed no activity in the right hemiabdomen. The radiologic investigation was completed by retrograde cys tography, with introduc tion of air into the rectum delineating an extrinsic compression of the trigone due to a mass between the rectum and bladder . Genitography was performed by simultaneous filling of the vagina and the bladder with contrast material (Figs. 2 and 3) after inspection of the external genitalia which were normal. The vagina was displaced slightly to the left and was not responsible for the extrinsic compression of the bladder . Only a left fallopian tube was filled. These findings led us to suspect a duplication of the vagina and uterus with hydrocolpos of the right half , the absence of renal function on the right being related to renal agenesis or hypoplasia with an ectopic atretic ureter opening into the genital tract. At cystoscopy no right ureteral orifice was found . During vaginal endoscopy the septum separating the left hemivagina from the right was excised with release of clear fluid . This revealed a large right vaginal cavity with a uterine cervix. The patient recovered uneventfully. The urinary tract infection cleared and the follow-up cystogram was normal.
Comment: The importance of lateral views is stressed in the investigation of filling defects of the bladder, so that a three-dimensional evaluation is obtained. 1
Fig. 1. On the excretory urogram a large left kidney with a duplicated collecting system is noted . No renal function is seen on the right . A right-sided filling defect in the bladder is apparent.
From the Department of Radiology, Hopital des Enfants-Malades, Paris . France. Accepted for publication in May 1975.
381
dk
382
TUDOR
J.
SUTTON AND NOELLE GAUTHIER
November 1975
Fig. 2. A frontal view of the gen itogram with simultaneous filling of the bladder outl ining the vagina displaced slightly to the left. The uterine cavity is opacified and a single fallopian tube is beginning to fill . Fig . 3 . The lateral view with simultaneous opacification of the bladder and vagina demonstrates a smooth, rounded compression of the trigone independent of the opacified vag ina.
This last entity could not be entirely excluded since neither abdominal surgery nor postoperative genitography were performed. CASE II: V . R., an infant boy, presented at birth with an imperforate anus and hypopadias. At excretory urography numerous deformities of the lumbosacral spine were noted , as were the small size of the left kidney and a pelvicalyceal system which seemed to drain only the inferior pole. A filling defect was outlined in the left lower portion of the bladder. Complete ureteral duplication was present on the right (Fig. 4). This combination of findings suggested a diagnosis of left ectopic ureterocele. A suprapubic cystogram demonstrated reflux into the left lower pole ureter during filling of the bladder and into the seminal vesicle, vas deferens and left upper pole ureter during voiding (Figs. 5 and 6).
Comment: The reflux into the upper pole ureter via a dilated seminal vesicle is un ique. Catheterization or direct puncture of the seminal vesicle is usually required. DISCUSSION
It is only since the works of Ericsson (5) and Stephens (10) in respect to the ectopic ureterocele that the anatomy and embryology of this structure has been fully appreciated, although the condition had long been recognized. By definition, the ectopic ureterocele opens ei-
ther near the bladder neck or into the posterior urethra. The radio logic appearance has been widely discussed (3, 6, 11, 12), but on the whole, little emphasis has been placed upon the different conditions which may present with similar findings. This is not surprising when one considers that the radiologic diagnosis of ectopic ureterocele is not only dependent upon the recognition of the basic anatomic defect, .but must also take into consideration the variable presentation related to obstruction or reflux in the remainder of the urinary tract. It is therefore unlikely for bladder tumors or pelvic masses alone to mimic ectopic ureterocele when they only present with the vesical deformity. Inversely, suprarenal masses such as neuroblastoma or adrenal hemorrhage, by displacing the kidney inferiorly, may reproduce to some extent the renal component of ectopic ureterocele. The excretory urograms of the two cases are unique insofar as they demonstrate both the renal and bladder components so characteristic of ectopic ureterocele. CASE I was felt to present an ectopic ureterocele obstructing the lower pole ureter, explaining the absent renal function on the same side as the vesical filling defect. Although several reports mention the frequent coexistence of urinary tract malformations and hydrometrocolpos (4, 8), and particularly the association of
