CASE REPORT
Uterus didelphys with unilateral obstructed hemivagina and renal agenesis on the same side EFTALERDOGAN, GULINOKANAND OMERDARAGENLI From the Department of Obstetrics and Gynecology, University Hospita1,Uludag University, Bursa, Turkey
Actci
Oh.ci~r(;ynecol .%rind 1092; 71 : 7G77
A rare condition. complcte or incomplete duplication of uterus and cervix with unilateral vaginal obstruction. is usually associated with ipsilateral renal agenesis. In such kinds of Miillerian and Wolffim duct anomalies as the one reported here with accumulation o f mcnstrual blood in the obstructed vagina, the patient usually complains of a pelvic mass and associated sevcre and increasing dysmenorrhea. A case, diagnosed and treated at our Department, is prcsented.
Key words: Mullerian duct developmental anomaly; hematocolpos; uterus didelphys Suhmittc~dApril N, 1991
R~.cepti~il Septrtnher 9, 1991
Case report N . K., a 15-year-old. white virgin female was admitted because o f a pelvic mass and with complaints o f constipation and pain in the lower abdomen for the past 2-3 months. Her first menstrual period was K months previously and she was menstruating regularly. She described severe dysmenorrhea with the last three menstruations. Her past and family history was negative. Physical examination was normal. Pelvic examination revealed an intact hymen and normal external genitalia. On rectal examination ( l ) , a poorly defincd semi-solid pelvic mass was palpated. According to our customs, virgins are not examined vaginally by routine unless there is a vital problem. On abdominal ultrasound, the right kidney could not be visualized and the left renal pelvis was cnlarged. There wcrc two masses in the pelvic cavity, measuring 1 3 . 8 ~ 8 . 4cm and 6 . 5 ~ 4 . 3cm resembling semi-solid ovarian cysts. On recto.\igmoidoscopy, pressure in the lower rectum due to a n external mass was noted. Intravenous pyelography (IVP) revealed an absent right urinary systcm and dilated calices o n the left side. tystoscopy was performed under general anestheAi’Iu Oh.\ll’l Cytl(’(’fJ/. k I f l d 71 (IYYZ)
sia, which revealed a normal bladder and the left ureteral orifice. As the right ureteral orifice could not be visualized, the ureteral catheter was placed on the left side. The patient then underwent laparotomy. At laparotomy, no right kidney and ureter were present, confirming the findings of previous examinations. There were double uteri, each normal in size and with normal fallopian tubes. Between the two uteri, there was a soft semi-solid mass measuring 10x5 cm extending down to the vagina and displacing the two uteri upwards and pressing on the rectum (Fig. 1). The second mass visualized by ultrasound was probably the two uteri. The aspiration material from the cystic mass was dark colored defibrinated blood which was thought to be hematocolpos with a blind vagina. A vaginal examination was therefore deemed necessary. The situation was explained to her parents and, by getting their legal permission, vaginal examination was performed. On vaginal examination, a normal vagina and cervix were seen on the left side. On the other side there was the mass which, as we thought, was the accumulation of blood in the right obstructed hemivagina, beginning 1 cm behind the introitus vagina, extending and displacing the two uteri up into the pelvic cavity. About SO0 cc of defibrinated blood was
Uterus didelphys with obstructed hemivagina
77
from the hymenal ring, is not affected. Laparotomy in such cases is not indicated, and should be avoided. The excision of the vaginal septum is regarded as the curative treatment (2). Delay in diagnosis and treatment may give way to infertility. Because of chronic cryptomenorrhea, hematometra, hematosalpinx, and even in some cases hemoperitoneum can occur. Unification of the uteri ought to be delayed, and can be performed at some future time, if deemed necessary (1, 5 ) .
References Fig. I
drained through a longitudinal incision in the mass wall and at the end of the cavity a normal cervix was seen. The septum between the mass and the normal vagina was excised. A new single vagina with two uteri and cervices was obtained.
