A Rare Case of Acute Pseudo-Meigs ’ Syndrome Presenting with Pleural Effusion and Ascites and a Ruptured Leiomyoma and Hemorrhagic Shock

C l i n M e d International Library Citation: Cowan M, Crantz J, Welsh S, Nahhas W, Lindheim SR (2016) A Rare Case of Acute Pseudo-Meigs’ Syndrome Presenting with Pleural Effusion and Ascites and a Ruptured Leiomyoma and Hemorrhagic Shock. Obstet Gynecol Cases Rev 3:070 Received: September 15, 2015: Accepted: January 25, 2016: Published: January 28, 2016 Copyright: © 2016 Cowan M, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Cowan et al. Obstet Gynecol cases Rev 2016, 3:070 Volume 3 | Issue 1


Introduction
Meigs' Syndrome is classically described as a triad of benign solid ovarian tumor with the gross appearance of a fibroma (either a fibroma, thecoma, or granulosa cell tumor) accompanied with ascites and pleural effusion [1].In contrast, pseudo-Meigs' Syndrome presents with ascites and pleural effusion but with any other pelvic mass [2], including mature teratomas, struma ovarii, and leiomyomata of the ovary, fallopian tube, or uterus [3].While leiomyomata are the most common benign tumors of the uterus, they are rarely implicated in pseudo-Meigs' Syndrome or intraperitoneal hemorrhage.We present a case of a uterine leiomyoma presenting with both of these serious and rare complications.

Case Report
A 56-year-old Gravida 1, Para 0 female presented to the emergency department by ambulance after acute onset of sharp abdominal pain followed by a pre-syncopal episode.While driving home from a massage, she experienced a sudden acute onset of abdominal pain and light-headedness.She managed to steer her car to the shoulder of the highway, but she side-swiped a concrete median at low speed.Alert and awake, she was able to call for ambulance assistance.
Her past medical history was only significant for increasing abdominal girth.She had not seen a physician for medical care for about ten years, but was otherwise is good health.On further questioning, she did note recent progressive shortness of breath, but ascribed it to a lack of conditioning and exercise.Her gynecologic history was significant for a fibroid uterus.
On initial exam, she was ill-appearing, diaphoretic, and displayed discomfort taking deep breaths.Her BMI was 32 kg/m 2 , pulse was 107 beats per minute, blood pressure 67 mmHg systolic and 44 mmHg diastolic, with respirations of 16 breaths per minute.Her abdomen was significantly distended, moderately tender, but without rebound or guarding.
Her hemodynamic status responded well to crystalloid fluid blood loss was minimal compared to pre-existing blood.Total blood and ascitic fluid volume removed from the abdomen was 3800 milliliters.Additionally, 1650 milliliters of pleural fluid was drained in the operating room.Six units of packed red blood cells and two units of fresh frozen plasma were transfused intraoperatively.
Her post-operative recovery was uneventful with complete resolution of her symptoms as well as her pleural effusion on chest X-ray.She was discharged home on post-operative day 3. Pathologic examination of the specimen revealed benign leiomyomata, adenomyosis, unremarkable endometrium, ovaries and tubes.Cytology from the pleural fluid revealed mesothelioma cells and mixed inflammatory cells with the absence of malignant cells; peritoneal fluid was extravascular fluid of low protein content without malignancy or blood clot.