Vol. 117
Two CONDITIONS SIMULATING ECTOPIC URETEROCELE
renal agenesis with unilateral hydrometrocolpos of an uterus didelphys (2, 7, 13), the radiologic similarity to ectopic ureterocele is not discussed since these pelvic masses are usually quite large, causing marked displacement of the bladder and ureters. When smaller, they may understandably mimic ectopic ureterocele. Only additional lateral views during excretory urography or cystography may eventually define the pathologic process involved, by demonstrating the extrinsic nature of the pelvic mass in relationship to the bladder. This criterion, however, is not of absolute value since dilatation of the extravesical portion of the ureter may be severe enough in ectopic ureterocele to produce extrinsic compression of the bladder (3) . In boys the frequency of ureteral ectopy is one-fourth of that in girls and , when this occurs, the ureter opens into the seminal vesicle in 28 % (5). The left side is involved in two-thirds of the patients (9) . The kidney is usually small and nonfunctioning (1). Exceptionally, ureteral duplication is observed and then the upper pole ureter is the ectopic one. The cystic dilatation of the seminal vesicle remains unexplained. In our patient, the ureteral duplication with apparent function of only the lower pole , the ureter of the nonfunctioning upper pole draining into a cystically dilated seminal ves icle compressing the bladder, explain how this abnormality could be mistaken for an ectopic ureterocele. Lateral views associated with the anteroposterior projections could have demonstrated the extrinsic nature of the mass during the excretory urography. The correct diagnosis was not made until a voiding suprapubic cystogram outlined reflux through the seminal vesicle into the upper pole ureter. ACKNOWLEDGMENTS: The authors express the ir appreciation to Pro J. Lefebvre for his suggestions, and to Elisabeth Richard for her help in preparing the manuscript.
REFERENCES 1. Allansmith R: Ectopic ureter terminating in seminal vesicle; unilateral polycystic kidney: report of a case and rev iew of the literature. J UroI80:425-435. Dec 1958 2. Bennet J, Bronstein H, Faure C: Agenesie renale urnlaterale associee a une gynatresie hornolaterale sur uterus double . Ann RadioI7:381 -387, May-Jun 1964 3. Berdon WE, Baker DH, Becker JA, et al : Ectopic ureterocele. Radiol Clin N Am 6:205-214, Aug 1968 4. Cook GT, Marshall VF : Hydrocolpos causing urinary obstruction. J Urol 92: 127 -132, Aug 1964 5. Ericsson NO: Ectopic ureterocele in infants and children: clinical study. Acta Ch ir Scand Suppl 197:1-92, 1954 6. Friedland GW , Cunningham J: The elusive ectopic ureteroceles. Am J Roentgenol 116:792·-811 , Dec 1972 7. Lefebvre J, Fortier-Beaulieu M, Capdeville R: Les hydrohematocolpos du nouveau-ne avec malformations gen itales et urinaires assoclaas , Ann Radiol 9:23-30, Jan-Feb 1966
383
Pediatric Radiology
Fig. 4. Excretory urogram shows a large right kidney w ith a duplicated collecting system. On the 'aft the kidney is small with opacification of calyces which seem to drain only the lower pole. A filling defect is apparent in the left lower quadrant of the bladder.
8. Reed MH, Griscom NT: Hydrometrocolpos in infancy. Am J RoentgenoI118:1-13, May 1973 9. Schnitzer B: Ectopic ureteral opening into seminal vesicle: a report of four cases. J Urol 93 :576-581, May 1965 10. Stephens FD: Ureterocele in infants and children. Aust NZ J Surg 27:288-295, May 1958 11. Thornbury JR: The roentgen diagnosis of ureterocele in children . Am J Roentgenol 90: 15-25, Jul 1963 12 . Vinstein AL , Franken EA Jr: Unilateral hematocolpos associated with agenesis of the kidney . Radiology 102:625- 627 , Mar 1972 13. Williams DI, Woodard JR: Problems in the management of ectopic ureteroceles. J Urol 92:635-652, Dec 1964
Departement de Radiologie Service de Radiologie Pediatrique Hopital des Enfants-Malades 149, rue de Sevres 75730 Paris , France
(Figures 5 and 6 are on page 384)
384
TUDOR
J.
