© 1991 S. Karger AG, Basel 0302-2838/91/0201 -0085S2.75/0
Eur Urol 1991;20:85-88
Urological Complications Associated to Uterus Didelphys with Unilateral Hematocolpos A Case Report and Review of the Literature
E. Broseta, F. Boronat, J.L. Ruiz, M. Alonso, J.M. Osca, J.F. Jiménez-Cruz
1606053
Service of Urology, La Fe Hospital, Valencia, Spain
Key Words. Uterus didelphys • Hematocolpos • Renal agenesis Abstract. Complete duplication of vagina, cervix and uterus, with imperforate hemivagina and renal agenesis is a rare condition with less than 120 cases published. In those articles, urological complications are uncommon with only a 10% of the total. We report 2 cases of uterus didelphys with unilateral hematocolpos and ipsilateral renal agenesis with urological clinical complications. One of them presented a very rare onset complaining of acute urinary retention. The other patient was referred for difficulty in micturition and abdominal pain which is the most common symptom of this entity. A description of both cases and a literature review of this congenital complex syndrome and its urological complications are reported.
Complete duplication of vagina, cervix and uterus with imperfo rate hemivagina is a rare condition caused by combined miillerian and mesonephric duct abnormalities. This syndrome is always asso ciated with unilateral renal agenesis on the same side of the vaginal obstruction. Uterus didelphys consists of a complete duplication of the uterus with two separate horns fused at the level of both cer vices. In 1922, Purslow [1] was the first author reporting a case of hematocolpos, hematometra and hematosalpinx with a double uterus but without any reference to renal agenesis. In the same year, Miller [2] reported a 14-year-old patient with uterus didelphys, hematocolpos and left renal agenesis complaining of lower left quad rant pain and severe dysmenorrhea. The isolated incidence of unilateral renal agenesis varies from 1/500 to 1/1,300 being associated with genital anomalies in 25-70% [3-5]. The incidence of uterus didelphys is between 1 per 2,000 and 1 per 28,000 with 43% ipsilateral renal agenesis [6, 7], In women with a solitary kidney, 55-70% of associated genital anomalies are also reported [3]. In a study of 52 women with unilateral renal agen esis, Thompson and Lynn [8] found 32 % of associated uterus didel phys. The clinical features of this syndrome are cyclic dysmenorrhea in teenagers with lower abdominal pain and unilateral pelvic mass. Sometimes it is possible to notice a very important sign, a purple mass - suggesting its blood content - bulging into one of the lateral walls of the vagina. Diagnostic studies confirm the ipsilateral renal
agenesis, uterine, cervical and vaginal duplication with an imperfo rate hemivagina resulting in a hematocolpos. Other frequent symp toms are profuse vaginal discharge, intermenstrual pain and occa sionally acute urinary retention due to mechanical compression by the hematocolpos.
Case Reports Case 1
A 12-year-old girl was referred to our hospital with acute urinary retention of 12 h of evolution. Her menarche had started 1 year ago followed by a 6-month period of amenorrhea. After that, she began with apparently normal menses till the moment of her consultation. On examination, a distended bladder was revealed. Urethral cathe terization was used obtaining 600 ml of clear urine. Vaginal explo ration revealed a soft purple mass bulging the anterior and left lat eral wall of the vagina (fig. 1). On bimanual examination, a smooth, round and painless mass of 10 cm bulging the anterior wall of the rectum was present. Biochemical and hematologic parameters were normal and so was the plain abdominal film. An ultrasound scan was performed showing a 13 X 19 X 10-cm mass beside the vagina with echoes inside and with liquid character istics (fig. 2). A didelphys uterus was also evident and no left kidney could be found. Cystoscopy showed a lateral mass in the left portion of the bladder base and lack of left ureteral meatus. Excretory urog raphy (IVP) confirmed the left renal agenesis, and a large round defect in the upper part of the bladder was demonstrated (fig. 3). Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
Introduction
Broseta/Boronat/Ruiz/Alonso/Osca/Jiménez-Cruz
86
Fig. 1. Mass bulging in the introitus.