Discussion
Meigs' Syndrome and pseudo-Meigs' Syndrome are very rare conditions.Meigs' Syndrome is present in approximately 1% of all ovarian fibroma, thecoma, and granulosa cell tumors.Specifically in ovarian fibromas, ascites and pleural effusion have been described in 10-15% and 1% of cases, respectively [4].The incidence of pseudo-Meigs' Syndrome is even lower and is rarely associated with benign leiomyomata, which was first reported by Authur in 1909 [5].Since, an additional 33 cases have been reported in the literature (MEDLINE search terms: pseudo-Meigs' and leiomyoma, leiomyoma and ascites).Likewise, hemorrhage from uterine leiomyomata is also a rare occurrence, of which 52 cases have been reported in the literature (MEDLINE search terms: leiomyoma intraperitoneal hemorrhage).
Paracentesis and thoracentesis can be performed for symptomatic relief in either syndrome, but treatment is focused on surgical removal of the tumor.The retrospective diagnosis can be made when the ascites and pleural effusions resolve and do not re-accumulate.
Our case is unusual in that the patient presented in acute distress from leiomyoma rupture as well as pleural effusion.These two overlapping rarities confounded the diagnosis initially.While it is difficult to differentiate ascites in the setting of hemoperitoneum, we can assume our patient did have the triad of pseudo-Meigs' Syndrome prior to the rupture of the leiomyoma because of the cytology of the peritoneal fluid, and her pleural effusion.
The pathophysiology of the ascites and pleural effusion in Meigs' or pseudo-Meigs' Syndrome is not fully understood.It is believed that the ascitic fluid may be transudative or exudative, and the amount of fluid often correlates with the size of the tumor.It has been postulated that interstitial edema accumulates in the mass itself due to limited venous and lymphatic return channels in relation to a large amount of arterial blood flow.This interstitial edema can cause a transudative ascites [6,7], hence the amount of fluid often correlates with the size of the tumor.Another plausible mechanism of ascites may be direct transudate from surrounding lymphatics or vessels due to tumor compression or tumor torsion [8,9].The pleural effusion likely is transferred via lymphatic channels from the peritoneum across the diaphragm and often is on the right side [6,10].Serosanguineous and hemorrhagic effusions have been reported both with benign and malignant pathology and are thought to be related to pleural inflammation [11].
Others have suggested that there is an increase in vascular endothelial growth factor (VEGF) and interleukin 6 (IL-6) which is known to cause vascular hyperpermeability [12] and may be involved in the clinical manifestations.A case of pseudo-Meigs' Syndrome has been described where increased VEGF production from the ovaries was noted [7].This is comparable to the elevated IL-6 in ovarian hyperstimulation, which can have similar presentations with massive ascites and pleural effusions [13].In another case of pseudo-Meigs' Syndrome caused by a metastatic ovarian mass of colorectal origin, removal of the mass resulted in decreased serum VEGF levels and resolution of the patient's pleural effusions and ascites [6].Interestingly, the VEGF was found to be expressed from the oviducts but not from the tumor itself in this case.
Hemorrhagic shock associated with leiomyomata is usually due to rupture of a vessel traversing the surface of a large subserosal leiomyoma.In the case of a large fibroid, the feeding vessels can be quite massive and their walls attenuated.An increase in intraabdominal pressure, trauma, pregnancy, or menstruation has been postulated to increase congestion and subsequent rupture of these vessels [14].Other cases have been described wherein trauma causes damage to the tumor itself, leading to hemorrhage [15], while still others have described rupture of a degenerating cystic leiomyoma as cause of hemorrhage [16].In our case, the cystic leiomyoma cavity was filled with congealed blood, leading us to believe the hemorrhage originated from a disrupted vessel within the wall of the leiomyoma.
While these syndromes should be considered in women with the triad of pelvic mass, ascites and pleural effusion, they can mimic the presentation of much more serious malignant tumors.In a hemodynamically stable patient, cancer antigen 125 (CA-125) measurements may be done, however in our case given the acute presentation, this was not done.Furthermore, this may further cloud the diagnosis, as CA-125 can be elevated in many types of intraabdominal benign pathology [17].

Conclusion
Although pseudo-Meigs' Syndrome is very rare, this case study is a reminder that benign leiomyoma can cause significant sequela if not addressed.The life-threatening complications encountered in this case mimic behaviors and complications of malignant neoplasms, thus a broad differential diagnosis is necessary for surgical planning.

Figure A :
Figure A: (A) Uterine mass on Computer Tomography with IV contrast (arrows); (B) Ascites can be seen displacing the liver medially (arrows); (C1,2) Right-sided pleural effusion in coronal and transverse planes (arrows); (D) Resected Pelvic Mass in a 12-inch basin with the ruptured cavity at the superior pole (arrows).