1. Berman L, Stringer D A , Stonge 0 , Daneman A , Smith C. Case report. Unilateral hematocolpos in utcrine duplication associated with renal agenesis. Clin Radio1 1987; 38: 545-7. 2. Hording U , Legarth J . Uterus didelphys with a unilateral imperforate hemivagina and ipsilateral renal agenesis. Acta Obstet Gynecol Scand 1987; 66: 277-8. 3. Felding C. Obstetric studies in women with congenital solitary kidneys. Acta Obstet Gynecol Scand 1Y65; 64:
555,
Discussion Uterine malformation due to partial or total abnormal fusion of the Mullerian ducts, has been found in some 0.3-1.5% in different series. In women, ipsilateral renal agenesis associated with genital organ malformations, especially with uterine duplication, is not so rare as is thought. In the literature, the incidence is variously reported as 7489% (1, 2, 3 ) . According to the most accepted theory, during intra-uterine development, the Wolffian o r mesonephric ducts precede the Miillerian ones (2, 4). Between the 6th and 16th week of intra-uterine life, the Mullerian ducts, just lateral to the Wolffian ducts, grow toward midline and come into contact with each other. They then fuse and continue to grow downwards. Completing the fusion, they join the urogenital sinus. When fusion is complete, by the 16th week, the uterus, Fallopian tubes on each side and vagina down to the hymenal ring are formed. If one of the Wolffian ducts is absent, the kidney and ureter on the same side will fail to develop. The Mullerian ducts may also fail to fuse in the midline, either completely or incompletely. If the failure to fuse is complete, a uterus didelphys is formed. The Miillerian duct on the side where Wolffian duct is absent, displaces itself laterally, and cannot come into contact with the urogenital sinus in the center. Thus the contralateral Miillerian duct gives way to a vagina, while its already displaced component on the other side forms a blind sac, i.c. an imperforate or obstructed hemivagina. The distal part of the vagina which derives from the urogenital sinus, being distal
4. Marshall FF, Beisel DS. The association of uterinc and renal anomalies. Obstet Gynecol 1978; 51: 559-61. 5. Green LK. Harris LE. Uterine anomalies. Frequcncy of
diagnosis and associated obstetric complications. Obstet Gynecol 1976; 47 (4): 427-9. Address for correspondence:
Eftal Erdogan, M.D. Department of Obstetrics & Gynecology University Hospital Uludag University Bursa Turkey
Acta Ohsrer Gynecol Scand 71 (1992)
Uterus didelphys with unilateral obstructed hemivagina and renal agenesis on the same side EFTALERDOGAN, GULINOKANAND OMERDARAGENLI From the Department of Obstetrics and Gynecology, University Hospita1,Uludag University, Bursa, Turkey
Actci
Oh.ci~r(;ynecol .%rind 1092; 71 : 7G77
A rare condition. complcte or incomplete duplication of uterus and cervix with unilateral vaginal obstruction. is usually associated with ipsilateral renal agenesis. In such kinds of Miillerian and Wolffim duct anomalies as the one reported here with accumulation o f mcnstrual blood in the obstructed vagina, the patient usually complains of a pelvic mass and associated sevcre and increasing dysmenorrhea. A case, diagnosed and treated at our Department, is prcsented.
Key words: Mullerian duct developmental anomaly; hematocolpos; uterus didelphys Suhmittc~dApril N, 1991
R~.cepti~il Septrtnher 9, 1991
Case report N . K., a 15-year-old. white virgin female was admitted because o f a pelvic mass and with complaints o f constipation and pain in the lower abdomen for the past 2-3 months. Her first menstrual period was K months previously and she was menstruating regularly. She described severe dysmenorrhea with the last three menstruations. Her past and family history was negative. Physical examination was normal. Pelvic examination revealed an intact hymen and normal external genitalia. On rectal examination ( l ) , a poorly defincd semi-solid pelvic mass was palpated. According to our customs, virgins are not examined vaginally by routine unless there is a vital problem. On abdominal ultrasound, the right kidney could not be visualized and the left renal pelvis was cnlarged. There wcrc two masses in the pelvic cavity, measuring 1 3 . 8 ~ 8 . 4cm and 6 . 5 ~ 4 . 3cm resembling semi-solid ovarian cysts. On recto.\igmoidoscopy, pressure in the lower rectum due to a n external mass was noted. Intravenous pyelography (IVP) revealed an absent right urinary systcm and dilated calices o n the left side. tystoscopy was performed under general anestheAi’Iu Oh.\ll’l Cytl(’(’fJ/. k I f l d 71 (IYYZ)
