Double simultaneous torsion: A case report of concurrent torsion of the right adnexa and a right round ligament fibroma

Abstract

Introduction and importance

Round ligament leiomyomas (RLL) are uncommon benign tumors of the pelvic region. When torsion occurs, it can lead to acute or chronic pelvic pain and potentially severe complications such as gangrene, peritonitis, hemoperitoneum, and shock if not promptly diagnosed and treated. Torsion of the adnexa and torsion of round ligament fibromas are rare gynecological emergencies, and their simultaneous occurrence is exceedingly uncommon. This report highlights what appears to be the first documented case of double torsion involving both the right adnexa and a round ligament fibroid, emphasizing its clinical significance and diagnostic challenges.

Case presentation

We describe a rare case of a leiomyoma of the round ligament that was preoperatively misdiagnosed as a large malignant ovarian tumor. The patient presented with chronic pelvic pain progressing to acute exacerbation and abdominal bloating. She was found to have simultaneous torsion of the right adnexa and torsion of the round ligament fibroid. The adnexal torsion involved the right ovary and fallopian tube, complicating the clinical picture. Imaging studies and diagnostic tools were utilized, leading to a complex diagnostic process. Surgical intervention was performed to manage the condition, and the postoperative course was uneventful.

Clinical discussion

The simultaneous torsion of the adnexa and a round ligament fibroid presents a significant diagnostic challenge due to overlapping clinical and imaging findings with other pelvic pathologies, particularly malignancies. Misdiagnosis can lead to delays in treatment and worsen outcomes. This case highlights the importance of considering rare pathologies like round ligament torsion in patients presenting with acute or chronic pelvic pain, especially in the presence of complex imaging findings. Early recognition and timely surgical intervention are critical to prevent severe complications such as gangrene and peritonitis.

Conclusion

Double torsion involving the adnexa and a round ligament fibroid is an extremely rare and complex clinical scenario. This case underscores the need for heightened awareness and careful diagnostic evaluation of pelvic pain in women, as prompt surgical management is essential to avoid life-threatening complications. Further studies are warranted to better understand and manage this unusual combination of pathologies.

Highlights

• •Unprecedented Case: This report details what appears to be the first documented instance of simultaneous torsion involving a right adnexal torsion and a torsion of a round ligament fibroid, highlighting its rarity and clinical significance.
• •Diagnostic Challenges: The case emphasizes the significant diagnostic challenges due to overlapping clinical and imaging findings, often leading to misdiagnosis, as the fibroid was initially suspected to be a malignant ovarian tumor.
• •Importance of Imaging and Surgery: Imaging techniques such as ultrasound and MRI played a critical role in evaluating the masses, although definitive diagnosis was only achieved intraoperatively, underscoring the importance of surgical intervention in such complex cases.
• •Clinical Insights and Management: The study provides valuable insights into the clinical presentation, imaging findings, and surgical management of this unusual combination of pathologies, advocating for heightened awareness among clinicians to ensure timely treatment and avoid severe complications.

1. Introduction

Fibroids, or leiomyomas, are common benign tumors primarily affecting premenopausal women, most often arising from the uterine myometrium. However, leiomyomas can rarely originate from the round ligament, a fibromuscular band extending from the uterine cornu to the labia majora through the inguinal canal. These round ligament leiomyomas (RLLs) may mimic adnexal or inguinal masses depending on their anatomical location [1,3]. While most are asymptomatic, symptomatic cases may present with pelvic pain, particularly if complications such as torsion or degeneration occur.

Adnexal torsion, involving the ovary and/or fallopian tube, is a gynecological emergency that typically presents with acute pelvic pain, nausea, and vomiting. Simultaneous torsion of the adnexa and a round ligament leiomyoma is exceedingly rare and presents a diagnostic challenge due to overlapping clinical features with other pelvic pathologies, including malignancy. Here, we report what appears to be the first documented case of concurrent torsion involving both the right adnexa and a round ligament fibroid, emphasizing its clinical significance and the importance of early recognition and surgical intervention [3,4].

We describe here a rare case of leiomyoma of round ligament which was preoperatively diagnosed as huge malignant ovarian tumor associated with simultaneous torsion of the adnexa.

