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Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)01029-0
10.1016/j.ijscr.2024.110248
110248
Case Report
Symptomatic leiomyoma in a patient with a bicornuate uterus: A rare case report
Mwidibo Yusuph ab
Masunga Daniel S. ab
Rwenyagila Doris ab
Mlay Joseph ab
Mremi Alex alex.mremi@kcmuco.ac.tz
bcd⁎
a Department of Obstetrics and Gynecology, Kilimanjaro Christian Medical Center, Moshi, Tanzania
b Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania
c Department of Pathology, Kilimanjaro Christian Medical Center, Moshi, Tanzania
d Kilimanjaro Clinical Research Institute, Moshi, Tanzania
⁎ Corresponding author at: Faculty of Medicine, Kilimanjaro Christian Medical University College, Box 2240, Moshi, Tanzania. alex.mremi@kcmuco.ac.tz
06 9 2024
10 2024
06 9 2024
123 11024811 8 2024
28 8 2024
4 9 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction and importance

Uterine anomalies are congenital malformations caused by fusion or resorption defects during embryogenesis. A bicornuate uterus is a rare condition that results from abnormal development of the paramesonephric ducts. It results from an incomplete fundus-level fusion of uterovaginal horns.

Case presentation

A 30-year-old female presented with a longstanding abdominal distension. An abdomino-pelvis CT scan result was suggestive of a huge right ovarian mass (25.8 × 25.9 × 14.3 cm). Intraoperatively, a bicornuate uterus with normal left cornua was encountered. The right cornua was not extending to the vagina vault and had a huge cystic mass arising from the fundus. The right cornua excision along with the mass was performed, preserving the left normal cornua and its adnexa. Histopathology confirmed the diagnosis of leiomyoma. The patient was kept in follow-up. To date, six months have passed; she has resumed her menstrual cycles and is free from symptoms.

Discussion

Symptomatic fibroids in patients with a birconuate uterus are rare. Thus, diagnostic challenges can be encountered, as was seen in this case. A huge size of the fibroid in one horn of the bicornuate uterus projecting into the pelvic cavity can contribute to recurrent pregnancy loss. An MRI of the pelvis is recommended for assessment of the internal and external contours of the uterus.

Conclusion

A bicornuate uterus with lieomyomas is an uncommon but difficult condition. An appropriate pre-operative assessment of the mullerian anomaly type, quantity, location, and size of lieomyomas, as well as urinary tract mapping, are essential for effective management.

Highlights

• A fusion failure of mullerian ducts resulting in a uterus divided into two horns.

• May cause dysmenorrhea and or difficulties in conception.

• Poses diagnostic challenges due to the bicornuate nature.

• An MRI is recommended for assessing internal and external contours of the uterus.

• The surgical removal of fibroids while preserving the uterus is recommended.

Keywords

Mullerian duct
Bicornuate uterus
Leiomyoma
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pmc1 Introduction

Abnormal fusion of the mullerian ducts during embryological life results in a variety of congenital uterine malformations [1]. A bicornuate uterus is a type of uterine duplication anomaly. There is a partial failure of fusion of mullerian ducts, resulting in a uterus divided into two horns. The etiology of such abnormalities remains unknown. Studies have reported that bicornuate uterus may be associated with renal tract anomalies [2,3]. Bicornuate uteri can be divided according to the involvement of the cervical canal: (a) bicornuate bicollis is when there are two cervical canals; the central myometrium extends to the external cervical os; (b) bicornuate unicollis is when there is one cervical canal; the central myometrium extends to the internal cervical os [4]. Overall, congenital uterine anomalies occur in about 1.5 % of females, ranging from 0.1 to 3 %. Bicornuate uteri are estimated to represent 25 %, ranging from 10 to 39 % of mullerian duct anomalies [5]. When the pelvis is scanned, a bicornuate uterus is typically unintentionally found. Cervical incompetence and early pregnancy loss are the most typical symptomatic presentations [1]. This kind of deformity typically does not cause infertility because it does not affect embryo implantation.