sia, which revealed a normal bladder and the left ureteral orifice. As the right ureteral orifice could not be visualized, the ureteral catheter was placed on the left side. The patient then underwent laparotomy. At laparotomy, no right kidney and ureter were present, confirming the findings of previous examinations. There were double uteri, each normal in size and with normal fallopian tubes. Between the two uteri, there was a soft semi-solid mass measuring 10x5 cm extending down to the vagina and displacing the two uteri upwards and pressing on the rectum (Fig. 1). The second mass visualized by ultrasound was probably the two uteri. The aspiration material from the cystic mass was dark colored defibrinated blood which was thought to be hematocolpos with a blind vagina. A vaginal examination was therefore deemed necessary. The situation was explained to her parents and, by getting their legal permission, vaginal examination was performed. On vaginal examination, a normal vagina and cervix were seen on the left side. On the other side there was the mass which, as we thought, was the accumulation of blood in the right obstructed hemivagina, beginning 1 cm behind the introitus vagina, extending and displacing the two uteri up into the pelvic cavity. About SO0 cc of defibrinated blood was
Uterus didelphys with obstructed hemivagina
77
from the hymenal ring, is not affected. Laparotomy in such cases is not indicated, and should be avoided. The excision of the vaginal septum is regarded as the curative treatment (2). Delay in diagnosis and treatment may give way to infertility. Because of chronic cryptomenorrhea, hematometra, hematosalpinx, and even in some cases hemoperitoneum can occur. Unification of the uteri ought to be delayed, and can be performed at some future time, if deemed necessary (1, 5 ) .
References Fig. I
drained through a longitudinal incision in the mass wall and at the end of the cavity a normal cervix was seen. The septum between the mass and the normal vagina was excised. A new single vagina with two uteri and cervices was obtained.
1. Berman L, Stringer D A , Stonge 0 , Daneman A , Smith C. Case report. Unilateral hematocolpos in utcrine duplication associated with renal agenesis. Clin Radio1 1987; 38: 545-7. 2. Hording U , Legarth J . Uterus didelphys with a unilateral imperforate hemivagina and ipsilateral renal agenesis. Acta Obstet Gynecol Scand 1987; 66: 277-8. 3. Felding C. Obstetric studies in women with congenital solitary kidneys. Acta Obstet Gynecol Scand 1Y65; 64:
555,
Discussion Uterine malformation due to partial or total abnormal fusion of the Mullerian ducts, has been found in some 0.3-1.5% in different series. In women, ipsilateral renal agenesis associated with genital organ malformations, especially with uterine duplication, is not so rare as is thought. In the literature, the incidence is variously reported as 7489% (1, 2, 3 ) . According to the most accepted theory, during intra-uterine development, the Wolffian o r mesonephric ducts precede the Miillerian ones (2, 4). Between the 6th and 16th week of intra-uterine life, the Mullerian ducts, just lateral to the Wolffian ducts, grow toward midline and come into contact with each other. They then fuse and continue to grow downwards. Completing the fusion, they join the urogenital sinus. When fusion is complete, by the 16th week, the uterus, Fallopian tubes on each side and vagina down to the hymenal ring are formed. If one of the Wolffian ducts is absent, the kidney and ureter on the same side will fail to develop. The Mullerian ducts may also fail to fuse in the midline, either completely or incompletely. If the failure to fuse is complete, a uterus didelphys is formed. The Miillerian duct on the side where Wolffian duct is absent, displaces itself laterally, and cannot come into contact with the urogenital sinus in the center. Thus the contralateral Miillerian duct gives way to a vagina, while its already displaced component on the other side forms a blind sac, i.c. an imperforate or obstructed hemivagina. The distal part of the vagina which derives from the urogenital sinus, being distal
4. Marshall FF, Beisel DS. The association of uterinc and renal anomalies. Obstet Gynecol 1978; 51: 559-61. 5. Green LK. Harris LE. Uterine anomalies. Frequcncy of
diagnosis and associated obstetric complications. Obstet Gynecol 1976; 47 (4): 427-9. Address for correspondence:
Eftal Erdogan, M.D. Department of Obstetrics & Gynecology University Hospital Uludag University Bursa Turkey
Acta Ohsrer Gynecol Scand 71 (1992)