Fig. 2. Abdominal echography. B = Bladder; H = hematocolpos.
Fig. 3. IVP. Left renal agenesis. Mass compressing the bladder. Fig. 4. IVP. Absence of right kidney. Bladder compressed by right mass.
Case 2
A 14-year-old girl, gravida 0 and with menarche at the age of 13, having regular menses with dysmenorrhea, presented with a history of lower abdominal crampy pain associated with nausea, vomiting and impairment of micturition and defecation. On examination, a
palpable tender and cystic lower abdominal mass was revealed and confirmed on bimanual exploration. After urethral catheterization draining 550 ml, the mass was reduced. IVP showed a solitary slightly enlarged left kidney, absence of the right one, and a bladder compressed by a right posterior large mass (fig. 4). Colpography revealed an uterus didelphys (fig. 5). An ultrasound scan confirmed the solitary left kidney and a cystic mass of 9.5 X 6 cm due to hematocolpos and uterus didelphys (fig. 6). The vaginal septum was excised and 400 ml of dark fluid drained out. The patient was dis charged a few days later and has remained asymptomatic since the surgical procedure. Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
With the diagnosis of uterus didelphys, hematocolpos and left renal agenesis, the vaginal septum was excised draining 500 ml of brown ish fluid. A didelphys uterus with normal ovaries and renal agenesis was confirmed by computed tomography. The patient was dis charged a few days later.
Uterus Didelphys with Unilateral Hematocolpos
87
I
.
r
p
Fig. 5. Colpography. Uterus didelphys.
Fig. 6. Abdominal echography. B = Bladder; H = hematocolpos; UT = uterus.
Discussion
reported in the literature reviewed. Extraurological manifestations were: lumbar pain (36%), lower abdominal pain (35%), dysmenor rhea (21 %), vaginal discharge (14%), peritonitis (4%), rectal pres sure (4%), mass sensation (4%) and dyspareunia (3%) [1-3, 5, 6, 8-22], The proportion of associated urological complications is note worthy in 10% of cases: difficult micturition [21, 23], urinary fre quency [3, 24], urinary tract infection [9, 25], pain radiating to ure thra [5], stress urinary incontinence [26], and 5 particularly remark able cases of acute urinary retention, 4 as a first symptom [13, 2729] and the other preceded by dysuria [17], In our own cases, the urological symptomatology was the main cause of consultation. Therefore, an acute urinary retention in a teenager must alert the urologist in order to consider this entity among the diagnostic pos sibilities. Based on the reported data, renal agenesis seems to occur more frequently and for unknown causes on the right side, in 66 % of cases in the Morgan et al. [14] series and in 83 % of the cases of Wiersma et al. [5], Taking the classification of Magee et al. [9], the more frequent group with anomalies is group 3 (60 %) followed by group 2 (32%). In this last group can be included our 2 cases. The mean age at onset of urological complications was 13 years (range 5 months 20 years). The mean lapse of time from menarche was 14 months. In relation to diagnosis, lower abdominal pain is the more com mon symptom. When associated to unilateral pelvic mass and renal ipsilateral agenesis, the suspicion of this polymalformative syn drome is mandatory. Pelvic examination, abdominal echography, IVP, colpography and hysterosalpingography are the usual diagnos tic procedures. Cystoscopy may aid this diagnosis confirming the absence of ipsilateral hemitrigone although it cannot demonstrate the unilateral renal agenesis because a kidney may be present and drains though an ectopically located orifice. In other cases, diagnosis is made during an exploratory laparotomy [15]. Complications reported regarding this syndrome are sponta neous abortions in 25-53%, a greater incidence of twins and breech presentations and a lack of correct dilation of the small cervix requiring cesarean section in 25% of cases [5, 30]. The most common treatment is incision of the obstructing sep tum providing adequate drainage to the retained blood. In a further
