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Twin pregnancy in the right horn of a uterus didelphys: a case report
European Journal of Obstetrics & Gynecology and Reproductive Elsevier
mo
Biology, 32 (1989) 287-292
287
00797
Twin pregnancy in the right horn of a uterus didelphys: a case report N. Kanakas Department
*, R. Boos and W. Schmidt
of Obstetrics and Gynecology, University of Heidelberg, F. R. G Accepted for publication 9 December 1988
Summary A rare case of a twin pregnancy occurring in the right horn of a uterus didelphys (double uterus, double cervix and septate vagina) is reported. The occasional diagnosis of this uterine anomaly was made after the pregnancy was detected. Both embryos, with cardiac actions which proved the viability of the gestation from the very early stage of pregnancy, were detected by transvaginal ultrasonography. Two male infants were delivered by Cesarean section in the 34th week. The importance of the transvaginal sonography and the handling of the case during this high-risk pregnancy are reported. Uterus didelphys; Twin pregnancy; Transvaginal sonography
Introduction Fusion of the two Mullerian ducts and the establishment of the vaginal canal are usually completed between the 10th and 17th week of intra-uterine development. It is obvious that a variety of malformations may appear. Several degrees of duplication of the uterus are possible, ranging from a complete duplication of the uterus, cervix and vaginal canal, such as the so-called uterus didelphys with a septate vagina, to the simple uterus arcuatus, which has nothing more abnormal than an indentation in the fundal portion. According to different observations, uterine anomalies have an incidence of 1 in 200-600 women [6]. Uterus didelphys is found in 1 in every 2000 women. Uterine malformations have clinical importance because of the many obstetrical complications that can take place in the pregnant patient [4,8]. According to Green
Correspondence: N. Kanakas, 52 Kifissias Avenue, GR - 11526 Athens, Greece. * Supported by a Scholarship of the Deutscher Akademischer Austauschdienst (DAAD, Bonn).
0028-2243/89/$03.50
0 1989 Elsevier Science Publishers B.V. (Biomedical Division)
288
and Harris [4] pregnancies in a functional hemi-uterus originating from a single Mullerian duct (one horn of a uterus didelphys, unicornuate unicollis) have a better prognosis with regard to the fetal wastage rate than a pregnancy in a uterus bicornuate, septate or arcuatus. Though the delivery of a normal infant, and very rarely twins, is possible in a patient with uterus didelphys [1,3], the usual history is one of abortion or premature labour. Patients with uterus didelphys belong to a high-risk group and deserve a particular prenatal care. Therefore it is of great importance for the clinical management of these cases that abnormalities of the reproductive tract are detected in an early stage. In the study of uterine anomalies, ultrasound is used which is as’reliable as laparoscopy or hysterosalpingography [2]. A caJe of a twin pregnancy in the same horn of a uterus didelphys with delivery of two male infants by Cesarean section, and especially the use of the transvaginal ultrasonography in identifying the twin pregnancy and the uterine abnormality in a very early stage of development, is reported. Case report Mrs. G.B. (0 para, I gravida) a 29-year-old was first examined in the obstetrical department of the University of Heidelberg on 8 December 1986, in the 7 + 5 week
Fig. 1. Transabdominal sonography (full-bladder technique). Uterus didelphys with an obscure gestational sac (diameter 3 cm, 7 + 5 week) in the right horn (Kretz Combison 320, sector scanner 5 MHz).
289
Fig. 2. Tramvaginal sonography uterus didelphys (7 + 5 wk) with a twin pregnancy in the right horn. Both embryos are clearly visible in the same gestational sac CRLl = 8 mm, CRL2 = 10 mm (Kretz Combison 320, panorama vaginal sector scanner 240) Cardiac action was detected.
of her first pregnancy. The last menstrual period was October 15, 1986. Using the full bladder technique, the transabdominal ultrasound examination with the Sector scanner Combison 320 (KRETZ technik, 5 MHz) revealed the uterine malformation of an uterus didelphys. The gynecological examination showed a double cervix and a septum vaginae. Furthermore, the transabdominal sonographic examination on the 8th of December demonstrated one obscure gestational sac with a diameter of 3 cm in the right horn of the uterus didelphys but not the embryos or their cardiac action (Fig. 1). Only the transvaginal ultrasound examination revealed very clearly a twin pregnancy with both embryos having cardiac actions in the same gestational sac (Fig. 2). During the transvaginal ultrasound examination, a full bladder was not necessary (Combison 320, Panorama 5 MHz Sector scanner 240, KRETZtechnik, Austria). One week later (15.12.86) on the 8 + 5 gestational week the transvaginal sonography showed further normal development of the twin pregnancy and both embryos with their yolk sacs were clearly detected (Fig. 3). The endocrinological monitoring of the pregnancy showed normal /?-HCG values of 18389 mE/ml in Serum (normal values: 12900-95 000) and progesteron 19.4 ng/ml (normal values: 12-32 ng/ml) and estradiol 1.1 ng/ml.