SUTTON AND NOELLE GAUTHIER
November 1975
Fig. 5 . The frontal projection of the suprapubic cystogram demonstrates the opacified bladder and vesicoureteral reflux into the lower pole ureter. Brief micturition had occurred with filling of a dilated left seminal vesicle and vas deferens. Fig. 6 . The lateral view of the suprapubic cystogram during micturition demonstrates both vesicoureteral reflux into the lower pole ureter and reflux from the posterior urethra into the dilated seminal vesicle. vas deferens and left upper pole ureter.
Two Unusual Conditions Simulating Ectopic Ureterocele
Pedia tric Radiology
Unilateral Hydrometrocolpos with Ipsilateral Renal Agenesis or Hypoplasia, and Ectopic Ureteral Opening into a Seminal Vesicle 1 Tudor J. Sutton, M.D., F.R.C.P.(C) and Noelle Gauthier, M.D. The cl inical and radiologic findings in two infants are presented, one with unilateral hydrometrocolpos and ipsilateral renal agenesis or hypoplasia and the other with ureteral duplication with one of the ureters opening into a cystically dilated seminal vesicle. The excretory urograms of both infants suggested ectopic ureterocele. Further radiologic investigation, including lateral views of the bladder, led to the correct preoperative diagnosis. Both conditions are relat ively rare , and their appearance simulating ectopic ureterocele is even more uncommon . INDEX TERMS: Genitourinary tract, abnormalities. Kidneys, abnormalities. Seminal vesicle • Ureters, abnormalities. Vagina , abnormalities Radiology 117:381-384, November 1975
•
•
DIAGNOSIS of ectopic ureterocele was entertained in two patients after the initial clinical and radiologic work-up. Further investigation led to the diagnosis of two unusual conditions.
A
At cystoscopy no right ureteral orifice was found, implying homolateral renal agenesis. However, rare cases of ectopic atretic nonduplicated ureter opening into a hydrometrocolpos and associated with a hypoplastic nonfunctioning kidney have been described (8).
CASE REPORTS CASE I: N. S., a 6-month-old girl, had been admitted to another hospital for urinary tract infection with pyuria. Excretory urography revealed a right-sided bladder filling defect without apparent function of the right kidney, suggesting the presence of a mass obstructing the ureteral orifice. In view of the complete ureteral duplication on the left, the mass was felt to represent an ectopic ureterocele (Fig. 1).
The infant was admitted to the Hopital des Enfants-Malades. A firm, cystic, rounded pelvic mass was palpated. A renal scintigram revealed no activity in the right hemiabdomen. The radiologic investigation was completed by retrograde cys tography, with introduc tion of air into the rectum delineating an extrinsic compression of the trigone due to a mass between the rectum and bladder . Genitography was performed by simultaneous filling of the vagina and the bladder with contrast material (Figs. 2 and 3) after inspection of the external genitalia which were normal. The vagina was displaced slightly to the left and was not responsible for the extrinsic compression of the bladder . Only a left fallopian tube was filled. These findings led us to suspect a duplication of the vagina and uterus with hydrocolpos of the right half , the absence of renal function on the right being related to renal agenesis or hypoplasia with an ectopic atretic ureter opening into the genital tract. At cystoscopy no right ureteral orifice was found . During vaginal endoscopy the septum separating the left hemivagina from the right was excised with release of clear fluid . This revealed a large right vaginal cavity with a uterine cervix. The patient recovered uneventfully. The urinary tract infection cleared and the follow-up cystogram was normal.
