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Urinary Tract Anomalies Associated with Unicornuate Uterus
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VOl. 155,&47-&48. March 1996 Printed in U S A .
JOURNAL OF UROLOGY
Copyright 0 1996 by AMERICAN U R O ~ I CA~SOCUTION, AL INC.
URINARY TRACT ANOMALIES ASSOCIATED WITH UNICORNUATE UTERUS LUIGI FEDELE,* STEFAN0 BIANCHI, BENEDETTA AGNOLI, LUCA TOZZI
AND
MARIO VIGNALI
Milano,Milano, Italy
From the Department of Obstetrics and Gynecotogy, University of
ABSTRACT
Purpose: We investigated the association between the various subclasses of the unicornuate uterus and urinary tract anomalies. Materials and Methods: We studied the urinary tract of 37 patienta with a unicornuate uterus by urography andor ultrasonography. Unicornuate uterus was diagnosed at laparoecopy or laparotomy. Results: A total of 15 patients (40.5%) had urinary tract anomalies, includingectopic kidney in 4, renal agenesis in 6, double renal pelvis in 2, horseshoe kidneys in 2 and unilateral medullary sponge kidney in 1.No differences were observed in the frequency of urinary anomalies among the various subclasses of unicornuate uterus. Conclusions: Our results indicate the need to suspect a urinary tract anomaly,particularly of the kidney, in all women with a unicornuate U ~ N S . KEYW o w : uterus, kidney, urinary tract, abnormalities
The association between urinary and genital malformations has long been recognized. Marshall and Beisel demonstrated that a single mesonephric duct abnormality can result in renal and uterine anomalies.1 Large series of some genital malformations, such as uterovaginal atresia, have been reported that clearly delineate the types of associated urinary tract anomalies2 but to date only small series of unicornuate uterus have been reported. The anatomical presentations of unicornuate uterus are particularly heterogeneous. Therefore, it seemed appropriate to investigate the association between the various subclasses of this rare malformation and urinary tract anomalies. MATERIALS AND METHODS
Between 1975 and 1994,49 patients 14 to 40 years old were diagnosed with a unicornuate uterus at laparoseopy or laparotomy at our department. Five patients underwent emergency surgery for an acute abdomen due to a ruptured rudimentary horn containing an ectopic pregnancy (1)and hemoperitoneum caused by hematometra of the rudimentary FIG.1. Buttram and Gibbons elaeeifieationof unimmuate utehorn (4). The remaining 44 patients were referred to us for Reprinted with infertility (35) or pelvic pain (9).The malformationwas classified according to the Buttram and Gibbons system (fig. 1hS Association between unicornuate uterus subclasses and uml&cal The urinary tract was studied in 37 patients and 12 did not anomalies undergo urological investigationfor organizational, personal or medical reasons. No patient reported symptoms relating to the urinary tract. Until 1980 patients were studied only by _ _ umgraphy. Af'ter that date ultrasonography of the urinary ~ 2 a 2. R e d agenesis tract was performed on all patients and urography was done Ectopic kidney 1 1 2* 1 1 only if urological anomalies were suspected. Horseshoe kidney ~
-
Double renal pelvie Unilat. medullary sponge kidney
RESULTS
1 __ ~
1
-
1
-
__
Total No. (46) 4 (40) s (36 6 (64) The table summarizes the frequency of associations beNo renal anomalies were noted in 2 patients with a subelass Ala unicomuate tween the unicornuate uterus subclasses and the types of UtelUS. urinary anomalies in our series. Overall, 15 patients (40.5%) * Both anomalies oeeurred in 1 patient. had urinary tract anomalies.The most fresuent anomaly was renal agenesis contralateral to the unicornuate uterus in 6 cases. In 1 woman renal agenesis was associated with an was observed in the tkequency of urinary tract anomalies ectopic kidney ipsilateral to the hemi-uterus. No difference among the various subclasses of unicornuate uterus. DISCUSSION
F p t e d for publication July 28,1995. Requests for reprints:Department of Obstetrics and Gynecology. University of Milano, via Commenda 20122, Milano, Italy.
