Uterine didelphys presenting as pelvic organ prolapse and second trimester uterine rupture: a case series and literature review.

Case 1: A 26-year-old primigravida, married, with no formal employment, and living in an urban setting, presented to our hospital with abdominal pain and vaginal bleeding. Her last normal menstrual period was unknown, but she had been amenorrheic for approximately 4 months. The pregnancy was unplanned and initially unwanted but later supported; she had not yet initiated antenatal care. She first noticed spotty vaginal bleeding followed by progressively increasing lower abdominal pain over the subsequent 24 h. In the hours immediately preceding admission, she developed blurring of vision, lightheadedness, and vertigo, prompting her family to bring her to our hospital when her condition worsened. She denied any history of abdominopelvic surgery, abnormal vaginal discharge or treatment of pelvic inflammatory disease, or contraceptive use. She also had no history of chronic medical illness (e.g., asthma, hypertension, or cardiac disease), and she did not smoke or consume alcohol. On examination, she appeared acutely ill and pale. Her blood pressure was 90/60 mm Hg, her pulse rate was 110 beats per minute, and she was afebrile. Abdominal examination revealed tenderness on deep palpation below the umbilicus, with no palpable mass or obvious fluid collection. Pelvic examination showed cervical motion tenderness, a tender posterior fornix, and blood on the examination finger; the cervix was closed, and bimanual examination suggested a 16-week-sized uterus. Initial bedside abdominopelvic ultrasonography was performed by a general practitioner in the emergency department. The scan demonstrated a live intrauterine fetus at approximately 17 weeks’ gestation and free fluid in the pelvic cul-de-sac and paracolic gutters, with probe tenderness. A solid right adnexal mass was also noted, which was later intraoperatively identified as the contralateral, nongravid uterine horn. Consultation was made with the on-duty gynecologist for further evaluation while on resuscitation. The laboratory at that time showed a hemoglobin of 9 g/dL, with other hematologic parameters within the normal range. On reassessment by the gynecologist after 2 h, the impression remained the same, but repeat ultrasound demonstrated increasing intra-abdominal fluid, and repeat hemoglobin was 8 g/dL. In view of a live intrauterine pregnancy, solid right adnexal mass, and hemoperitoneum, a ruptured ectopic pregnancy was considered in the differential diagnosis. Given the worsening abdominal pain, increasing intra-abdominal collection, and a falling hemoglobin level, an exploratory laparotomy was indicated despite the uncertain preoperative diagnosis. Emergency exploratory laparotomy was performed after 3 h by a senior obstetrician and gynecologist. Intraoperative findings revealed approximately 1,500 mL of hemoperitoneum was evacuated, and the fetus with placenta was found within the peritoneal cavity. Further exploration showed a didelphys uterus with rupture of the fundal region of the left uterine horn. The rupture site was ragged and irregular, extending into the broad ligament with an expanding hematoma and active bleeding. In view of uncontrolled hemorrhage, distorted anatomy, and intraoperative hemodynamic instability, a supracervical hysterectomy of the ruptured left horn was performed (Fig. 1 ). Hemostasis was secured; intraoperative resuscitation included rapid infusion of crystalloid and blood transfusion. After the count of materials was confirmed correct, the abdomen was closed in layers, and the patient was transferred to recovery. Figure 1. Intraoperative specimen following supracervical hemihysterectomy of the ruptured left uterine horn in a patient with uterus didelphys. The expelled fetus (indicated by the arrow), approximately 17 weeks of gestation, is shown adjacent to the resected ruptured uterine horn (marked with a star), with the placenta and umbilical cord still attached. This specimen illustrates the catastrophic nature of second-trimester rupture in a malformed uterine horn. Intraoperative specimen following supracervical hemihysterectomy of the ruptured left uterine horn in a patient with uterus didelphys. The expelled fetus (indicated by the arrow), approximately 17 weeks of gestation, is shown adjacent to the resected ruptured uterine horn (marked with a star), with the placenta and umbilical cord still attached. This specimen illustrates the catastrophic nature of second-trimester rupture in a malformed uterine horn. Postoperatively, the patient stabilized, and her hemoglobin improved following transfusion. Because the right uterine horn was