Uterine Didelphys with Intrauterine Gestation: A Comprehensive Review

on

 

Introduction

Uterine anomalies are a crucial aspect of reproductive medicine, often influencing fertility, pregnancy outcomes, and obstetric management. Among these, uterine didelphys, also known as a duplicated uterus, represents a rare Müllerian duct anomaly. This column will explore the cause, etiology, pathophysiology, epidemiology, clinical presentation, imaging features, treatment, and prognosis of uterine didelphys with intrauterine gestation, supported by imaging examples and case findings. 

Etiology and Pathophysiology

Uterine didelphys (duplicated uterus) is classified as a Class III Müllerian duct anomaly. It results from abnormal or failed fusion of the paramesonephric ducts (Müllerian ducts) during fetal development around the 8th to 9th weeks of gestation. Unlike a septate uterus, which arises from failed resorption of the medial septum, uterine didelphys is a true duplication anomaly, producing two completely separate uterine cavities, two endometrial canals, and frequently two cervices, with or without a longitudinal vaginal septum.

  • Cause: Genetic and developmental abnormalities of ductal fusion.

  • Mechanism: Complete non-fusion of the paired Müllerian ducts.

  • Distinctive feature: Presence of two separate uterine horns and cervices, occasionally associated with vaginal duplication.

Epidemiology

Congenital Müllerian anomalies are estimated to occur in approximately 1% of all women, with a higher prevalence among women with infertility and recurrent miscarriage. Uterine didelphys specifically represents 0.3% of uterine anomalies, making it a rare condition. Importantly, it has been reported in association with renal anomalies, such as renal agenesis and ectopic ureters, due to the embryological relationship between the urinary and reproductive tracts.

Clinical Presentation

Many women with uterine didelphys are asymptomatic and may remain undiagnosed until adulthood. Symptoms and complications vary, but common findings include:

  • Menstrual abnormalities: Dysmenorrhea, dyspareunia (if vaginal septum is present).

  • Reproductive complications: Increased risk of miscarriage, preterm delivery, malpresentation, and intrauterine growth restriction (IUGR).

  • Pregnancy outcomes: Women can carry pregnancies to term; however, adverse outcomes are more common than in women with normal uterine anatomy.

Imaging Features

Ultrasound

  • Shows two separate endometrial cavities with divergent uterine horns.

  • A gestational sac may be visualized in one horn, as in the presented case of a 33-year-old woman with 6 weeks and 1 day of intrauterine gestation.

Figure 1. Transabdominal ultrasound showing two distinct uterine cavities and a gestational sac in the left uterine horn.


MRI

  • Provides detailed anatomical evaluation.

  • Demonstrates two endometrial cavities separated by >10 mm, with two distinct cervices.

  • T2-weighted MRI may reveal a longitudinal vaginal septum.

Figure 2. MRI T2-weighted image demonstrating two separate uterine cavities with distinct endometrial canals and double cervices.


Hysterosalpingography (HSG)

  • Reveals two distinct, non-communicating endometrial cavities, each connected to its respective fallopian tube.

Differential Diagnosis

  • Bicornuate uterus: Partial fusion defect, with a common cervical canal.

  • Septate uterus: Failed resorption, appearing as two cavities but with shared outer contour.

  • Unicornuate uterus with rudimentary horn: Asymmetric underdevelopment.

Treatment and Management

Management of uterine didelphys depends on symptoms and reproductive history:

  • Surgical correction is generally not required, unlike a septate uterus.

  • Resection of a vaginal septum may be necessary if it causes dyspareunia or obstructed menstruation.

  • Pregnancy management: Requires close obstetric monitoring due to increased risks of miscarriage, preterm birth, and fetal malpresentation.

  • Assisted reproductive technologies (ART): May be considered in infertility cases, although many women conceive naturally.

Prognosis

The prognosis for women with uterine didelphys is generally favorable if properly monitored. Although associated with higher risks of miscarriage and preterm delivery, many women successfully achieve live births. Early diagnosis through imaging and individualized obstetric care improves outcomes significantly.

Case Discussion

A 33-year-old woman presented with suspected pregnancy. Transabdominal ultrasound revealed two uterine horns, with a single intrauterine gestational sac in the left horn. MRI confirmed the presence of two distinct endometrial cavities and two separate cervices, consistent with uterine didelphys with intrauterine gestation.

Quiz Section

Quiz 1:

Q1: How does the uterine orientation appear in this patient?

a) Anteverted anteflexed

b) Anteverted retroflexed

c) Retroverted anteflexed

d) Retroverted retroflexed

✅ Answer: a) Anteverted anteflexed
Explanation: Ultrasound findings indicated a normal anteverted anteflexed uterine orientation.

Q2: A single intrauterine gestational sac was identified. True or False?
✅ Answer: True
Explanation: Ultrasound confirmed a viable pregnancy in the left uterine horn.

Q3: Fetal cardiac activity was within the normal range for gestational age. True or False?
✅ Answer: True
Explanation: Ultrasound confirmed normal embryonic cardiac activity at 6+1 weeks.

Quiz 2:

Q1: Which best describes this patient’s anatomy?

a) Two uterine cavities with two cervices

b) Two uterine cavities with one cervix

c) Single cavity with a midline septum

d) Unicornuate uterus with rudimentary horn

✅ Answer: a) Two uterine cavities with two cervices
Explanation: MRI confirmed complete duplication.

Q2: This Müllerian anomaly results from incomplete resorption of a septum. True or False?
✅ Answer: False
Explanation: Uterine didelphys arises from failure of fusion, not septal resorption.

References

[1] Y. Y. Chan, K. Jayaprakasan, A. Tan, J. G. Thornton, A. Coomarasamy, and N. J. Raine-Fenning, “Reproductive outcomes in women with congenital uterine anomalies: A systematic review,” Ultrasound Obstet Gynecol., vol. 38, no. 4, pp. 371–382, 2011.

[2] A. Ćwiertnia, D. Borzyszkowska, A. Golara, et al., “The impact of uterus didelphys on fertility and pregnancy,” Int. J. Environ. Res. Public Health, vol. 19, no. 17, 10571, 2022.

[3] J. Garratt and E. S. Siegelman, “MR imaging of Müllerian anomalies,” Magn Reson Imaging Clin N Am., vol. 31, no. 1, pp. 11–28, 2023.

[4] S. Rezai, P. Bisram, I. L. Alcantara, R. Upadhyay, C. Lara, and M. Elmadjian, “Didelphys uterus: A case report and review of the literature,” Case Rep Obstet Gynecol., vol. 2015, 865821, 2015.

[5] M. Grimbizis, E. Camus, D. Tarlatzis, J. Bontis, and P. Devroey, “Clinical implications of uterine malformations and hysteroscopic treatment results,” Hum. Reprod. Update, vol. 7, no. 2, pp. 161–174, 2001.

[6] A. Saravelos, Y. C. Li, and T. Li, “Diagnosis and management of congenital uterine anomalies,” Hum. Reprod. Update, vol. 14, no. 5, pp. 415–429, 2008.

[7] S. Woelfer, S. Salim, P. Banerjee, C. Elson, J. Regan, and D. Jurkovic, “Reproductive outcomes in women with congenital uterine anomalies detected by three-dimensional ultrasound screening,” Obstet Gynecol., vol. 98, no. 6, pp. 1099–1103, 2001.

Comments

Post a Comment