To our knowledge our series is the largest reported to date and our results indicate that unicornuate uterus is associ-
a47
a48
URINARY TRACT ANOMALIES ASSOCIATED WITH UNICORNLJATE UTERUS
Therefore, it appears obvious that a single mesonephric duct abnormality can result in renal and uterine anomalies. Analysis of our series did not demonstrate a direct relatiomhip between damage to the miillerian and mesonephric ducts. In fact, the frequency and severity of urological anomalies associated with subclass B, in which there is complete agenesis of a miillerian duct, were no greater than those in the subclasses with partial development of this duct. The latter findingagrees with the study by Wmlf and Allen of 11 women with unicomuate uterus in which unilateral renal agenesis was observed in all 3 cases of subclass A h 5 Heinonen found 9 cases of urinary tract anomaly in 15 women with a unicomuate uterus investigated by urography.6 More recently Moutos et al reported an association between urological anomalies and unicomuate uterus in 7 of the 19 patients studied.' From the clinical viewpoint our results indicate the need to suspect a urinary anomaly, particularly of the kidney, in all women in whom a unicomuate uterus has been diagnosed. Therefore, a preliminary study of the urinary system is mandatory before performing any invasive procedure, diagnostic or therapeutic, that could result in serious damage to renal function, especially in the presence of a pelvic kidney. CONCLUSIONS
Urinary tract anomalies are frequently found in women with a unimmuate uterus. The most frequent anomaly was renal agenesis contralateral to the hemi-uterus and edopic kidney. The frequency of urinary anomalies was similar in the various subclasses of unicornuate uterus. A study of the urinary system should precede any invasive procedure in women suspected to have a unicornuate uterus. FIG.2. G r o d l of miillerian duct along wol&m duct. Reprinted with permiesion.
ated with urinary tract anomalies in almost half of the cases. Because unicomuate uterus is a rare malformation that is difficult to recognize without invasive investigations, it is diagnosed only by the presence of gynecological symptoms (pelvic pain, infertility or repeated abortion) and for this reason the exact prevalence and actual clinical prome are unknown. However, none of our patients had urological symptom and we consider that in the absence of this bias our reaulta reflect the actual frequency of the association between unicomuate uterus and urinary tract anomalies. Accordiag to the classical experiences of Gruenwald,' and Marehall and Beisel' the meeonephric ducts would act as an important guide in the downward growth of the miillerian ducts (fig. 2). The ureteral bud also arises from the mesonephric duct, and a reciprocal induction is known to exist between the ureteral bud and the metanephric blastema.
REFERENCES
1. Marshall, F.F.and Beisel, D. S.:The association of uterine and renal anomalies. Obst.Gynec.,51: 559,1978. 2. SMbbe, E. H.,Willemsen, W.N.,Lemmens, J. A., Thijn, C. J. and Rolland, R.: Mayer-Rokitansky-Kiister-Hausersyndrome: distinction between two forms based on excretory urographic, sonographic and lapamsmpic findings. AJR, 160: 331, 1993. 3. Buttram, V. C., Jr. and Gibbons, W. E.: Miillenan anomalies: a proposed claseilication. (An analysis of 144 cases). Fertil. Steril., 3 2 40,1979. 4. Gruenwald, P.:Relation of growing miillenan duct to wohlian duct and its importance for genesis of malformations. h a t . Rec., 81: 1, 1941. 5. Woolf, R. B. and Allen, W. M.: Concomitant malformations; frequent, simultaneous occurence of congenital malformatiom of reproductive and Urinary tracts. Obst. Gynec., 2: 236,1963. 6. Heinonen, P.K.: Clinical implications of the unicornuate uterus with rudimentary horn. Int. J. Gynec. Obst., 21: 145,1983. 7. Moutos, D. M., Damewood, M. D., SchlafF, W. D. and Rock, J. A: A comparison of the reproductive outcome between women with a unicornuate uterus and women with a didelphic uterus. Fertil. Steril., 68: 88,1992.