preserved, she received postoperative fertility counseling. She was informed that future conception remains possible; however, such pregnancy carries increased risks – including malpresentation, preterm birth, and recurrent uterine rupture. She was advised to seek early preconception consultation, undergo a first-trimester ultrasound to confirm the implantation site and uterine anatomy, and plan delivery in a facility capable of performing emergency cesarean sections. Her postoperative course was otherwise uneventful, and she was discharged on the third postoperative day in stable condition with oral iron therapy. Case 2: A 50-year-old para 5, abortion 4 woman, who had been amenorrheic for 5 years, presented with a protruding mass per vagina of 5 years’ duration. The mass had gradually increased in size and was associated with urinary difficulties, including dribbling and incomplete emptying unless the mass was manually reduced. She also reported a sensation of pelvic heaviness and constipation. All her deliveries had been vaginal, conducted at home, with prolonged labor reported in some. She is a housewife whose daily life involved strenuous physical activity such as farming and carrying water from a distant river. She had no history of chronic medical illness (e.g., asthma, chronic obstructive pulmonary disease, cardiac disease, hypertension, and diabetes mellitus), pelvic surgery, smoking, or instrumental deliveries. On examination, her vital signs were in the normal range. The abdominal exam was unremarkable, with no tenderness, palpable mass, or signs of fluid collection. A genitourinary exam revealed a visible bulge protruding beyond the hymenal ring on the Valsalva maneuver. The cervix was noted at approximately +3 cm relative to the hymen. The anterior and posterior vaginal walls were also lax but less prominent than the uterine descent. POP-Q measurements were Aa: +1 cm, Ba: +2 cm, C: +3 cm, Ap: 0 cm, Bp: 0 cm, D: +1 cm, genital hiatus: 4 cm, perineal body: 2.5 cm, and total vaginal length: 8 cm. No ulcerative lesion or signs of infection were seen. A clinical diagnosis of third-degree uterovaginal prolapse (UVP) was made. Preoperative laboratory evaluations, including complete blood count, liver, and renal function tests, were within normal limits. Abdominopelvic ultrasound confirmed a uterus without additional masses. As the patient was symptomatic, she was counseled for definitive management and consented to transvaginal hysterectomy with anterior colporrhaphy. Under spinal anesthesia, vaginal hysterectomy was initiated by an obstetrician and gynecologist. Following anterior colpotomy and entry into the posterior peritoneum, unexpected difficulty was encountered in mobilizing the uterus, as the surgical plane appeared distorted and a firm proximal mass limited descent of the uterus. The uterosacral ligament, cardinal ligament, and uterine artery were then subsequently clamped, ligated, and cut. At this stage, the uterus was noted to be widened and divided into two horns. Each horn was attached to its own infundibulopelvic ligament, which was apparently ligated, transfixed, and cut. Hemostasis was secured, and the procedure was completed with anterior colporrhaphy (Fig. 2 ). Figure 2. Intraoperative specimen following vaginal hysterectomy for third-degree uterovaginal prolapse in a 50-year-old multiparous woman, demonstrating uterus didelphys. The specimen shows complete duplication of the uterine horns and cervices. The right uterine horn and its corresponding cervix (indicated by the arrow) are seen on one side, while the left uterine horn and cervix (marked with a star) are visible on the opposite side. This rare congenital anomaly illustrates how Müllerian duct malformations may remain unrecognized until surgical exploration reveals duplicated uterine anatomy. Intraoperative specimen following vaginal hysterectomy for third-degree uterovaginal prolapse in a 50-year-old multiparous woman, demonstrating uterus didelphys. The specimen shows complete duplication of the uterine horns and cervices. The right uterine horn and its corresponding cervix (indicated by the arrow) are seen on one side, while the left uterine horn and cervix (marked with a star) are visible on the opposite side. This rare congenital anomaly illustrates how Müllerian duct malformations may remain unrecognized until surgical exploration reveals duplicated uterine anatomy. The patient remained stable throughout surgery and was transferred to recovery with normal vital signs. Postoperatively, her course was uneventful. She was discharged on the third postoperative day and was well at her 1-week follow-up visit. The final diagnosis was third-degree UVP in a didelphys uterus.

