Cases
A 22-year-old, nulligravida, female, married 3 months back, presented with severe lower abdominal pain during menstruation, in the last 1 year. She attained menarche at 14 years of age and had regular menstrual cycles every 30 days with an average of 3–4 days of flow with occasional dysmenorrhea that required medication. On examination, her vulva, vagina, and cervix were anatomically normal. Bimanual pelvic examination showed normal-sized anteverted uterus with the presence of uterine motion tenderness and free and nontender bilateral fornices.
Combined transabdominal and transvaginal ultrasound [ Figure 1 ] revealed septate uterus (64 mm ×52 mm ×27 mm) with normal external uterine fundus and hypoechoic septum dividing uterine cavity into the two halves and normal bilateral ovaries. A small right ovarian hemorrhagic/endometriotic cyst (9 mm ×8 mm) was present. The cervix was normal in size and echo texture, and left renal agenesis was there.
Ultrasound images of (a) bilateral ovaries; (b) transverse section of uterus showing two uterine hemicavities separated by thick myometrium (blue arrow); (c) left uterine hemicavity continuous with cervical canal
To further evaluate the location, extent, and size of the septum, we performed the 3D pelvic ultrasound [ Figure 2 ] which unveiled, tubular left uterine cavity (6.3 mm) continuous with endocervical canal and noncommunicating right uterine cavity (5.8 mm). The distance between two endometrial cavities is 12 mm. No evidence of (e/o) hematometra. The diagnosis of Robert’s uterus was made.
Three-dimensional ultrasound images of uterus (a) coronal section of the uterus, right hemicavity (yellow arrow) not continuous with cervical canal (grey arrow); (b) transverse section of the uterus showing two hemicavities separated by myometrium
Hysteroscopic metroplasty along with diagnostic laparoscopy [ Figure 3 ] was performed. Laparoscopy showed that the fundus of the uterus was wide with agenesis of the right fallopian tube with right periovarian omental adhesions. Patency of left fallopian tube was confirmed with spill of methylene blue dye from its distal end. Hysteroscopy showed left uterine hemicavity with ostium without any communication with the right hemicavity. A longitudinal incision of the septum with a hysteroscopic scissor was performed to enter the right cavity and unification of the two cavities under transabdominal ultrasonic guidance. She had regular menstruation without pain postoperatively on follow-up.
Laparoscopy images showing (a) pelvic anatomy, (c) left tube and ovary, (d) right tubal agenesis (yellow arrow); Hysteroscopy image showing (b) right hemicavity after septal incision by hysteroscopic scissor (red arrow)
Robert’s uterus is a rare congenital Müllerian abnormality, with one unicornuate uterine cavity and other blind hemicavity.[ 5 ] It can be of three varieties based on blind hemicavity as follows: with a large hematometra and acute pelvic pain; with a small hematometra; with an inactive blind hemicavity without hematometra and recurrent miscarriages. The third variety usually presents with delayed diagnosis, as in our case. Hematometra may be associated with hematosalpinx, endometriosis, and ipsilateral renal agenesis. Blind horns are more common on the right side due to the early development of the left Müllerian duct.[ 6 ]
As very few case reports of the condition are available in the literature, the diagnosis and management of Robert’s uterus are not fully established. Currently, 3D ultrasound is found to be more cost-effective, accessible diagnostic modality, and ultrasound-guided hysteroscopic management to unify the two cavities is found to be a practical, minimally invasive, safe choice for Robert’s uterus. Simultaneously laparoscopic management can be done for hematosalpinx and endometriosis.[ 7 8 ] Risk of intrauterine adhesions, placenta accreta syndrome, and ruptured uterus during pregnancy may be increased and close monitoring and intervention required.
The present case was without hematometra and so asymptomatic for a long period of time made the early diagnosis challenging. Systematic 3D ultrasound was performed to reach the diagnosis instead of MRI considering its easy availability, cost-effectiveness, and reproducibility. Transabdominal ultrasound-guided hysteroscopic scissor use was crucial for accuracy of the surgery, avoid false passage, and injury to the outer myometrium, and to protect the endometrium and in turn protect future fertility.
Gynecologists and sonologists should be made aware of the condition, as early diagnosis can avoid future morbidity and protect fertility.
This study was conducted in accordance with the ethical principles outlined in the Declaration of Helsinki and its amendments. The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.
AS, SP, RP and PP managed the case and collected relevant data. AS drafted the manuscript with direction from SP, RP, and PP. AS, SP, RP, and PP critically evaluated the manuscript. All authors approved the final version of the manuscript.
Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.
There are no conflicts of interest.
Intro
The prevalence of Mullerian duct anomaly is around 4.3%.[ 1 ] Female genital tract namely the uterus, cervix, fallopian tube, and upper one-third of the vagina develops from the Mullerian duct. The common presenting symptoms range from primary amenorrhea to heavy menstrual bleeding and dysmenorrhea, chronic pelvic pain, palpable mass per abdomen, and urinary complaints.[ 2 3 ] Diagnosis of the condition is usually done by three-dimensional (3D)-ultrasound, magnetic resonance imaging (MRI), or laparoscopy. With the technical advances and expertise, in the majority of cases, management can be done by conservative laparoscopic surgery.[ 4 ]