VOl. 155,&47-&48. March 1996 Printed in U S A .
JOURNAL OF UROLOGY
Copyright 0 1996 by AMERICAN U R O ~ I CA~SOCUTION, AL INC.
URINARY TRACT ANOMALIES ASSOCIATED WITH UNICORNUATE UTERUS LUIGI FEDELE,* STEFAN0 BIANCHI, BENEDETTA AGNOLI, LUCA TOZZI
AND
MARIO VIGNALI
Milano,Milano, Italy
From the Department of Obstetrics and Gynecotogy, University of
ABSTRACT
Purpose: We investigated the association between the various subclasses of the unicornuate uterus and urinary tract anomalies. Materials and Methods: We studied the urinary tract of 37 patienta with a unicornuate uterus by urography andor ultrasonography. Unicornuate uterus was diagnosed at laparoecopy or laparotomy. Results: A total of 15 patients (40.5%) had urinary tract anomalies, includingectopic kidney in 4, renal agenesis in 6, double renal pelvis in 2, horseshoe kidneys in 2 and unilateral medullary sponge kidney in 1.No differences were observed in the frequency of urinary anomalies among the various subclasses of unicornuate uterus. Conclusions: Our results indicate the need to suspect a urinary tract anomaly,particularly of the kidney, in all women with a unicornuate U ~ N S . KEYW o w : uterus, kidney, urinary tract, abnormalities
The association between urinary and genital malformations has long been recognized. Marshall and Beisel demonstrated that a single mesonephric duct abnormality can result in renal and uterine anomalies.1 Large series of some genital malformations, such as uterovaginal atresia, have been reported that clearly delineate the types of associated urinary tract anomalies2 but to date only small series of unicornuate uterus have been reported. The anatomical presentations of unicornuate uterus are particularly heterogeneous. Therefore, it seemed appropriate to investigate the association between the various subclasses of this rare malformation and urinary tract anomalies. MATERIALS AND METHODS
Between 1975 and 1994,49 patients 14 to 40 years old were diagnosed with a unicornuate uterus at laparoseopy or laparotomy at our department. Five patients underwent emergency surgery for an acute abdomen due to a ruptured rudimentary horn containing an ectopic pregnancy (1)and hemoperitoneum caused by hematometra of the rudimentary FIG.1. Buttram and Gibbons elaeeifieationof unimmuate utehorn (4). The remaining 44 patients were referred to us for Reprinted with infertility (35) or pelvic pain (9).The malformationwas classified according to the Buttram and Gibbons system (fig. 1hS Association between unicornuate uterus subclasses and uml&cal The urinary tract was studied in 37 patients and 12 did not anomalies undergo urological investigationfor organizational, personal or medical reasons. No patient reported symptoms relating to the urinary tract. Until 1980 patients were studied only by _ _ umgraphy. Af'ter that date ultrasonography of the urinary ~ 2 a 2. R e d agenesis tract was performed on all patients and urography was done Ectopic kidney 1 1 2* 1 1 only if urological anomalies were suspected. Horseshoe kidney ~
-
Double renal pelvie Unilat. medullary sponge kidney
RESULTS
1 __ ~
1
-
1
-
__
Total No. (46) 4 (40) s (36 6 (64) The table summarizes the frequency of associations beNo renal anomalies were noted in 2 patients with a subelass Ala unicomuate tween the unicornuate uterus subclasses and the types of UtelUS. urinary anomalies in our series. Overall, 15 patients (40.5%) * Both anomalies oeeurred in 1 patient. had urinary tract anomalies.The most fresuent anomaly was renal agenesis contralateral to the unicornuate uterus in 6 cases. In 1 woman renal agenesis was associated with an was observed in the tkequency of urinary tract anomalies ectopic kidney ipsilateral to the hemi-uterus. No difference among the various subclasses of unicornuate uterus. DISCUSSION
F p t e d for publication July 28,1995. Requests for reprints:Department of Obstetrics and Gynecology. University of Milano, via Commenda 20122, Milano, Italy.