Comment: The importance of lateral views is stressed in the investigation of filling defects of the bladder, so that a three-dimensional evaluation is obtained. 1
Fig. 1. On the excretory urogram a large left kidney with a duplicated collecting system is noted . No renal function is seen on the right . A right-sided filling defect in the bladder is apparent.
From the Department of Radiology, Hopital des Enfants-Malades, Paris . France. Accepted for publication in May 1975.
381
dk
382
TUDOR
J.
SUTTON AND NOELLE GAUTHIER
November 1975
Fig. 2. A frontal view of the gen itogram with simultaneous filling of the bladder outl ining the vagina displaced slightly to the left. The uterine cavity is opacified and a single fallopian tube is beginning to fill . Fig . 3 . The lateral view with simultaneous opacification of the bladder and vagina demonstrates a smooth, rounded compression of the trigone independent of the opacified vag ina.
This last entity could not be entirely excluded since neither abdominal surgery nor postoperative genitography were performed. CASE II: V . R., an infant boy, presented at birth with an imperforate anus and hypopadias. At excretory urography numerous deformities of the lumbosacral spine were noted , as were the small size of the left kidney and a pelvicalyceal system which seemed to drain only the inferior pole. A filling defect was outlined in the left lower portion of the bladder. Complete ureteral duplication was present on the right (Fig. 4). This combination of findings suggested a diagnosis of left ectopic ureterocele. A suprapubic cystogram demonstrated reflux into the left lower pole ureter during filling of the bladder and into the seminal vesicle, vas deferens and left upper pole ureter during voiding (Figs. 5 and 6).
Comment: The reflux into the upper pole ureter via a dilated seminal vesicle is un ique. Catheterization or direct puncture of the seminal vesicle is usually required. DISCUSSION
It is only since the works of Ericsson (5) and Stephens (10) in respect to the ectopic ureterocele that the anatomy and embryology of this structure has been fully appreciated, although the condition had long been recognized. By definition, the ectopic ureterocele opens ei-
ther near the bladder neck or into the posterior urethra. The radio logic appearance has been widely discussed (3, 6, 11, 12), but on the whole, little emphasis has been placed upon the different conditions which may present with similar findings. This is not surprising when one considers that the radiologic diagnosis of ectopic ureterocele is not only dependent upon the recognition of the basic anatomic defect, .but must also take into consideration the variable presentation related to obstruction or reflux in the remainder of the urinary tract. It is therefore unlikely for bladder tumors or pelvic masses alone to mimic ectopic ureterocele when they only present with the vesical deformity. Inversely, suprarenal masses such as neuroblastoma or adrenal hemorrhage, by displacing the kidney inferiorly, may reproduce to some extent the renal component of ectopic ureterocele. The excretory urograms of the two cases are unique insofar as they demonstrate both the renal and bladder components so characteristic of ectopic ureterocele. CASE I was felt to present an ectopic ureterocele obstructing the lower pole ureter, explaining the absent renal function on the same side as the vesical filling defect. Although several reports mention the frequent coexistence of urinary tract malformations and hydrometrocolpos (4, 8), and particularly the association of
Vol. 117
Two CONDITIONS SIMULATING ECTOPIC URETEROCELE
renal agenesis with unilateral hydrometrocolpos of an uterus didelphys (2, 7, 13), the radiologic similarity to ectopic ureterocele is not discussed since these pelvic masses are usually quite large, causing marked displacement of the bladder and ureters. When smaller, they may understandably mimic ectopic ureterocele. Only additional lateral views during excretory urography or cystography may eventually define the pathologic process involved, by demonstrating the extrinsic nature of the pelvic mass in relationship to the bladder. This criterion, however, is not of absolute value since dilatation of the extravesical portion of the ureter may be severe enough in ectopic ureterocele to produce extrinsic compression of the bladder (3) . In boys the frequency of ureteral ectopy is one-fourth of that in girls and , when this occurs, the ureter opens into the seminal vesicle in 28 % (5). The left side is involved in two-thirds of the patients (9) . The kidney is usually small and nonfunctioning (1). Exceptionally, ureteral duplication is observed and then the upper pole ureter is the ectopic one. The cystic dilatation of the seminal vesicle remains unexplained. In our patient, the ureteral duplication with apparent function of only the lower pole , the ureter of the nonfunctioning upper pole draining into a cystically dilated seminal ves icle compressing the bladder, explain how this abnormality could be mistaken for an ectopic ureterocele. Lateral views associated with the anteroposterior projections could have demonstrated the extrinsic nature of the mass during the excretory urography. The correct diagnosis was not made until a voiding suprapubic cystogram outlined reflux through the seminal vesicle into the upper pole ureter. ACKNOWLEDGMENTS: The authors express the ir appreciation to Pro J. Lefebvre for his suggestions, and to Elisabeth Richard for her help in preparing the manuscript.