Fig. 3. Transvaginal sonography: Twin pregnancy in the right horn of the uterus didelphys 1 week later (8 + 5 week). Both yolk sacs and embryos are visible (Kretz Combison 320, panorama vaginal sector scanner 240).
The patient attended our clinic once weekly as a high-risk pregnancy, /3-HCG, estradiol and progesterone in serum were determined and a transabdominal ultrasound examination was made. Between the 10th and 12th week the patient was admitted to the clinic because of bleeding, with the diagnosis of abortus imminens although hemorrhage could be from the nonpregnant uterus caused by decidual reaction [6]. In the 15 + 1 week the vaginal septum was resected and a prophylactic cervical cerclage was made in the right cervix. Hormone determinations ranged in the normal values for each week and were measured until the 17th week. Ultrasound scanning of the diamniotic twin pregnancy showed a discrepancy in the development of the two fetuses from the 20th week of the gestation onwards. Successive ultrasound examinations in the 22nd and 24th week also showed developmental discrepancy and an intra-uterine growth retardation of 14 days of one fetus. The patient was admitted to our clinic, on the 24th week (1.4.87) because of premature contractions, and intravenous tocolytic agents (Partusisten) were administered until the 33rd week. Identification of the lecithin/sphingomyelin ratio in the amniotic fluid by amniocentesis (2.6.87) was 5.3, showing lung maturation. On the 4th of June (34th week) a Cesarean section, using a lower-segment transverse incision was performed with delivery of a 1830 g male infant scoring l/8/8 on the Apgar scale
and a 1340 g also male infant scoring 4/S/8 on the Apgar scale, on the lst, 5th and 10th minute, respectively. The first infant had a vertex (occiput) presentation while the second a breech presentation. The indications for the Cesarean section were a pathological CTG (cardiotocogram) and uterine contractions, in spite of long administration of the maximum dose tocolysis with 4 pg per minute. Both infants were transferred to the neonatologic department for further care. One month later, the paediatric department reported that both infants were absolutely healthy. Discussion Congenital defects of the reproductive tract are often associated with an increase in breech presentation, great liability for premature labour, abnormal presentation with dystocia, and the necessity for Cesarean section [4]. On the other hand, multiple pregnancies are always regarded as high-risk pregnancies [lo]. Neonatal findings and complications include low Apgar scores, small-for-date infants, hyaline membrane disease, and an increased incidence of deaths and morbidity in twin pregnancies as compared to singleton pregnancies. The recent trend for the delivery of multiple fetuses has been to use Cesarean section more frequently than in the past [12]. Twin pregnancies in the same horn of a uterus didelphys are a very rare phenomenon, and women that have such pregnancies belong to a high risk group and deserve meticulous prenatal care. Although a normal pregnancy is possible, the majority of uterine anomalies produce a considerably lower percentage of viable babies. As has already been mentioned, a pregnancy in a functional hemiuterus has a better prognosis with regard to the fetal survival rate than a pregnancy in a uterus bicomuate, septate or arcuatus [4]. In spite of this, the incidence of breech presentation and prematurity are higher in the first group. In our case report, after a long administration of tocolysis, both infants (breech and vertex presentations) were delivered by Cesarean section in the 34th week. The early detection of uterine anomalies is of great importance because of the increased obstetric complications. Sonography has been reported to be useful in identifying abnormal uterine development in most of the cases [7]. Transvaginal sonography offers a new reliable diagnostic method in predicting uterine anomalies in the very early stages of pregnancy. Transvaginal sonography is already an established method in gynecology, and especially in IVF programs as well as in obstetrics in the first trimester [5,9,11]. In the above mentioned case report, using the transvaginal sonography, it was possible to detect a uterus didelphys with a viable twin pregnancy in the right horn at a very early stage of development. As twin pregnancies associated with uterine malformations are certainly of special interest, due to the obstetric complications, we believe that early detection with the use of transvaginal sonography is of great importance for dealing with these high-risk pregnancies in the future. References 1
Brody
S. (1954)
2 Fedele diagnosis
L,
Double
Ferrazzi of ‘double’
E,
uterus with double pregnancy. Dorta
M, Vercellini
uteri. Fertil
Am J Obstet Gynecol
P, Candiani
Steril 1988;50:361-364.