Mullerian duct abnormalities (MDAs) are structural defects of the female reproductive tract. They occur when the normal development of the paired Mullerian (paramesonephric) ducts into the uterus, fallopian tubes, cervix, and upper vagina is disturbed [ 1 ] . MDAs are observed in 5.5% of the general population, with higher prevalence among women with infertility and recurrent miscarriage cases (reported rates of ~8% and 3.3–24.5%, respectively) [ 2 ] . Embryologically, these anomalies may arise at different developmental stages: Failure of ductal development results in agenesis or hyperplasia, failure of ductal fusion produces anomalies such as uterus didelphys (complete failure) or bicornuate (incomplete failure), and defective septal resorption yields septate uterus or arcuate uterus and longitudinal vaginal septum [ 3 – 5 ] . HIGHLIGHTS Uterine didelphys can present with both catastrophic and chronic gynecologic outcomes. Second-trimester uterine rupture may occur in unscarred didelphic uterus. Pelvic organ prolapse may unmask previously undiagnosed Mullerian anomalies. Uterine didelphys can present with both catastrophic and chronic gynecologic outcomes. Second-trimester uterine rupture may occur in unscarred didelphic uterus. Pelvic organ prolapse may unmask previously undiagnosed Mullerian anomalies. Uterine didelphys is a fusion anomaly that results from complete or partial failure of Mullerian duct fusion, leading to duplication of the uterine horns, cervix, and proximal vagina. It is reported in 0.1–0.5% of women and accounts for about 11% of all MDAs. This anomaly is associated with adverse reproductive outcomes, including recurrent pregnancy loss, preterm birth, malpresentations, and obstructed labor. Endometriosis and infertility are also among the long-term consequences of this condition [ 5 – 7 ] . Pelvic organ prolapse (POP) is relatively common in postmenopausal multiparous women, mainly due to weakening of the supporting pelvic floor structures. However, uterine prolapse confined to a unilateral didelphys uterus is exceedingly rare, with only a handful of cases reported in the literature. Documented presentations include prolapse of one uterus while the contralateral uterus carried a pregnancy to term, postmenopausal women with both cervixes protruding outside the introitus, and prolapse of a rudimentary didelphic horn in a young woman. Asymmetric uterine support in congenital uterine anomalies, parity, and pelvic floor integrity can contribute to atypical variant presentations of such cases [ 8 – 11 ] . Uterine rupture is a catastrophic obstetric complication that typically occurs in the third trimester or during labor, mainly in women with a previous uterine scar. It is very uncommon to observe first- or second-trimester rupture of a uterus, specifically in the absence of uterine scar [ 12 , 13 ] . Mullerian duct anomalies have been identified as risk factors for rupture, especially in the presence of a prior uterine scar or in late gestation [ 14 ] . However, second-trimester uterine rupture in a didelphys uterus, particularly with unilateral involvement, is exceptionally rare in the literature. We present two contrasting cases of uterus didelphys with distinct clinical outcomes: one involving POP of a unilateral uterus in postmenopausal women, and another involving spontaneous second-trimester uterine rupture of a gravid horn in a young woman. This case report emphasizes the importance of recognizing Mullerian anomalies as potential underlying factors in both complex gynecologic conditions and obstetric emergencies. This case report is written according to the SCARE guideline 2025 [ 15 ] .