Based on the actual knowledge on the development of the female genitourinary tract [9-14], the lack of fusion of the miillerian ducts or the absence of resorption of the septum, results in varied genital anomalies: vaginal atresia, uterus didelphys, unicomate or bicornate uterus. The displaced miillerian duct cannot contact the uroge nital sinus, forming a blind sac instead of a vagina. The distal onefifth is not affected because it derives from the urogenital sinus. Renal, ureteral and trigonal ipsilateral agenesis are the logical result of a failed differentiation of the mesonephric duct and ureteral bud. The hypothetical teratogenic activity responsible for the anoma lies in the miillerian and mesonephric ducts is located between the 4th and 13th week of fetal development [5]. Magee et al. [9] reported a classification that helps to simplify the problem: (a) unilateral renal agenesis type I: nondifferentiation of nephrogenic ridge with a certain degree of retardation of meso nephric and miillerian components. If unilateral, there is an uterus of simple miillerian origin associated with contralateral renal agen esis. If bilateral, the absence of kidneys, ureters and fallopian tubes is complete; (b) unilateral renal agenesis type II: faulty differentia tion of ureteral bud and mesonephric duct. The latter prevents its crossover to the midline creating a duplicated state. The displaced miillerian component forms a blind sac; (c) unilateral renal agenesis type III: absence of development of the metanephric blastema with normal growth of mesonephric and miillerian ducts. In this case, a normal genital tract is the norm. In 1980, Rock and Jones [10] proposed a subdivision depending on the existence of a whole vaginal septum and uterine communica tion: group 1: Complete vaginal obstruction with hematocolpos; group 2: Incomplete vaginal obstruction. The retained fluid drains through different points in the partially obstructed vaginal pouch. The clinical result is an intermittent foul mucopurulent discharge; group 3: Complete vaginal obstruction with a laterally communicat ing double uterus. Clinically, the patients have vaginal mass and dysmenorrhea. The association of uterus didelphys, unilateral renal agenesis and hematocolpos is rather uncommon with less than 120 cases
Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
!
Broseta/Boronat/Ruiz/Alonso/Oscayjiménez-Cruz
operation, vaginal septum should be excised totally resulting in a single vagina with two cervices [3, 11-14]. Early diagnosis is impor tant in order to perform the safest procedure that improves the fer tility and life expectancy.
References 1 Purslow CE: A case of unilateral haematocolpos, haematometra and haematosalpinx. J Obstet Gynecol Br Emp 1922;28:643. 2 Miller NF: Clinical aspects of uterus didelphys. Am J Obstet Gynecol 1922;4:398. 3 Anderson KA, McAninch JW: Uterus didelphia with left hematocolpos and ipsilateral renal agenesis. J Urol 1982;127:550553. 4 Perlmutter AD, Retik A, Bauer S: Anomalies of the upper uri nary tract; in Walsh PC, Gittes RF, Perlmutter AD, Stamey TA (eds): Campbell’s Urology. London, Saunders, 1986, voi 2, pp 1665-1759. 5 Wiersma AF, Peterson LF, Justema EJ: Uterine anomalies asso ciated with unilateral renal agenesis. Obstet Gynecol 1975;47: 654-657. 6 Semmens JP: Congenital anomalies of the female genital tract: Functional classification based on review of 56 personal cases and 500 reported cases. Obstet Gynecol 1962; 19;328. 7 Smith FR: The significance of incomplete fusion of the miiller ian ducts in pregnancy and parturition with a report on 35 cases. Am J Obstet Gynecol 1931 ;22:714. 8 Thompson DP, Lynn HB: Genital anomalies associated with sol itary kidney. Mayo Clin Proc 1966;41:538. 9 Magee MC, Lucey DT, Fried F: A new embryologie classifica tion for uro-gynecologic malformations: The syndromes of me sonephric duct-induced miillerian deformities. J Urol 1979; 121: 265-267. 10 Rock JA, Jones HW: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Ob stet Gynecol 1980;138:339-342. 11 Gilliland B, Dyck F: Uterus didelphys associated with unilateral imperforate vagina. Obstet Gynecol 1976;48:5s-7s. 12 Burbige KA, Hensle TW: Uterus didelphys and vaginal duplica tion with unilateral obstruction presenting as a newborn abdom inal mass. J Urol 1984; 132:1195-1198. 13 Eisenberg E, Färber M, Mitchell GW, Turksoy RN, Rule AH: Complete duplication of the uterus and cervix with a unilaterally imperforate vagina. Obstet Gynecol 1982;60:259-262. 14 Morgan MA, Thumau GR, Smith ML: Uterus didelphys with unilateral hématocolpos, ipsilateral renal agenesis and menses. A case report and literature review. J Reprod Med 1987;32:47— 58. 15 Sayer T, O’Reilly PH: Bicomuate and unicomuate uterus asso ciated with unilateral renal aplasia and abnormal solitary kid neys: Report of three cases. J Urol 1985;135:110—111.
