Minimally Invasive Management of a Mullerian Anomaly: A Clinical Decision-Making Tree

Müllerian duct abnormalities (MDAs) are congenital malformations of the female reproductive tract affecting 3–7% of women worldwide. In obstructive cases, they can present with chronic pelvic pain, dysmenorrhea, and abnormal uterine bleeding. If left untreated they can lead to complications like infection and endometriosis. A timely diagnosis and treatment are essential to preserve reproductive potential. This is a case of a 17-year-old nulligravid sexually active female that presented to the gynecologic office with complaints of 5 years of on and off pelvic pain, irregular menstrual cycles with dysmenorrhea and heavy bleeding. Her past medical history was significant for a congenitally absent left kidney and ureter confirmed on a prior computed tomography (CT) scan. The patient was sent for a screening transabdominal pelvic sonogram which suggested a bicornuate uterus. Magnetic resonance imaging (MRI) with contrast was done for confirmation which diagnosed her with a unicornuate uterus with left noncommunicating rudimentary horn with functional endometrium, containing hematometra consistent with an ASRM Class 2b anomaly ( Figure 1 ). 1 Due to the patient’s progressively worsening symptoms and to prevent complications, the decision was made to proceed with operative intervention. Office based vaginoscopy confirmed the presence of a single cervix. Hysteroscopy ruled out septate uterus and other causes of abnormal uterine bleeding. Of note, there was only one tubal ostia present on the right side, the left ostia was unable to be visualized. On diagnostic laparoscopy, the right side of the uterus and fallopian tube appeared normal. The left rudimentary horn was resected with bipolar cautery. The ipsilateral fallopian tube was removed to decrease ectopic pregnancy risks. Appendiceal inflammation was noticed, appendectomy was performed with histopathology confirming appendiceal endometriosis ( Figures 2 – 6 ). The patient recovered well postoperatively and was placed on hormonal suppression for endometriosis. At her six-month visit she had regular menstrual cycles and was pain-free. MRI images of bicornuate uterus showing the left showing rudimentary horn and right fully developed uterine cavity. The two horns are far apart which helped guide the surgical steps. Diagnostic laparoscopy showing the right unicornuate uterus with normal fallopian tube. Normal appearing liver. Resection of rudimentary horn and ipsilateral fallopian tube. Removal of specimen. Chromotubation with methylene blue confirmed patency of right fallopian tube. Right unicornuate uterus, inflamed appendix.

MDAs affect young adolescent females who may present with nonspecific complaints, but in severe cases may present with obstructive symptoms like pelvic pain, dysmenorrhea, menorrhagia. 2 Timely diagnosis and treatment are essential to prevent complications such as pelvic abscess, severe endometriosis, pelvic adhesions, appendicitis, and advanced infertility. 3 It is important to recognize the presence of MDAs in the differential diagnosis of pelvic pain. These patients need a screening ultrasound, after which one can follow a simple decision-making tree to arrive at a definitive diagnosis. Accurate and detailed imaging is essential for meticulous preoperative planning in order to restore functional anatomy and maintain reproductive potential. 4 The type and technique of the surgery should take into consideration the symptoms, anatomy, associated malformations, and anticipated complexity of the procedure. Referrals to higher centers and multidisciplinary management can be undertaken as needed. 5 Renal anomalies are present in around 40% of patients with mullerian agenesis and up to 95% in unilateral obstructed anomalies. 1 If there is a urological abnormality found, for example a single kidney, the patient must be counseled on cautious use of nonsteroidal antiinflammatory drugs (NSAIDs) and contact sports which may affect the solitary kidney, along with referral to a urologist as needed. Patients need to be counseled on pregnancy, preterm birth, cesarean section, and small for gestational age. However, patients can be reassured that resection of the rudimentary horn has favorable obstetric outcomes when compared with expectant management. 6 Endometriosis is associated with MDA in up to 90% of cases and it should be looked for and treated during the initial surgery. 7 Psychosocial support is paramount for a teenager for a teenager because of all of the above factors. 8 Long term follow up is required by the gynecologist especially one that develops endometriosis. 9 This case highlights the importance of prompt management of MDAs with advanced imaging and surgical intervention with minimally invasive techniques for symptomatic relief, preventing complications, and preserving fertility. Our clinical decision-making tree provides a guide for clinicians to provide individualized care to patients ( Figure 7 ). Clinical decision-making tree for management of Müllerian duct anomaly.