On the other hand, uterine leiomyomas (fibroids) affect all ages, especially in the fifth decade of life. They are more common (up to 80 %) in African American women [5,6]. The cause of fibroids remains unclear, and their biology is poorly understood. Clinically, about 25–50 % of patients with fibroids are symptomatic, and this may affect the quality of their life [7,8]. Symptoms depend on size and location. Menorrhagia and pelvic pain occur in up to 33 % of patients. The management of these benign lesions depends on their location (subserosa, intramural, and submucosa) and clinical presentation with regards to their size. Smaller myomas can be managed conservatively, but large myomas require myomectomy, and those that weigh more than 10 kg may end up with total or subtotal hysterectomy [9,10]. Herein, the authors describe a case of symptomatic uterine leiomyoma in a patient with a bicornuate uterus that was managed successfully. A brief literature review is highlighted. This work has been reported in line with the SCARE 2023 criteria [11].

2 Case report

A 30-year-old female of African origin was referred from a peripheral health facility to our center with a chief complaint of an abdominal distension. She reported early satiety, tiredness, and easily fatigability on walking, with an increased frequency of urination. She also reported a history of feeling of abdominal fullness and difficulty conceiving or maintaining a pregnancy.

Her history of the present illness revealed that she had been experiencing these symptoms for about two years. Although the abdominal distension was of gradual onset, it was progressively increasing in size over time, and it was associated with lower abdominal pain of no specific periodicity. Initially, she sought local traditional medicines that were unknown to us for six months without improvement. She denied a history of weight loss, coughing, and chest pain. She denied a history of a surgical treatment. No history of headache, blurred vision, loss of consciousness, or changes in stool pattern. However, she reported that the worsening symptoms were negatively affecting the quality of her life, particularly sexual function and daily work.

Her obstetric history revealed that she delivered at term a 3.6 kg female baby in 2013 through a spontaneous vaginal delivery (SVD) without post-delivery complications. She reported a history of recurrent abortions. Her gynecological history indicated that she attained menarche at the age of 14 years, and she had a cycle of 28 days with 5–6 days of painful heavy menstruation, of which she can change up to 4 pads a day, which are fully soaked and associated with severe lower abdominal pain. She denied the use of contraceptives, and she had never been screened for cervical cancer before.

Her past medical and surgical history indicated that she had no chronic illness such as diabetes mellitus or hypertension. She also denied a history of abdominal surgery. This was her first hospital admission. She denied a history of blood transfusions, major or minor surgeries, and she had no reported history of any known drug or food allergies. She was not aware of similar presentations among her close family relatives. Her family history indicated that she was married, living with her family. She was a small businesswoman, non-smoker, and non-alcoholic. Her social history revealed that she had a desire for future fertility and childbearing. Her hormonal history revealed that she had never used hormonal treatment such as oral contraceptives or hormonal replacement therapy. Her drug history indicated that she often uses 1 g of paracetamol for the pain relief of the abdominal pain.

On examination, the patient was alert, not pale, nor jaundiced, afebrile, not tachypneic, with no lymphadenopathy. Her vital signs were within the normal range. Per abdomen, she had a grossly distended abdomen that was moving with respiration with a fundal height (FH) of 30/37 weeks. There were traditional incision scars on the suprapubic area that were slightly tender on deep palpation. A pelvic examination revealed tenderness on the right adnexal with a normal speculum exam. The pregnancy test was negative, and urinalysis and stool analysis were essentially normal. She had a normal complete blood count (CBC) with a blood group B positive. Her serum sodium, potassium, and creatinine were within the normal range, as were her normal hepatic enzymes. Hepatitis B surface antigen and hepatitis C virus tests were negative. Radiological examination revealed a normal chest x-ray study. An abdominal pelvic ultrasound (USS) revealed features of splenomegaly. A computed tomography (CT) scan report described a cystic mass with enhancing solid components and thickened enhancing septa seen probably arising from the right adnexa measuring 25.8 × 25.9 × 14.3 cm. The lesion exerts a mass effect on the intraperitoneal organs with significant displacement of the bowel loops supero-posteriorly. The uterus was displaced to the left, and the urinary bladder was compressed anteriorly with an impression of a right adnexal complex cystic mass (Fig. 1A). The patient was counseled for the surgery, and the procedure was scheduled after obtaining her informed consent.Fig. 1 A CT scan image demonstrating a huge pelvic mass (PM) filling the peritoneal cavity, giving the impression of the right adnexa tumor (A). An opened abdomen with a protruding uterine mass exerting pressure effect on the abdomen visceral organs (B).