16 Hording U, Legarth J: Uterus didelphys with unilateral imperfo rate hemivagina and ipsilateral renal agenesis. Acta Obstet Gy necol Scand 1987;66:277-278. 17 Miyazaki Y, Ebisuno S, Uekado Y, Ogawa T, Senzaki A, Ohkawa T: Uterus didelphys with unilateral imperforate vagina and ipsilateral renal agenesis. J Urol 1985;135:107-109. 18 Legino L, Penney LL: Uterus didelphys with obstructed hemi vagina and pyocolpos, ipsilateral tubal occlusion and renal agen esis, and bilateral cervical hypoplasia. J Reprod Med 1989;34: 237-240. 19 Denis X, Aubineau JM, Rousseau O, Mazeman E: Utérus didelphe avec hémi-vagin borgne et agénésie rénale homolatérale. Ann Urol 1989;23:31-34. 20 Tarvidel-Raimondeau P, Bouche-Pillon M, Boulenger E, Lefort G, Daoud S: Hématocolpos unilatéral sur duplicité utéro-vaginale associé à une agénésie rénale homolatérale. Chir Pédiatr 1988;29:208-212. 21 Gibberd AA: Unilateral hématocolpos haematometra. Br Med J 1957;i:213. 22 Tridenti G, Armanetti M, Flisi M, Benassi L: Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: Report of three cases. Am J Obstet Gynecol 1988; 159: 882-883. 23 Hill RM: Uterus didelphys with hématocolpos and hematometra on one side and normal menses on the other: Report of an unusual case. J Int Coll Surg 1958;29:422. 24 Azouz EM, Williams RL: Uterus didelphys with unilateral hematometrocolpos and ipsilateral renal agenesis. J Urol 1982; 127:550. 25 Allan N, Cowan LE: Uterus didelphys with unilateral imperfo rate vagina: Report of 4 cases. Obstet Gynecol 1963;22:422. 26 Ball TL, Douglas G: Cryptomenorrhea, congenital and adquired. Am J Surg 1949;78:40. 27 Adams GW, Wilson CE, Holloway JH, Williamson HF: Uterus didelphys with unilateral imperforate vagina: A rare cause of acute urinary retention. J Urol 1979;121:131-132. 28 Passas J, Espuela R, Nogueras M, Abad F: Retención urinaria aguda en una mujer secundaria a utero didelfo con imperforación vaginal unilateral. Actas Urol Esp 1984;7:333-334. 29 Pansini L, Torricelli M, Gomarasca A, Brambilla C, Beolchi S, Sideri M: Acute urinary retention due to didelphys uterus asso ciated with an obstructed hemivagina in a 5-month-old infant. J Pediatr Surg 1988;23:984-985. 30 Lewis BV, Brant HA: Obstetric and gynecologic complications associated with miillerian duct abnormalities. Obstet Gynecol 1966;28:315.