Uterine didelphys is a Mullerian duct anomaly that results from failure of fusion of the paired Mullerian ducts, leading to duplication of uterine horns and cervix, and sometimes the proximal vagina when associated with vaginal septum [ 16 ] . Although many women are asymptomatic – with the condition often discovered incidentally during imaging or intraoperatively – others may present with reproductive or gynecologic morbidities such as recurrent spontaneous abortions, preterm birth, malpresentations, endometriosis, and infertility [ 17 – 20 ] . Reports on Herlyn–Werner–Wunderlich syndrome (HWWS), a classic variant of uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis, further emphasize the embryologic link between Mullerian and mesonephric duct development. Up to 40.6% of patients with didelphys variants demonstrate ipsilateral renal anomalies, supporting the recommendation for routine renal imaging when a uterine anomaly is identified [ 21 – 24 ] . These studies also highlight that two-dimensional ultrasound frequently under-recognizes complex Mullerian anomalies; here magnetic resonance imaging (MRI) or three-dimensional (3-D) ultrasound significantly improves diagnostic accuracy [ 21 , 22 ] . Importantly, despite increased obstetric risks, fertility outcomes in didelphys and HWWS are generally favorable, with high conception and live-birth rates reported following conservative or fertility-preserving management [ 24 ] . These insights strengthen the rationale for our postoperative fertility counseling after unilateral horn excision in case 1. Our case report describes two very rare and contrasting manifestations of uterus didelphys: [ 1 ] a catastrophic second-trimester uterine rupture and [ 2 ] UVP of one uterus with the contralateral uterine horn remaining in situ in a postmenopausal woman. Uterine rupture is a life-threatening obstetric emergency commonly associated with prior uterine scars. Rupture of an unscarred uterus, especially in the second trimester, is exceedingly rare [ 12 , 13 ] . MDA are at an increased risk of uterine rupture, possibly due to atypical myometrial architecture, reduced distensibility, and a poorly developed lower uterine segment compared to a normal uterus [ 25 , 26 ] . Ravasia et al described the risk of uterine rupture in women with MDAs as 8% compared to 0.61% in those with a normal uterus during trials of vaginal birth after cesarean delivery [ 14 ] . In our case, rupture happened at 17 weeks of gestation, likely when the gravid horn of the uterus attained its maximum capacity or possibly due to implantation in the corneal angle of the uterus. Similar reports have described second-trimester rupture of a gravid Mullerian uterus, often presented with catastrophic intra-abdominal bleeding and expulsion of pregnancy products into the peritoneal cavity [ 26 ] . What differs from most published cases, however, is the extremely early gestational age and diagnostic challenge caused by the seemingly “intrauterine” pregnancy on ultrasound. Similarities with prior studies include the difficulty of preoperative diagnosis and the rapid hemodynamic deterioration, while the earlier gestation age and absence of precipitating factors in our patient contrast with many reported cases that involved later gestation or triggers such as misoprostol or prior uterine scars. To better conceptualize our case, we reviewed similar reports of uterine rupture in women with uterine didelphys. We have summarized key demographic, obstetric, anatomic, and surgical details from comparable published cases in Table 1 [ 27 – 32 ] . Across these reports, second-trimester presentation, massive hemoperitoneum, diagnostic uncertainty, and the need for emergency laparotomy were common themes, similar to our case (Table 1 ). Table 1 Summary of similar published cases of uterine rupture in uterus didelphys S.No. Citation (first author, year) Age(years) Gestational age Horn involved Key presentation Management Outcome 1 Haberal et al , 2016 35 18 wks Not specified Acute abdomen/second trimester bleeding Laparotomy + hemisupracervical hysterectomy Mother survived 2 Gupta et al , 2014 30 19 wks + 6 days Left Acute abdomen /Massive hemoperitoneum Laparotomy + hemisupracervical hysterectomy Mother survived 3 Suthar et al , 2011 26 39 weeks Right Acute abdomen/rupture Laparotomy + Repair Mother survived 4 Suhrud et al , 2025 24 18 Left horn Shock, hemoperitoneum Laparotomy/repair + BTL Mother survived 5 Conde-Gutiérrez et al , 2020 27 21.2 weeks Not specified Shock, hemoperitoneum Laparotomy/Strassman metroplasty Mother survived 6 Lee et al , 2009 28 26 weeks Right Acute abdomen/shock Laparotomy/repair Mother survived Summary of similar published cases of uterine rupture in uterus didelphys Preoperative diagnosis of such cases is particularly challenging, as standard ultrasound imaging of pregnancy in one uterine horn may mimic the normal cavity of the uterus. Although difficult to obtain in acute emergencies, 3-D ultrasound or MRI provides better delineation of congenital uterine anomalies [ 33 ] . As in our case, when unexplained hemoperitoneum is identified despite apparent intrauterine pregnancy during initial sonography, clinicians must maintain a high index of suspicion for uterine rupture in the setting of a Mullerian anomaly. POP is most commonly observed in postmenopausal multiparous women as a consequence of cumulative childbirth-related trauma to the pelvic