This work has been reported in accordance with the SCARE (Surgical CAse REport) criteria [2].

2. Case presentation

A 50-year-old woman with no significant medical history presented with chronic pelvic pain for several months, which acutely worsened over the past 48 h, accompanied by abdominal bloating. Genitourinary exam was normal, and on physical exam, she appeared distended with right lower quadrant tenderness. Suprapubic and endovaginal pelvic ultrasound showed a uterus of normal size and echostructure, two laterouterine masses on the right: one 1410 cm of hypoechogenic appearance suggestive of a solid component, and a second mass of heterogeneous solidocystic appearance, measuring 0605 cm, dependent on the right adnexa, classified as IOTAM3 (International Ovarian Tumor Analysis group classification, indicating a mass with features suggestive of malignancy due to intracystic vegetations), with free fluid in the cul-de-sac. Pelvic MRI was performed to better characterize the two masses and showed a large right latero-uterine mass showing the sign of the spur with the right ovary in favor of its right ovarian origin, well-defined, measuring 118 × 96 mm in the axial plane and 158 mm in height in the sagittal plane.

It appeared solid, with an intermediate heterogeneous T2 signal, a T1 hyposignal. It took up contrast heterogeneously and more prominently than the external myometrium. It presented a cystic portion in its lower part of 6 cm (Fig. 1).

Fig. 1.

Sagittal MRI scan (+iv contrast) of abdomen reveals a large heterogeneous pelvic mass (*).

Several diagnoses were discussed: necrotic pedunculated fibroid, fibrothecoma, torsion of a fibroid, malignant tumor of the right ovary, torsion of an adnexa. This case was atypical due to the combination of two distinct conditions: torsion of a round ligament leiomyoma and simultaneous torsion of the right adnexa, involving both the ovary and fallopian tube.

At the time, the patient was scheduled for laparoscopy, but due to a technical problem, we performed a laparotomy.

Surgery revealed a normal uterus with a normal left adnexa. A 15 cm fibroid was observed, located at the level of the right broad ligament. This fibroid showed signs of torsion. Below this fibroid, a twisted 6 cm cyst was also identified, arising from the right ovary and involving the fallopian tube, confirming adnexal torsion (Fig. 2).

Fig. 2.

The yellow arrow shows a torsioned solid mass arising from the right round ligament. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

The surgical procedure included detorsion of the structures, a cystectomy, and a myomectomy (Fig. 3). The specimen was examined by histopathology, which showed a staggered fascicle of smooth muscle cells. The diagnosis was leiomyoma of the round ligament.

Fig. 3.

Surgical image after detortion of both cyst and round ligament fibroma.

3. Discussion

Embryologically, the round ligament is composed of smooth muscle, connective tissue, blood vessels, and nerves, covered by a mesothelial layer, distinguishing it from the more common uterine leiomyomas. Round ligament leiomyomas are exceptionally rare, with few documented cases in the literature, making their exact incidence unknown. The simultaneous occurrence of round ligament leiomyoma torsion and adnexal torsion is even rarer, likely due to the independent anatomical origins of these structures. The round ligament, extending from the uterus to the inguinal canal, is anatomically distinct from the adnexa, which includes the ovary and fallopian tube. The rarity of their concurrent torsion may be attributed to the low probability of two independent pedunculated structures twisting simultaneously, compounded by the infrequent occurrence of round ligament leiomyomas themselves. In our case, the patient presented with chronic pelvic pain progressing to acute exacerbation, a presentation that reflects the dual pathology: chronic discomfort from the fibroid's mass effect and acute pain from torsion of both the fibroid and adnexa [2,3].

The clinical presentation of simultaneous torsion is nonspecific, often mimicking other acute abdominal conditions such as appendicitis, pelvic inflammatory disease, or isolated ovarian torsion. The torsion of a round ligament leiomyoma is a surgical emergency due to the risk of necrosis, gangrene, or peritonitis if blood supply is compromised. Similarly, adnexal torsion can lead to ovarian ischemia, threatening fertility if not addressed promptly. In our patient, the chronic pain likely stemmed from the enlarging fibroid, while the acute exacerbation was due to the torsion events. This dual presentation complicated preoperative diagnosis, as the imaging findings overlapped with those of malignant ovarian tumors [4,5].