Broseta E. Service of Urology La Fe Hospital Avda. Campanar 21 E-46009 Valencia (Spain)
Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
88
Eur Urol 1991;20:85-88
Urological Complications Associated to Uterus Didelphys with Unilateral Hematocolpos A Case Report and Review of the Literature
E. Broseta, F. Boronat, J.L. Ruiz, M. Alonso, J.M. Osca, J.F. Jiménez-Cruz
1606053
Service of Urology, La Fe Hospital, Valencia, Spain
Key Words. Uterus didelphys • Hematocolpos • Renal agenesis Abstract. Complete duplication of vagina, cervix and uterus, with imperforate hemivagina and renal agenesis is a rare condition with less than 120 cases published. In those articles, urological complications are uncommon with only a 10% of the total. We report 2 cases of uterus didelphys with unilateral hematocolpos and ipsilateral renal agenesis with urological clinical complications. One of them presented a very rare onset complaining of acute urinary retention. The other patient was referred for difficulty in micturition and abdominal pain which is the most common symptom of this entity. A description of both cases and a literature review of this congenital complex syndrome and its urological complications are reported.
Complete duplication of vagina, cervix and uterus with imperfo rate hemivagina is a rare condition caused by combined miillerian and mesonephric duct abnormalities. This syndrome is always asso ciated with unilateral renal agenesis on the same side of the vaginal obstruction. Uterus didelphys consists of a complete duplication of the uterus with two separate horns fused at the level of both cer vices. In 1922, Purslow [1] was the first author reporting a case of hematocolpos, hematometra and hematosalpinx with a double uterus but without any reference to renal agenesis. In the same year, Miller [2] reported a 14-year-old patient with uterus didelphys, hematocolpos and left renal agenesis complaining of lower left quad rant pain and severe dysmenorrhea. The isolated incidence of unilateral renal agenesis varies from 1/500 to 1/1,300 being associated with genital anomalies in 25-70% [3-5]. The incidence of uterus didelphys is between 1 per 2,000 and 1 per 28,000 with 43% ipsilateral renal agenesis [6, 7], In women with a solitary kidney, 55-70% of associated genital anomalies are also reported [3]. In a study of 52 women with unilateral renal agen esis, Thompson and Lynn [8] found 32 % of associated uterus didel phys. The clinical features of this syndrome are cyclic dysmenorrhea in teenagers with lower abdominal pain and unilateral pelvic mass. Sometimes it is possible to notice a very important sign, a purple mass - suggesting its blood content - bulging into one of the lateral walls of the vagina. Diagnostic studies confirm the ipsilateral renal
agenesis, uterine, cervical and vaginal duplication with an imperfo rate hemivagina resulting in a hematocolpos. Other frequent symp toms are profuse vaginal discharge, intermenstrual pain and occa sionally acute urinary retention due to mechanical compression by the hematocolpos.
Case Reports Case 1
A 12-year-old girl was referred to our hospital with acute urinary retention of 12 h of evolution. Her menarche had started 1 year ago followed by a 6-month period of amenorrhea. After that, she began with apparently normal menses till the moment of her consultation. On examination, a distended bladder was revealed. Urethral cathe terization was used obtaining 600 ml of clear urine. Vaginal explo ration revealed a soft purple mass bulging the anterior and left lat eral wall of the vagina (fig. 1). On bimanual examination, a smooth, round and painless mass of 10 cm bulging the anterior wall of the rectum was present. Biochemical and hematologic parameters were normal and so was the plain abdominal film. An ultrasound scan was performed showing a 13 X 19 X 10-cm mass beside the vagina with echoes inside and with liquid character istics (fig. 2). A didelphys uterus was also evident and no left kidney could be found. Cystoscopy showed a lateral mass in the left portion of the bladder base and lack of left ureteral meatus. Excretory urog raphy (IVP) confirmed the left renal agenesis, and a large round defect in the upper part of the bladder was demonstrated (fig. 3). Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
Introduction
Broseta/Boronat/Ruiz/Alonso/Osca/Jiménez-Cruz
86
Fig. 1. Mass bulging in the introitus.