G.
Ultrasonography
1954;67:161-167. in the differential
292 3 Getts JS. Double pregnancy in a uterus duplex unicollis. Am J Obstet Gynecol 1967;97:277-278. 4 Green LK, Harris RE. Uterine anomalies; frequency of diagnosis and associated obstetric complications. Obstet Gynecol 1976;47:427-429. 5 Haid C, Zech H, Martin J. Verbesserte Friihdiagnose der intrauterinen Schwangerschaft durch Ultraschall - Vaginalsonde. Geburtsh Frauenheilk 1985;45:371-374. 6 Heinonen PK. Uterus didelphys: a report of 26 cases. Eur J Obstet Gynecol Reprod Biol 1984;17:345-350. 7 Hochner-Celnikier D, Hunvita A, Beller U, Milwidsky A, Yagel S. Case report; uterus didelphys. Ultrasound diagnosis in the case of an adnexal mass as a presenting symptom in early pregnancy. Eur J Obstet Gynecol Reprod Biol 1984;16:339-342. 8 Jones HW Jr, Delfs E, Jones GES. Reproductive difficulties in double uterus. Am J Obstet Gynecol 1956;72:865-883. 9 Kanakas N, Boos R, Schmidt W. Vaginosonographische Follikulometrie. Ultraschall Klin Prax 1988;3:13-18. 10 Kuen Ho S, Wu PYK. Perinatal factors and neonatal morbidity in twin pregnancy. Am J Obstet Gynecol 1975;122:979-987. 11 Rempen A. Vaginale Sonographie der intakten Graviditaet im ersten Trimenon. Geburtsh Frauenheilk 1987;47:477-482. 12 Pritchard J, MacDonald P, Gant NF. (eds) Williams Obstetrics, 17th edn, Abnormalities of the reproductive tract. Norwalk CN: Appleton, Century, Crofts, 1985:494-497.
mo
Biology, 32 (1989) 287-292
287
00797
Twin pregnancy in the right horn of a uterus didelphys: a case report N. Kanakas Department
*, R. Boos and W. Schmidt
of Obstetrics and Gynecology, University of Heidelberg, F. R. G Accepted for publication 9 December 1988
Summary A rare case of a twin pregnancy occurring in the right horn of a uterus didelphys (double uterus, double cervix and septate vagina) is reported. The occasional diagnosis of this uterine anomaly was made after the pregnancy was detected. Both embryos, with cardiac actions which proved the viability of the gestation from the very early stage of pregnancy, were detected by transvaginal ultrasonography. Two male infants were delivered by Cesarean section in the 34th week. The importance of the transvaginal sonography and the handling of the case during this high-risk pregnancy are reported. Uterus didelphys; Twin pregnancy; Transvaginal sonography
Introduction Fusion of the two Mullerian ducts and the establishment of the vaginal canal are usually completed between the 10th and 17th week of intra-uterine development. It is obvious that a variety of malformations may appear. Several degrees of duplication of the uterus are possible, ranging from a complete duplication of the uterus, cervix and vaginal canal, such as the so-called uterus didelphys with a septate vagina, to the simple uterus arcuatus, which has nothing more abnormal than an indentation in the fundal portion. According to different observations, uterine anomalies have an incidence of 1 in 200-600 women [6]. Uterus didelphys is found in 1 in every 2000 women. Uterine malformations have clinical importance because of the many obstetrical complications that can take place in the pregnant patient [4,8]. According to Green
Correspondence: N. Kanakas, 52 Kifissias Avenue, GR - 11526 Athens, Greece. * Supported by a Scholarship of the Deutscher Akademischer Austauschdienst (DAAD, Bonn).
