Keywords
uterine didelphys, pregnancy, caesarean section, breech presentation
uterine didelphys, pregnancy, caesarean section, breech presentation
Müllerian duct abnormalities (MDA) are a spectrum of genital abnormalities arising from failure of fusion of the Müllerian ducts during the embryological stage of development.1 The prevalence of congenital uterine anomalies varies and can range between 5.5% in the general population and 24.5% in patients treated for miscarriages and infertility.2 The most common forms of uterine anomalies include unicornuate uterus, bicornuate uterus, arcuate uterus, septate uterus and didelphys uterus. Uterine didelphys belongs to MDA class III as defined by American Society of Reproductive Medicine (ASRM). This classification includes adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Müllerian anomalies and intrauterine adhesions that result from failure of Müllerian duct fusion at week eight of gestation.3,4 Though uterine didelphys rarely affects fertility, it is commonly associated with adverse pregnancy outcomes such as recurrent miscarriages, preterm deliveries, intrauterine growth-restricted fetuses, low-birth-weight fetuses, and fetal malpresentations. While many cases will remain undiagnosed until evaluations for other gynaecological conditions,5,6 fetal malpresentation diagnosed during pregnancy or at delivery is one of the commonest indications for Caesarean delivery.7 We present a case of Caesarean section in a multiparous woman with uterine didelphys with breech presentation at term in our obstetric service.
A 36-year-old G4P1+2 1 alive Nigerian of Yoruba ethnicity, presented for antenatal service following spontaneous conception of singleton gestation at gestational age of 17 weeks. She was the third of her parents’ four children, including three females and one male. The older two sister were multiparous with no significant obstetric complaints. Obstetrics scan done at 23 weeks revealed live singleton fetus in breech presentation within the right horn and empty left horn of the uterus (Figure 1). She was regular at clinic visits and her pregnancy period remained uneventful. Four years earlier, she had a vaginal delivery, at term, of a live neonate with a birth weight of 2.95 kg following spontaneous conception after two prior spontaneous pregnancies loss at about six weeks gestation each.
Review at the antenatal clinic at 37 weeks revealed fetal cephalic presentation at clinical examination. However, at 38 weeks and four days of gestation she presented in labour and obstetrics examination revealed a singleton fetus in longitudinal lie with footling breech presentation. She subsequently had emergency lower segment Caesarean section and was delivered of a live neonate with a birth weight of 3.75 kg via breech extraction from the right horn, the left horn was bulky and equivalent to about 14 weeks size gestation (Figure 2). Each horn had its adjoining normal looking fallopian tube and ovary. The urinary bladder was grossly normal. Her postoperative period was satisfactory and she was discharged on fourth day after surgery.
Congenital malformations of the female genital tract are embryological maldevelopments of the Müllerian or paramesonephric ducts.8,9 The prevalence of genital tract malformations has been reported to vary between 6.0-38%.9,10 In the general population it was reported to range between 5-7%, 7-8% in the infertile population and 16-25% in the recurrent miscarriage and infertile population.1,8,11 Malformations involving the uterus have been reported as the most common MDA with septate uterus being the commonest, and didelphys uterus the rarest.12 In a 15-year retrospective analysis, septate uterus were found in 55.6% of cases of female genital tract anomaly, while uterine didelphys accounted for 22.7% of cases.13 Presentations of genital tract anomalies also vary. In paediatric patients, presentation to emergency services with symptoms of severe abdominal pain, likely associated dysmenorrhea if menarche has been reached, obstructed hemivaginal septa, and abdominal swelling from haematometra with or without haematocolpos, are common.1,14
Congenital malformation of the female genital tract is of special interest in the practice of obstetrics as the obstetrics performance is determined by the type of MDA. Some authors have reported increased risk of first- and second-trimester miscarriages, preterm birth, low birth weight, fetal mal-presentation, and ultimately abdominal delivery with uterine didelphys.15,16 However, there have been many reported cases of successful pregnancies and deliveries in patients with uterine didelphys following appropriate pre-pregnancy surgical correction and appropriate obstetrics interventions like cervical cerclage.1,17 In a prospective multicenter study of 286 pregnant women with uterine didelphys conducted in Saudi Arabia, 5.2% of the women had first trimester abortion, 27.5% had preterm labour which were managed conservatively with all delivering at term and of all delivering at term, 5.9% had spontaneous vaginal delivery, 13.3% had operative vaginal delivery and 80.8% of the women delivered by Caesarean section.18 Our patient had two previous miscarriages prior to her first delivery.
Delivery by Caesarean section is, generally, not primarily indicated in cases of uterine didelphys except for additional obstetric indication.19 In our patient, she had successful vaginal delivery in her previous pregnancy and had Caesarean delivery in index case because of the fetal footling breech presentation at term.
Didelphys uterus is among the rarest of Müllerian duct anomalies and is commonly associated with successful pregnancies outcomes. Many will deliver vaginally and remain undiagnosed, as was the case for the first term pregnancy of the case we presented. However, its presence can be suspected in pregnant women with prior vaginal deliveries now presenting with malpresentations, like persistent fetal breech presentation as seen in this patient. Good antenatal care and management in a facility with adequate obstetric facilities for surgical intervention can prevent complications that can result from undiagnosed cases. This is especially important for cases showing malpresentations as seen in this case of fetal footling breech presentation.
All data underlying the results are available as part of the article and no additional source data are required.
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Is the background of the case’s history and progression described in sufficient detail?
Partly
Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?
Partly
Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?
Partly
Is the case presented with sufficient detail to be useful for other practitioners?
Partly
References
1. Ćwiertnia A, Borzyszkowska D, Golara A, Tuczyńska N, et al.: The Impact of Uterus Didelphys on Fertility and Pregnancy. International Journal of Environmental Research and Public Health. 2022; 19 (17). Publisher Full TextCompeting Interests: No competing interests were disclosed.
Reviewer Expertise: perinatal medicine
Is the background of the case’s history and progression described in sufficient detail?
Yes
Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?
Yes
Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?
Yes
Is the case presented with sufficient detail to be useful for other practitioners?
Yes
Competing Interests: No competing interests were disclosed.
Reviewer Expertise: Gynaecological oncology
Alongside their report, reviewers assign a status to the article:
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Version 1 13 Jul 22 |
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