Fig. 2. Abdominal echography. B = Bladder; H = hematocolpos.
Fig. 3. IVP. Left renal agenesis. Mass compressing the bladder. Fig. 4. IVP. Absence of right kidney. Bladder compressed by right mass.
Case 2
A 14-year-old girl, gravida 0 and with menarche at the age of 13, having regular menses with dysmenorrhea, presented with a history of lower abdominal crampy pain associated with nausea, vomiting and impairment of micturition and defecation. On examination, a
palpable tender and cystic lower abdominal mass was revealed and confirmed on bimanual exploration. After urethral catheterization draining 550 ml, the mass was reduced. IVP showed a solitary slightly enlarged left kidney, absence of the right one, and a bladder compressed by a right posterior large mass (fig. 4). Colpography revealed an uterus didelphys (fig. 5). An ultrasound scan confirmed the solitary left kidney and a cystic mass of 9.5 X 6 cm due to hematocolpos and uterus didelphys (fig. 6). The vaginal septum was excised and 400 ml of dark fluid drained out. The patient was dis charged a few days later and has remained asymptomatic since the surgical procedure. Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
With the diagnosis of uterus didelphys, hematocolpos and left renal agenesis, the vaginal septum was excised draining 500 ml of brown ish fluid. A didelphys uterus with normal ovaries and renal agenesis was confirmed by computed tomography. The patient was dis charged a few days later.
Uterus Didelphys with Unilateral Hematocolpos
87
I
.
r
p
Fig. 5. Colpography. Uterus didelphys.
Fig. 6. Abdominal echography. B = Bladder; H = hematocolpos; UT = uterus.
Discussion
reported in the literature reviewed. Extraurological manifestations were: lumbar pain (36%), lower abdominal pain (35%), dysmenor rhea (21 %), vaginal discharge (14%), peritonitis (4%), rectal pres sure (4%), mass sensation (4%) and dyspareunia (3%) [1-3, 5, 6, 8-22], The proportion of associated urological complications is note worthy in 10% of cases: difficult micturition [21, 23], urinary fre quency [3, 24], urinary tract infection [9, 25], pain radiating to ure thra [5], stress urinary incontinence [26], and 5 particularly remark able cases of acute urinary retention, 4 as a first symptom [13, 2729] and the other preceded by dysuria [17], In our own cases, the urological symptomatology was the main cause of consultation. Therefore, an acute urinary retention in a teenager must alert the urologist in order to consider this entity among the diagnostic pos sibilities. Based on the reported data, renal agenesis seems to occur more frequently and for unknown causes on the right side, in 66 % of cases in the Morgan et al. [14] series and in 83 % of the cases of Wiersma et al. [5], Taking the classification of Magee et al. [9], the more frequent group with anomalies is group 3 (60 %) followed by group 2 (32%). In this last group can be included our 2 cases. The mean age at onset of urological complications was 13 years (range 5 months 20 years). The mean lapse of time from menarche was 14 months. In relation to diagnosis, lower abdominal pain is the more com mon symptom. When associated to unilateral pelvic mass and renal ipsilateral agenesis, the suspicion of this polymalformative syn drome is mandatory. Pelvic examination, abdominal echography, IVP, colpography and hysterosalpingography are the usual diagnos tic procedures. Cystoscopy may aid this diagnosis confirming the absence of ipsilateral hemitrigone although it cannot demonstrate the unilateral renal agenesis because a kidney may be present and drains though an ectopically located orifice. In other cases, diagnosis is made during an exploratory laparotomy [15]. Complications reported regarding this syndrome are sponta neous abortions in 25-53%, a greater incidence of twins and breech presentations and a lack of correct dilation of the small cervix requiring cesarean section in 25% of cases [5, 30]. The most common treatment is incision of the obstructing sep tum providing adequate drainage to the retained blood. In a further