0028-2243/89/$03.50
0 1989 Elsevier Science Publishers B.V. (Biomedical Division)
288
and Harris [4] pregnancies in a functional hemi-uterus originating from a single Mullerian duct (one horn of a uterus didelphys, unicornuate unicollis) have a better prognosis with regard to the fetal wastage rate than a pregnancy in a uterus bicornuate, septate or arcuatus. Though the delivery of a normal infant, and very rarely twins, is possible in a patient with uterus didelphys [1,3], the usual history is one of abortion or premature labour. Patients with uterus didelphys belong to a high-risk group and deserve a particular prenatal care. Therefore it is of great importance for the clinical management of these cases that abnormalities of the reproductive tract are detected in an early stage. In the study of uterine anomalies, ultrasound is used which is as’reliable as laparoscopy or hysterosalpingography [2]. A caJe of a twin pregnancy in the same horn of a uterus didelphys with delivery of two male infants by Cesarean section, and especially the use of the transvaginal ultrasonography in identifying the twin pregnancy and the uterine abnormality in a very early stage of development, is reported. Case report Mrs. G.B. (0 para, I gravida) a 29-year-old was first examined in the obstetrical department of the University of Heidelberg on 8 December 1986, in the 7 + 5 week
Fig. 1. Transabdominal sonography (full-bladder technique). Uterus didelphys with an obscure gestational sac (diameter 3 cm, 7 + 5 week) in the right horn (Kretz Combison 320, sector scanner 5 MHz).
289
Fig. 2. Tramvaginal sonography uterus didelphys (7 + 5 wk) with a twin pregnancy in the right horn. Both embryos are clearly visible in the same gestational sac CRLl = 8 mm, CRL2 = 10 mm (Kretz Combison 320, panorama vaginal sector scanner 240) Cardiac action was detected.
of her first pregnancy. The last menstrual period was October 15, 1986. Using the full bladder technique, the transabdominal ultrasound examination with the Sector scanner Combison 320 (KRETZ technik, 5 MHz) revealed the uterine malformation of an uterus didelphys. The gynecological examination showed a double cervix and a septum vaginae. Furthermore, the transabdominal sonographic examination on the 8th of December demonstrated one obscure gestational sac with a diameter of 3 cm in the right horn of the uterus didelphys but not the embryos or their cardiac action (Fig. 1). Only the transvaginal ultrasound examination revealed very clearly a twin pregnancy with both embryos having cardiac actions in the same gestational sac (Fig. 2). During the transvaginal ultrasound examination, a full bladder was not necessary (Combison 320, Panorama 5 MHz Sector scanner 240, KRETZtechnik, Austria). One week later (15.12.86) on the 8 + 5 gestational week the transvaginal sonography showed further normal development of the twin pregnancy and both embryos with their yolk sacs were clearly detected (Fig. 3). The endocrinological monitoring of the pregnancy showed normal /?-HCG values of 18389 mE/ml in Serum (normal values: 12900-95 000) and progesteron 19.4 ng/ml (normal values: 12-32 ng/ml) and estradiol 1.1 ng/ml.
Fig. 3. Transvaginal sonography: Twin pregnancy in the right horn of the uterus didelphys 1 week later (8 + 5 week). Both yolk sacs and embryos are visible (Kretz Combison 320, panorama vaginal sector scanner 240).
The patient attended our clinic once weekly as a high-risk pregnancy, /3-HCG, estradiol and progesterone in serum were determined and a transabdominal ultrasound examination was made. Between the 10th and 12th week the patient was admitted to the clinic because of bleeding, with the diagnosis of abortus imminens although hemorrhage could be from the nonpregnant uterus caused by decidual reaction [6]. In the 15 + 1 week the vaginal septum was resected and a prophylactic cervical cerclage was made in the right cervix. Hormone determinations ranged in the normal values for each week and were measured until the 17th week. Ultrasound scanning of the diamniotic twin pregnancy showed a discrepancy in the development of the two fetuses from the 20th week of the gestation onwards. Successive ultrasound examinations in the 22nd and 24th week also showed developmental discrepancy and an intra-uterine growth retardation of 14 days of one fetus. The patient was admitted to our clinic, on the 24th week (1.4.87) because of premature contractions, and intravenous tocolytic agents (Partusisten) were administered until the 33rd week. Identification of the lecithin/sphingomyelin ratio in the amniotic fluid by amniocentesis (2.6.87) was 5.3, showing lung maturation. On the 4th of June (34th week) a Cesarean section, using a lower-segment transverse incision was performed with delivery of a 1830 g male infant scoring l/8/8 on the Apgar scale