To our knowledge our series is the largest reported to date and our results indicate that unicornuate uterus is associ-
a47
a48
URINARY TRACT ANOMALIES ASSOCIATED WITH UNICORNLJATE UTERUS
Therefore, it appears obvious that a single mesonephric duct abnormality can result in renal and uterine anomalies. Analysis of our series did not demonstrate a direct relatiomhip between damage to the miillerian and mesonephric ducts. In fact, the frequency and severity of urological anomalies associated with subclass B, in which there is complete agenesis of a miillerian duct, were no greater than those in the subclasses with partial development of this duct. The latter findingagrees with the study by Wmlf and Allen of 11 women with unicomuate uterus in which unilateral renal agenesis was observed in all 3 cases of subclass A h 5 Heinonen found 9 cases of urinary tract anomaly in 15 women with a unicomuate uterus investigated by urography.6 More recently Moutos et al reported an association between urological anomalies and unicomuate uterus in 7 of the 19 patients studied.' From the clinical viewpoint our results indicate the need to suspect a urinary anomaly, particularly of the kidney, in all women in whom a unicomuate uterus has been diagnosed. Therefore, a preliminary study of the urinary system is mandatory before performing any invasive procedure, diagnostic or therapeutic, that could result in serious damage to renal function, especially in the presence of a pelvic kidney. CONCLUSIONS
Urinary tract anomalies are frequently found in women with a unimmuate uterus. The most frequent anomaly was renal agenesis contralateral to the hemi-uterus and edopic kidney. The frequency of urinary anomalies was similar in the various subclasses of unicornuate uterus. A study of the urinary system should precede any invasive procedure in women suspected to have a unicornuate uterus. FIG.2. G r o d l of miillerian duct along wol&m duct. Reprinted with permiesion.
ated with urinary tract anomalies in almost half of the cases. Because unicomuate uterus is a rare malformation that is difficult to recognize without invasive investigations, it is diagnosed only by the presence of gynecological symptoms (pelvic pain, infertility or repeated abortion) and for this reason the exact prevalence and actual clinical prome are unknown. However, none of our patients had urological symptom and we consider that in the absence of this bias our reaulta reflect the actual frequency of the association between unicomuate uterus and urinary tract anomalies. Accordiag to the classical experiences of Gruenwald,' and Marehall and Beisel' the meeonephric ducts would act as an important guide in the downward growth of the miillerian ducts (fig. 2). The ureteral bud also arises from the mesonephric duct, and a reciprocal induction is known to exist between the ureteral bud and the metanephric blastema.
REFERENCES
1. Marshall, F.F.and Beisel, D. S.:The association of uterine and renal anomalies. Obst.Gynec.,51: 559,1978. 2. SMbbe, E. H.,Willemsen, W.N.,Lemmens, J. A., Thijn, C. J. and Rolland, R.: Mayer-Rokitansky-Kiister-Hausersyndrome: distinction between two forms based on excretory urographic, sonographic and lapamsmpic findings. AJR, 160: 331, 1993. 3. Buttram, V. C., Jr. and Gibbons, W. E.: Miillenan anomalies: a proposed claseilication. (An analysis of 144 cases). Fertil. Steril., 3 2 40,1979. 4. Gruenwald, P.:Relation of growing miillenan duct to wohlian duct and its importance for genesis of malformations. h a t . Rec., 81: 1, 1941. 5. Woolf, R. B. and Allen, W. M.: Concomitant malformations; frequent, simultaneous occurence of congenital malformatiom of reproductive and Urinary tracts. Obst. Gynec., 2: 236,1963. 6. Heinonen, P.K.: Clinical implications of the unicornuate uterus with rudimentary horn. Int. J. Gynec. Obst., 21: 145,1983. 7. Moutos, D. M., Damewood, M. D., SchlafF, W. D. and Rock, J. A: A comparison of the reproductive outcome between women with a unicornuate uterus and women with a didelphic uterus. Fertil. Steril., 68: 88,1992.