Müllerian duct anomalies (MDAs) encompass congenital disabilities in the female reproductive tract. Didelphic uterus, a rare MDA occurring in 1/3000 women and 11% with Müllerian anomalies, results from incomplete fusion, leading to two hemi uteri, two cervices, and often two vaginas or a longitudinal vaginal septum. Associated defects may extend to the renal system, vagina, and skeleton. Diagnosis, challenging and typically made during the reproductive period, involves symptoms like amenorrhea, dysmenorrhea, dyspareunia, pelvic pain, and obstetric complications, including recurrent pregnancy loss, premature delivery, malpresentation, intrauterine growth restriction (IUGR), placental abruption, and cervical insufficiency. Rarely, pelvic organ prolapse (POP) occurs, with the chance of a didelphic uterus presenting as POP in one uterus and pregnancy in the other being an exceedingly rare event, with only one reported case. 2,3
This article delves into a rare case where doctors found women had a second set of reproductive organs during C-section, in which one uterus carried a pregnancy while the other prolapsed, with a successful pregnancy outcome resulting in a alive-term delivery.1
Case 1
A 29-year-old mother, at 38w6d from LNMP, presented with a protruding mass per vagina persisting for 14 days, accompanied by lower abdominal pain and foul-smelling vaginal discharge. Despite second-trimester antenatal care at a nearby health center, no hospital or senior obstetrician evaluation occurred. Vital signs were stable, and obstetric abdominal palpations revealed a fundal height corresponding to 36 weeks, longitudinal lie, cephalic position, and a positive FHB (Fetal heartbeat) of 144 without uterine contractions. Genitourinary examination exposed a protruding mass per vagina measuring 5 × 7 cm, presenting a pink color, along with an ulcer and a whitish foul-smelling discharge. Upon palpation, the mass exhibited tenderness and irreducibility.
Figure 1. (a) A gravid uterus, POP (infected and ulcerated) and (b) focused view of the ulcerated POP.
Medical Evaluation 1
A blood workup revealed a WBC count of 12,790/mm³, hemoglobin 10.5 g/dL, hematocrit 32.3%, and platelet count 306,000 per microliter. Liver enzymes and renal function tests were within normal limits. Ultrasound findings indicated a singleton intrauterine pregnancy, cephalic position, aggregate gestational age of 36 weeks, and an estimated fetal weight of 2500 g.
Figure 2. (a) Intraoperative picture of the posterior view of the double uterus; (b) an anterior view of the double uterus with their tube and ovary, and the right uterus with a repaired cesarean incision.
Figure 3. Speculum examination showing two cervices and negative for VIA.
Figure 4. Two-dimensional ultrasound showing two uteruses and cervices.
Management 1
Admitted with a diagnosis of late-preterm pregnancy and stage 3 POP with an infected decubitus ulcer, the patient received therapeutic antibiotics, wound care, and a single course of corticosteroid for fetal lung maturity. With management, the prolapse gradually reduced, and on the 10th day, no mass was visible externally. On the 12th day, she entered the active phase of the first stage of labor at term and underwent an emergency cesarean section, resulting in the delivery of a 3000-gram male neonate with an APGAR score of 8 & 9. Two uteruses were identified intraoperatively, one gravid and the other not, with normal kidneys.
Discussion 4,5
MDAs affect 0.1%–3% of women, with the didelphic uterus accounting for 11% and typically remaining asymptomatic. Diagnostic tools include 2D transvaginal sonography, hysterosalpingography, 3D pelvic ultrasound, magnetic resonance imaging, and combined laparoscopy and hysteroscopy. Didelphys uterus has been linked to higher rates of infertility, spontaneous miscarriage, IUGR, and postpartum hemorrhage, but most women have good reproductive outcomes. Delivery mode for pregnant patients with MDAs varies, with 82% opting for CS. Despite the potential for complications like inert labor and uterine rupture, successful labor reports exist. Patients with uterine didelphys and POP may undergo various surgical treatments for improvement, with consideration for anatomical and symptomatic cure rates.
Conclusion 1-5
The occurrence of a didelphys uterus is a rare medical condition that requires an individualized approach to management. This approach should consider factors such as symptomatology, severity of prolapse, degree of obstruction, and patient preferences. The rarity of a didelphic uterus presenting as pelvic organ prolapse in one uterus and pregnancy in the other emphasizes the need for personalized and expert-guided treatment decisions, as exemplified in this unique case. It is important to note that this medical condition is extremely uncommon and requires a careful evaluation by a medical professional before deciding on the appropriate treatment plan. Therefore, it is crucial to seek the advice of a specialist in the field who can provide expert guidance on managing this condition effectively while considering the patient’s unique circumstances.
References
- Amanuel Admasu Yayna, Adane Ayza, Wokil Wolde Dana, et al. A rare case report of uterine didelphys, in which one uterus carried a pregnancy while the other prolapsed, with a successful pregnancy outcome resulting in an alive-term delivery. SAGE Open Medical Case Reports. 2023 Jan 1;11:2050313X2311595–5.
- Akhtar MA, Saravelos SH, Li TC, et al. Reproductive implications and management of congenital uterine anomalies: scientific impact paper No. 62 November 2019. BJOG 2020; 127(5): e1–e13.
- Moore KL, Persaud TVN, Torchia MG. Before we are born: essentials of embryology and birth defects. Philadelphia, PA: Saunders/Elsevier, 2013.
- Stearns K, Al Khabbaz A. Bicornuate bicollis uterus with obstruction of the lower uterine segment and cervical prolapse complicating pregnancy. Case Rep Obstet Gynecol 2018; 2018: 8910976.
- Rajamaheswari N, Seethalakshmi K, Gayathri KB. Case of longitudinal vaginal septum with pelvic organ prolapse. Int Urogynecol J Pelvic Floor Dysfunct 2009; 20(12): 1509–1510.