and a 1340 g also male infant scoring 4/S/8 on the Apgar scale, on the lst, 5th and 10th minute, respectively. The first infant had a vertex (occiput) presentation while the second a breech presentation. The indications for the Cesarean section were a pathological CTG (cardiotocogram) and uterine contractions, in spite of long administration of the maximum dose tocolysis with 4 pg per minute. Both infants were transferred to the neonatologic department for further care. One month later, the paediatric department reported that both infants were absolutely healthy. Discussion Congenital defects of the reproductive tract are often associated with an increase in breech presentation, great liability for premature labour, abnormal presentation with dystocia, and the necessity for Cesarean section [4]. On the other hand, multiple pregnancies are always regarded as high-risk pregnancies [lo]. Neonatal findings and complications include low Apgar scores, small-for-date infants, hyaline membrane disease, and an increased incidence of deaths and morbidity in twin pregnancies as compared to singleton pregnancies. The recent trend for the delivery of multiple fetuses has been to use Cesarean section more frequently than in the past [12]. Twin pregnancies in the same horn of a uterus didelphys are a very rare phenomenon, and women that have such pregnancies belong to a high risk group and deserve meticulous prenatal care. Although a normal pregnancy is possible, the majority of uterine anomalies produce a considerably lower percentage of viable babies. As has already been mentioned, a pregnancy in a functional hemiuterus has a better prognosis with regard to the fetal survival rate than a pregnancy in a uterus bicomuate, septate or arcuatus [4]. In spite of this, the incidence of breech presentation and prematurity are higher in the first group. In our case report, after a long administration of tocolysis, both infants (breech and vertex presentations) were delivered by Cesarean section in the 34th week. The early detection of uterine anomalies is of great importance because of the increased obstetric complications. Sonography has been reported to be useful in identifying abnormal uterine development in most of the cases [7]. Transvaginal sonography offers a new reliable diagnostic method in predicting uterine anomalies in the very early stages of pregnancy. Transvaginal sonography is already an established method in gynecology, and especially in IVF programs as well as in obstetrics in the first trimester [5,9,11]. In the above mentioned case report, using the transvaginal sonography, it was possible to detect a uterus didelphys with a viable twin pregnancy in the right horn at a very early stage of development. As twin pregnancies associated with uterine malformations are certainly of special interest, due to the obstetric complications, we believe that early detection with the use of transvaginal sonography is of great importance for dealing with these high-risk pregnancies in the future. References 1
Brody
S. (1954)
2 Fedele diagnosis
L,
Double
Ferrazzi of ‘double’
E,
uterus with double pregnancy. Dorta
M, Vercellini
uteri. Fertil
Am J Obstet Gynecol
P, Candiani
Steril 1988;50:361-364.
G.
Ultrasonography
1954;67:161-167. in the differential
292 3 Getts JS. Double pregnancy in a uterus duplex unicollis. Am J Obstet Gynecol 1967;97:277-278. 4 Green LK, Harris RE. Uterine anomalies; frequency of diagnosis and associated obstetric complications. Obstet Gynecol 1976;47:427-429. 5 Haid C, Zech H, Martin J. Verbesserte Friihdiagnose der intrauterinen Schwangerschaft durch Ultraschall - Vaginalsonde. Geburtsh Frauenheilk 1985;45:371-374. 6 Heinonen PK. Uterus didelphys: a report of 26 cases. Eur J Obstet Gynecol Reprod Biol 1984;17:345-350. 7 Hochner-Celnikier D, Hunvita A, Beller U, Milwidsky A, Yagel S. Case report; uterus didelphys. Ultrasound diagnosis in the case of an adnexal mass as a presenting symptom in early pregnancy. Eur J Obstet Gynecol Reprod Biol 1984;16:339-342. 8 Jones HW Jr, Delfs E, Jones GES. Reproductive difficulties in double uterus. Am J Obstet Gynecol 1956;72:865-883. 9 Kanakas N, Boos R, Schmidt W. Vaginosonographische Follikulometrie. Ultraschall Klin Prax 1988;3:13-18. 10 Kuen Ho S, Wu PYK. Perinatal factors and neonatal morbidity in twin pregnancy. Am J Obstet Gynecol 1975;122:979-987. 11 Rempen A. Vaginale Sonographie der intakten Graviditaet im ersten Trimenon. Geburtsh Frauenheilk 1987;47:477-482. 12 Pritchard J, MacDonald P, Gant NF. (eds) Williams Obstetrics, 17th edn, Abnormalities of the reproductive tract. Norwalk CN: Appleton, Century, Crofts, 1985:494-497.