REFERENCES 1. Allansmith R: Ectopic ureter terminating in seminal vesicle; unilateral polycystic kidney: report of a case and rev iew of the literature. J UroI80:425-435. Dec 1958 2. Bennet J, Bronstein H, Faure C: Agenesie renale urnlaterale associee a une gynatresie hornolaterale sur uterus double . Ann RadioI7:381 -387, May-Jun 1964 3. Berdon WE, Baker DH, Becker JA, et al : Ectopic ureterocele. Radiol Clin N Am 6:205-214, Aug 1968 4. Cook GT, Marshall VF : Hydrocolpos causing urinary obstruction. J Urol 92: 127 -132, Aug 1964 5. Ericsson NO: Ectopic ureterocele in infants and children: clinical study. Acta Ch ir Scand Suppl 197:1-92, 1954 6. Friedland GW , Cunningham J: The elusive ectopic ureteroceles. Am J Roentgenol 116:792·-811 , Dec 1972 7. Lefebvre J, Fortier-Beaulieu M, Capdeville R: Les hydrohematocolpos du nouveau-ne avec malformations gen itales et urinaires assoclaas , Ann Radiol 9:23-30, Jan-Feb 1966
383
Pediatric Radiology
Fig. 4. Excretory urogram shows a large right kidney w ith a duplicated collecting system. On the 'aft the kidney is small with opacification of calyces which seem to drain only the lower pole. A filling defect is apparent in the left lower quadrant of the bladder.
8. Reed MH, Griscom NT: Hydrometrocolpos in infancy. Am J RoentgenoI118:1-13, May 1973 9. Schnitzer B: Ectopic ureteral opening into seminal vesicle: a report of four cases. J Urol 93 :576-581, May 1965 10. Stephens FD: Ureterocele in infants and children. Aust NZ J Surg 27:288-295, May 1958 11. Thornbury JR: The roentgen diagnosis of ureterocele in children . Am J Roentgenol 90: 15-25, Jul 1963 12 . Vinstein AL , Franken EA Jr: Unilateral hematocolpos associated with agenesis of the kidney . Radiology 102:625- 627 , Mar 1972 13. Williams DI, Woodard JR: Problems in the management of ectopic ureteroceles. J Urol 92:635-652, Dec 1964
Departement de Radiologie Service de Radiologie Pediatrique Hopital des Enfants-Malades 149, rue de Sevres 75730 Paris , France
(Figures 5 and 6 are on page 384)
384
TUDOR
J.
SUTTON AND NOELLE GAUTHIER
November 1975
Fig. 5 . The frontal projection of the suprapubic cystogram demonstrates the opacified bladder and vesicoureteral reflux into the lower pole ureter. Brief micturition had occurred with filling of a dilated left seminal vesicle and vas deferens. Fig. 6 . The lateral view of the suprapubic cystogram during micturition demonstrates both vesicoureteral reflux into the lower pole ureter and reflux from the posterior urethra into the dilated seminal vesicle. vas deferens and left upper pole ureter.