Based on the actual knowledge on the development of the female genitourinary tract [9-14], the lack of fusion of the miillerian ducts or the absence of resorption of the septum, results in varied genital anomalies: vaginal atresia, uterus didelphys, unicomate or bicornate uterus. The displaced miillerian duct cannot contact the uroge nital sinus, forming a blind sac instead of a vagina. The distal onefifth is not affected because it derives from the urogenital sinus. Renal, ureteral and trigonal ipsilateral agenesis are the logical result of a failed differentiation of the mesonephric duct and ureteral bud. The hypothetical teratogenic activity responsible for the anoma lies in the miillerian and mesonephric ducts is located between the 4th and 13th week of fetal development [5]. Magee et al. [9] reported a classification that helps to simplify the problem: (a) unilateral renal agenesis type I: nondifferentiation of nephrogenic ridge with a certain degree of retardation of meso nephric and miillerian components. If unilateral, there is an uterus of simple miillerian origin associated with contralateral renal agen esis. If bilateral, the absence of kidneys, ureters and fallopian tubes is complete; (b) unilateral renal agenesis type II: faulty differentia tion of ureteral bud and mesonephric duct. The latter prevents its crossover to the midline creating a duplicated state. The displaced miillerian component forms a blind sac; (c) unilateral renal agenesis type III: absence of development of the metanephric blastema with normal growth of mesonephric and miillerian ducts. In this case, a normal genital tract is the norm. In 1980, Rock and Jones [10] proposed a subdivision depending on the existence of a whole vaginal septum and uterine communica tion: group 1: Complete vaginal obstruction with hematocolpos; group 2: Incomplete vaginal obstruction. The retained fluid drains through different points in the partially obstructed vaginal pouch. The clinical result is an intermittent foul mucopurulent discharge; group 3: Complete vaginal obstruction with a laterally communicat ing double uterus. Clinically, the patients have vaginal mass and dysmenorrhea. The association of uterus didelphys, unilateral renal agenesis and hematocolpos is rather uncommon with less than 120 cases
Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
!
Broseta/Boronat/Ruiz/Alonso/Oscayjiménez-Cruz
operation, vaginal septum should be excised totally resulting in a single vagina with two cervices [3, 11-14]. Early diagnosis is impor tant in order to perform the safest procedure that improves the fer tility and life expectancy.
References 1 Purslow CE: A case of unilateral haematocolpos, haematometra and haematosalpinx. J Obstet Gynecol Br Emp 1922;28:643. 2 Miller NF: Clinical aspects of uterus didelphys. Am J Obstet Gynecol 1922;4:398. 3 Anderson KA, McAninch JW: Uterus didelphia with left hematocolpos and ipsilateral renal agenesis. J Urol 1982;127:550553. 4 Perlmutter AD, Retik A, Bauer S: Anomalies of the upper uri nary tract; in Walsh PC, Gittes RF, Perlmutter AD, Stamey TA (eds): Campbell’s Urology. London, Saunders, 1986, voi 2, pp 1665-1759. 5 Wiersma AF, Peterson LF, Justema EJ: Uterine anomalies asso ciated with unilateral renal agenesis. Obstet Gynecol 1975;47: 654-657. 6 Semmens JP: Congenital anomalies of the female genital tract: Functional classification based on review of 56 personal cases and 500 reported cases. Obstet Gynecol 1962; 19;328. 7 Smith FR: The significance of incomplete fusion of the miiller ian ducts in pregnancy and parturition with a report on 35 cases. Am J Obstet Gynecol 1931 ;22:714. 8 Thompson DP, Lynn HB: Genital anomalies associated with sol itary kidney. Mayo Clin Proc 1966;41:538. 9 Magee MC, Lucey DT, Fried F: A new embryologie classifica tion for uro-gynecologic malformations: The syndromes of me sonephric duct-induced miillerian deformities. J Urol 1979; 121: 265-267. 10 Rock JA, Jones HW: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Ob stet Gynecol 1980;138:339-342. 11 Gilliland B, Dyck F: Uterus didelphys associated with unilateral imperforate vagina. Obstet Gynecol 1976;48:5s-7s. 12 Burbige KA, Hensle TW: Uterus didelphys and vaginal duplica tion with unilateral obstruction presenting as a newborn abdom inal mass. J Urol 1984; 132:1195-1198. 13 Eisenberg E, Färber M, Mitchell GW, Turksoy RN, Rule AH: Complete duplication of the uterus and cervix with a unilaterally imperforate vagina. Obstet Gynecol 1982;60:259-262. 14 Morgan MA, Thumau GR, Smith ML: Uterus didelphys with unilateral hématocolpos, ipsilateral renal agenesis and menses. A case report and literature review. J Reprod Med 1987;32:47— 58. 15 Sayer T, O’Reilly PH: Bicomuate and unicomuate uterus asso ciated with unilateral renal aplasia and abnormal solitary kid neys: Report of three cases. J Urol 1985;135:110—111.