Fig. 1

3 Surgical procedure

The surgery was performed by a team of experienced obstetricians and gynecologists. Under aseptic conditions, the patient was draped, and the abdomen was opened through a sub-umbilical midline incision (SUMI). The presence of the uterine mass extending up to the level of the liver pushing the diaphragm superiorly was noted. An incision was extended above the umbilicus to expose the entire mass (Fig. 1B). A bicornuate uterus was appreciated. The left cornua appeared normal, with the cervix extending to the vagina vault with only the left round ligament. The right cornua was not extending to the vagina vault, and it had a multinodular mass with mixed texture suggestive of a fibroid (Fig. 2A). The mass was arising from the fundus extending to the level of the liver. Both ovaries, fallopian tubes, and liver, omentum, and spleen appeared normal. The endometrium of both cavities was exposed. The clinical diagnosis of symptomatic uterine fibroid coexisting with a bicornuate uterus was established. The right uterine cornua resection along with the mass and its adnexa was performed, preserving the left normal cornua and its adnexa. In order to create a single uterine cavity, the myometrium was adhered to, excluding the endometrium, using interrupted sutures. Conventional surgical methods were employed to reconstruct the remaining portion of the uterus (Fig. 2B). The uterus was reperitonized, and the abdomen was closed. The resected specimen (Fig. 3A) was submitted for histopathology evaluation. A pathology report described a well-circumscribed uterine tumor displaying a diffuse growth pattern. The tumor comprised bundles of smooth muscles that were made up of spindle-shaped cells without cytologic atypia or mitoses (Fig. 3B). The diagnosis of uterine leiomyoma was concluded. The postoperative period was uneventful. At least six months have passed; the patient is experiencing normal menstruations, has resumed her routine life, and is free from symptoms.Fig. 2 A bicornuate uterus with normal appearing left corneal (green arrow) and a well-circumscribed multinodular fibroid arising from the fundus or the right cornua (black arrow), (A); a reconstructed uterus, (B). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 2

Fig. 3 The resected fibroid attached to the right cornea (A); histopathology of leiomyoma demonstrating a diffuse spindle cell tumor comprised of well defined borders. The cells are normocellular, forming intersecting fascicles of monotonous with indistinct borders, eosinophilic cytoplasm, cigar-shaped nuclei (with tapered ends), and small nucleoli. Neither mitoses nor necrosis are seen; H&E staining at 100 x original magnifications, (B).

Fig. 3

4 Discussion

Our report describes a rare case of a bicornuate uterus in addition to a symptomatic fibroid that was surgically treated at our facility. Uterine congenital malformations result from an anomaly in combination, canalization, and resorption of the septum during the development of mullerian ducts [1]. The American Society of Reproductive Medicine has classified mullerian duct anomalies into seven classes based on the degree of failure of mullerian duct development:a. Class I: Agenesis/hypoplasia

b. Class II: Unicornuate uterus, IIa with a rudimentary communicating horn; IIb with a rudimentary non-communicating horn; II c with a rudimentary horn without a cavity: II d without a rudimentary horn

c. Class III: Didelphys uterus

d. Class IV: Bicornuate uterus (IV a is complete, IV b is partial)

e. Class V: Septate uterus

f. Class VI: Arcuate uterus

g. Class VII: Diethylstilbestrol-related anomalies [4].

Contrary to this case, women with bicornuate uteri do not exhibit any signs and symptoms [1]. Unfavorable reproductive outcomes, such as premature labor and repeated abortions, have been linked to a bicornuate uterus [1,5]. Due to having two uterine cavities, some women may come to the clinic with menorrhagia or dysmenorrhea. A small number of pregnant women who come in for routine evaluations also receive diagnoses. When women exhibit symptoms of pregnancy problems, a considerable proportion of them receive a diagnosis. In isolated anomalies, physical examination is usually not noteworthy.