Although preoperative diagnosis of leiomyoma torsion is challenging because of its rarity and lack of specific symptoms, imaging findings are helpful for diagnosis. Fibroids usually appear as solid, echogenic masses with hypoechoic shadowing on ultrasound. Degenerative changes can create a heterogeneous or atypical look, leading to diagnostic uncertainty. Pedunculated fibroids may be challenging to identify and could be mistaken for ovarian lesions [6].

CT can be performed as a second-line imaging modality for the investigation of acute lower abdominal pain but is the primary tool in emergencies to diagnose uterine leiomyoma torsion. Contrast-enhanced pelvic CT highlights size changes, and the mass's link to the uterus confirms its origin [7].

MRI is more sensitive for evaluating leiomyoma size, location, and degeneration but is not always available in emergencies. A pedicle or necrotic leiomyoma on MRI suggests torsion. Necrotic leiomyomas show hyperintense T2 and hyper-isointense T1 signals, with no contrast enhancement due to lack of blood flow. Based on these imaging results and the clinical presentation, the diagnosis of leiomyoma torsion should be considered [8,9].

Management of simultaneous torsion requires urgent surgical intervention to prevent complications such as necrosis, peritonitis, or hemoperitoneum. In our case, laparotomy was performed due to technical limitations, allowing for detorsion, cystectomy, and myomectomy. This approach preserved the uterus and contralateral adnexa, minimizing impact on the patient's reproductive potential. Laparoscopy is preferred when feasible, as it offers reduced recovery time and fewer postoperative complications. The surgical findings confirmed a torsed round ligament leiomyoma and a torsed adnexal cyst, highlighting the complexity of managing dual pathologies. Potential complications of untreated torsion include gangrene, sepsis, and, in rare cases, malignant transformation of leiomyomas, though this was not observed in our patient. Histopathology confirmed the benign nature of the leiomyoma, consistent with the literature [10,11].

As discussed above, even with all imaging techniques, it is very complicated to make a good preoperative diagnosis of torsion of a leiomyoma. In fact, according to our case and previous case studies, the possibility of torsion of a leiomyoma was not considered pre-operatively.

4. Conclusion

The simultaneous torsion of a round ligament fibroid and the adnexa is an exceptionally rare and complex clinical scenario that poses significant diagnostic and therapeutic challenges. The rarity of this dual pathology, combined with its nonspecific presentation, underscores the need for a high index of suspicion in women with acute or chronic pelvic pain. Imaging techniques such as ultrasound and MRI are critical for identifying pelvic masses, but their findings may overlap with those of malignant tumors, necessitating surgical exploration for definitive diagnosis. Prompt surgical intervention, whether via laparoscopy or laparotomy, is essential to prevent life-threatening complications such as necrosis, peritonitis, or ovarian loss. This case highlights the importance of considering rare gynecological emergencies in differential diagnoses and advocates for further research to improve preoperative diagnostic accuracy and optimize management strategies for such uncommon conditions.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Ethical approval

Not applicable. Our institution requires no ethical approval for case reports.

Funding

Not applicable.

Author contribution

Dhekra Toumi: conception and design, acquisition of data, drafting the article, revising it critically for important intellectual content, final approval of the version to be published.

Asma Zouaghi: conception and design, acquisition of data, drafting the article, revising it critically for important intellectual content, final approval of the version to be published.

Montacer Hafsi: final approval of the version to be published.

Imen Ghaddab: final approval of the version to be published.

Malek Medimegh: final approval of the version to be published.

Raja Faleh: final approval of the version to be published.

Guarantor

The corresponding author is the guarantor of submission.

I, Montacer Hafsi hereby affirm that this manuscript is original, has not been published previously, and is not under consideration for publication elsewhere. All authors have contributed significantly to this work and have reviewed and approved the final version of the manuscript for submission. We confirm that all sources used have been properly cited and that any conflicts of interest have been disclosed.

Research registration number

None.

Conflict of interest statement

All authors declare that they have no conflicts of interest.