floor. The coexistence of Mullerian duct anomalies with uterine prolapse is a very rare clinical entity, with only about two dozen cases having been reported in the literature [ 8 , 10 ] . However, this scarcity of reports should not be misinterpreted as indicating that such anomalies are less likely to prolapse, nor should the presence of a bulky didelphic uterus or a vaginal septum be considered protective. Prolapse results fundamentally from global weakness of the pelvic supporting structures, rather than being related to uterine bulkiness or anatomic configuration [ 34 ] . Didelphys uterus accounts for the majority of Mullerian anomalies reported in association with POP, and most cases have been described in women of reproductive age. Notably, three to four reports have documented concomitant pregnancy in the contralateral uterine horn carried to term [ 8 – 10 ] . At the other extreme, neonatal POP has been reported in association with bicornuate uterus, emphasizing that prolapse in the setting of Mullerian anomalies can occur across the entire age spectrum [ 35 ] . Only a few cases of postmenopausal didelphys uterus with prolapse have been reported [ 36 ] . This rarity may reflect both the generally asymptomatic nature of didelphic uterus and the underreporting of Mullerian anomalies in older women. To provide context for our postmenopausal didelphys case presenting with POP, we have collated previously reported cases highlighting patient demographics, anatomy, clinical presentation, and surgical management (Table 2 ). These reports illustrate that although rare, didelphys-associated POP can occur across a wide age range, often requires tailored surgical planning, and outcomes are generally favorable when appropriately managed [ 8 – 10 , 37 ] . Importantly, this emphasizes the need for intraoperative vigilance when unexpected resistance, distorted planes, or unusual masses are encountered during vaginal surgery, as demonstrated in our case. Table 2 Summary of reported cases of uterine didelphys associated with pelvic organ prolapse (POP) S.No. Citation (first author, year) Age(years) Uterine anomaly/Horns status Key presentation (POP ± pregnancy) Management Outcome 1 Thorat et al , 2018 Elderly/ multiparous Didelphys (unicollis) Third-degree uterovaginal prolapse (cystocele + rectocele) Vaginal hysterectomy + pelvic floor repair Uneventful postoperative recovery 2 Yayna et al , 2023 28, G4P3 Didelphys – one horn gravid, other horn prolapsing Prolapse of one uterus + pregnancy in the other Expectant until term → Cesarean delivery Healthy term newborn; maternal recovery 3 Christopoulos et al , 2009 20, nulliparous (primary amenorrhea) Didelphys with non-canalized horns Third-degree uterine prolapse (rudimentary horn) Laparotomy + excision of prolapsed rudimentary uterus Not explicitly complicated; prolapse corrected 4 Abdalla et al , 24, prim gravida Didelphys – one horn gravid, other horn prolapsing Acute non gravid uterine prolapse Expectant until term → Cesarean delivery Healthy term newborn; maternal recovery Summary of reported cases of uterine didelphys associated with pelvic organ prolapse (POP) Important lessons we can take from these cases include: First, although rare, uterine anomalies could result in both catastrophic obstetric outcomes and chronic gynecologic sequelae. Second, preoperative identification of didelphys uterus is challenging, particularly when one cavity is dominant due to pregnancy or when evaluation occurs in an emergency situation. The clinical context and resource availability affect the use of highly sensitive MR or 3-D ultrasound in detecting such cases. Third, individualization is mandatory in the management of such cases. In case of unilateral uterine horn rupture, rapid recognition and repair or surgical removal, depending on the extent of the rupture and hemodynamic stability of patients, are lifesaving. In case of uterine prolapse, thorough preoperative evaluation, careful intraoperative dissection, and meticulous technique in the presence of altered anatomy are essential for a favorable postoperative outcome. In summary, our two cases highlight how a didelphys uterus can present both preoperative diagnostic challenges and unexpected intraoperative findings. In the first scenario, the diagnosis became apparent only after the decision to perform a laparotomy for massive hemoperitoneum, at which point a second-trimester uterine rupture was revealed. In the second scenario, a third-degree UVP with a single cervix on clinical examination was straightforward, yet intraoperatively a proximal uterine mass was encountered, making the surgery more difficult. A careful stepwise dissection eventually revealed a previously undetected contralateral uterine horn. These contrasting experiences highlight how this uncommon anomaly can present in both acute obstetric emergencies and seemingly routine gynecologic surgeries, reinforcing the need for vigilance, individualization, and preparedness for unexpected intraoperative anatomy in clinical practice.

These cases highlight the wide spectrum of presentations in uterine didelphys and the associated diagnostic challenges, particularly in emergency situations and resource-limited settings. They emphasize the need for a high index of suspicion, vigilance, individualized management, and preparedness for unanticipated intraoperative findings to optimize outcomes for women with this rare anomaly.