16 Hording U, Legarth J: Uterus didelphys with unilateral imperfo rate hemivagina and ipsilateral renal agenesis. Acta Obstet Gy necol Scand 1987;66:277-278. 17 Miyazaki Y, Ebisuno S, Uekado Y, Ogawa T, Senzaki A, Ohkawa T: Uterus didelphys with unilateral imperforate vagina and ipsilateral renal agenesis. J Urol 1985;135:107-109. 18 Legino L, Penney LL: Uterus didelphys with obstructed hemi vagina and pyocolpos, ipsilateral tubal occlusion and renal agen esis, and bilateral cervical hypoplasia. J Reprod Med 1989;34: 237-240. 19 Denis X, Aubineau JM, Rousseau O, Mazeman E: Utérus didelphe avec hémi-vagin borgne et agénésie rénale homolatérale. Ann Urol 1989;23:31-34. 20 Tarvidel-Raimondeau P, Bouche-Pillon M, Boulenger E, Lefort G, Daoud S: Hématocolpos unilatéral sur duplicité utéro-vaginale associé à une agénésie rénale homolatérale. Chir Pédiatr 1988;29:208-212. 21 Gibberd AA: Unilateral hématocolpos haematometra. Br Med J 1957;i:213. 22 Tridenti G, Armanetti M, Flisi M, Benassi L: Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: Report of three cases. Am J Obstet Gynecol 1988; 159: 882-883. 23 Hill RM: Uterus didelphys with hématocolpos and hematometra on one side and normal menses on the other: Report of an unusual case. J Int Coll Surg 1958;29:422. 24 Azouz EM, Williams RL: Uterus didelphys with unilateral hematometrocolpos and ipsilateral renal agenesis. J Urol 1982; 127:550. 25 Allan N, Cowan LE: Uterus didelphys with unilateral imperfo rate vagina: Report of 4 cases. Obstet Gynecol 1963;22:422. 26 Ball TL, Douglas G: Cryptomenorrhea, congenital and adquired. Am J Surg 1949;78:40. 27 Adams GW, Wilson CE, Holloway JH, Williamson HF: Uterus didelphys with unilateral imperforate vagina: A rare cause of acute urinary retention. J Urol 1979;121:131-132. 28 Passas J, Espuela R, Nogueras M, Abad F: Retención urinaria aguda en una mujer secundaria a utero didelfo con imperforación vaginal unilateral. Actas Urol Esp 1984;7:333-334. 29 Pansini L, Torricelli M, Gomarasca A, Brambilla C, Beolchi S, Sideri M: Acute urinary retention due to didelphys uterus asso ciated with an obstructed hemivagina in a 5-month-old infant. J Pediatr Surg 1988;23:984-985. 30 Lewis BV, Brant HA: Obstetric and gynecologic complications associated with miillerian duct abnormalities. Obstet Gynecol 1966;28:315.
Broseta E. Service of Urology La Fe Hospital Avda. Campanar 21 E-46009 Valencia (Spain)
Downloaded by: University of Exeter 144.173.6.94 - 5/6/2020 3:45:22 AM
88