Diagnosing bicornuate radiologically can be challenging. In our case, both USS and CT scans failed to diagnose bicornuate uterus. The reason for this diagnostic error is not clear. Perhaps it is due to the rarity of a birconuate uterine anomaly that was coexisting with a huge fibroid that was mistaken for an ovarian mass. Similarly, the inexperience of the radiologist who attended this case may have contributed to this diagnostic error. MRIs, hysterosalpingograms, and ultrasounds are the recommended imaging modalities for uterine abnormalities [12,13]. The uterine horns are widely divergent, and the exterior uterine contour is concave or heart-shaped. Usually, the intercornual distance is expanded, and the fundal cleft is deeper than 1 cm. It is believed that the uterus is made up of two symmetric, caudally united uterine cavities that are somewhat communicative (typically at the uterine isthmus). The angle between the horns of the bicornuate uterus is typically greater than 105°, although this is not a precise result. The two most significant differential diagnoses for birconuate uterus are (i) uterus didelphys, which is characterized by a persistent longitudinal septum that partially divides the uterine cavity, and (ii) septate uterus, which has a normal fundal contour but a complete failure of fusion occurs during the development of the paramesonephric ducts with duplication of the uterus, cervix, and vagina.

It is essential to distinguish the bicornuate uterus from the septate and didelphys uteri while managing this condition [9,12]. A definitive diagnosis requires a skilled radiologist who is knowledgeable about the embryology of uterine abnormalities [13,14]. The way a patient presents clinically determines how best to manage their bicornuate uterus. Women who had repeated abortions can consider having a Strassman metroplasty [1]. Luckily, our patient was not pregnant, this made surgery a little bit easier. Aggressive prenatal surveillance is advised to avoid obstetric issues if a pregnant lady shows up for a regular evaluation and is found to have a bicornuate uterus [15,16]. As an uncommon condition, a gynecologist's job is to educate patients about the associated complications and help them understand their diagnosis [1]. It is imperative that women diagnosed with bicornuate uterus receive comprehensive counseling [1]. Anxiety or depression are common in women who lose pregnancies repeatedly [17]. In a situation like this, the psychiatrist is quite important. It is therefore important for a primary care physician to keep an eye out for anomalies in teenagers who present with dysmenorrhea together with menorrhagia. This may result in an early detection of the anomaly and, thus, improved future reproductive results [18].

5 Conclusion

Bicornuate uterus with symptomatic fibroids is uncommon but a challenging condition. Appropriate care requires a thorough preoperative assessment of the kind of mullerian anomaly, the quantity, location, and size of fibroids, as well as a urinary tract mapping. A multidisciplinary strategy is necessary to guarantee the patient's safe recuperation. The documentation of these uncommon events can guide clinicians who will encounter similar cases in the future.

Consent

Written informed consent was obtained from the patient to publish this case report and accompanying images. On request, a copy of the written consent is available for review by the Editor-in-Chief of this journal.

Ethical approval

The ethical approval in this study was exempted on August 1, 2024 by the Research Ethics Committee, Kilimanjaro Christian Medical University College, Box 2210 Moshi, Kilimanjaro, Tanzania, email: info@kcmuco.ac.tz; Web: www.kcmuco.ac.tz; Tel: +255272753616); because individual case reports do not require ethical approval.

Funding

This work did not receive any fund from any source.

Author contribution

All authors made substantial contributions to this work.

Yusuph Mwidibo: Conceptualization, study design, data curation, patient management, and prepared initial manuscript version.

Daniel S. Masunga: Involved in the patient management, collected the data, and reviewed, and approved the final manuscript draft.

Doris Rwenyagila: Involved in the patient management, and supervision, and reviewed, and approved the final manuscript draft.

Joseph Mlay: A lead Obstetrician and Gynecologist, involved in the patient management, supervision, and reviewed and approved the final manuscript draft.

Alex Mremi: Conceptualization, and also performed histopathological analysis and prepared the final manuscript draft.

Guarantor

Alex Mremi.

Research registration number

1. Name of the registry: Not applicable.

2. Unique identifying number or registration ID: Not applicable.

3. Hyperlink to your specific registration (must be publicly accessible and will be checked): Not applicable.

Conflict of interest statement

All authors have declared that no competing interests exist.
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