Patients with Meigs syndrome usually present with symptoms related to pleural effusions and ascites and, in case of premenopausal women, with menstrual symptoms. They can also a have a family history of ovarian cancer. The most common presenting symptoms include fatigue, dyspnea that is initially present only with exertion, weight gain associated with swelling in the abdomen, a dry cough and menstrual symptoms such as amenorrhea or irregular cycles.

On physical exam, they may also have the following signs: tachypnea, tachycardia, decreased tactile fremitus, decreased breath sounds mostly on the right side but can also be present on the left, decreased vocal resonance and a shifting dullness or a fluid thrill in the abdomen indicating the presence of ascites. The majority of patients also present with a large, solid mass in the pelvis that is usually not associated with any symptoms and that is most commonly left sided but can also be bilateral.

• Fatigue

  These range from shortness of breath, fatigue and swelling in the abdomen to weight gain and irregular menses. Physical exam is important for the diagnosis of Meigs syndrome. [symptoma.com]

  Early clinical features include bloating, fatigue, and shortness of breath. Computed tomography is the imaging modality of choice for Meigs syndrome. [wikidoc.org]

  Changes in fatigue, autonomic functions, and blood biomarkers due to sitting isometric yoga in patients with chronic fatigue syndrome. [bpsmedicine.biomedcentral.com]

  Symptoms The symptoms and signs of Meigs syndrome consist of: Shortness of breath Fatigue Increased abdominal size Weight loss Amenorrhea for premenopausal women Bloating Dullness of percussion of lungs Tachycardia Present of ascites Present of pelvic [syndrome.org]

  CASE REPORT: A postmenopausal 50yrs old para3 presented with weakness, fatigue, decreased appetite, indigestion, abdominal pain and distension with loss of appetite and breathlessness since months. [jemds.com]

• Asymptomatic

  After removal of the ovarian tumor, the symptoms resolved and the patients became asymptomatic. In daily practice, Meigs' syndrome is at first sight often mistaken for ovarian cancer. [ncbi.nlm.nih.gov]

  The majority of patients with Meigs syndrome are asymptomatic. Early clinical features include bloating, fatigue, and shortness of breath. Computed tomography is the imaging modality of choice for Meigs syndrome. [wikidoc.org]

  The gold standard treatment is laparotomy and, by definition of the syndrome, after tumor removal, the symptoms resolves and the patients become asymptomatic. [academic.oup.com]

• Pleural Effusion

  Surgical removal and chemotherapy against the ovarian cancer resulted in disappearance of the ascites and pleural effusion, establishing a diagnosis of pseudo-Meigs’syndrome. [jstage.jst.go.jp]

  […] accumulated as pleural effusion. [bmcpulmmed.biomedcentral.com]

  Origin of pleural effusion The etiology of pleural effusion is unclear. Efskind and Terada et al theorize that ascitic fluid is transferred via transdiaphragmatic lymphatic channels. [emedicine.medscape.com]

  Symptoms related to pleural effusion include dyspnea, dry cough, and pleurisy. The pleural effusions are usually right-sided, even though the left and bilateral effusions are possible. [ncbi.nlm.nih.gov]

• Aspiration

  Procedural tests such as aspiration of pleural and ascitic fluids can relieve the bothering symptoms as well as provide samples of the fluid that may be sent for further analyses. [symptoma.com]

  Paracentesis and aspiration of pleural fluid: These procedures also help to relieve symptoms. Fluid should be sent for cytology. This is very important in distinguishing malignant ascites from Meigs' syndrome. [patient.info]

  In the presence of massive pleural effusion it may at times be impossible, even after partial aspiration, to exclude the presence of pulmonary metastasis by x-ray examination. [jamanetwork.com]

  The importance of recognising the syndrome lies in two facts, ( a ) the condition is usually a benign one, and ( b ) the ascites and hydrothorax clear up immediately following removal of the tumour and repeated aspiration is unnecessary. [birpublications.org]

  The aspiration of 15 liters of a brownish liquid permitted a midline laparotomy of limited size, under and above the umbilicus. The aspiration was completed (23 liters) and a right ovarian cyst was identified (Figures 5, 6). [jri.ir]

• Withdrawn

  Median volume of withdrawn pleural fluid was 2950 (1500-6000) mL. The classification of pleural effusion with the use of Light's criteria was possible in only 7 patients. In 6 of these patients pleural effusion met the criteria for an exudate. [ncbi.nlm.nih.gov]

  There was also a significant negative correlation between the volume of withdrawn pleural effusion and serum protein concentration, r = −0.62, P < 0.001. [journals.lww.com]

• Hematuria

  CASE REPORT Female patient, 73 year-old, admitted with hematuria, weight loss and dyspnea. Examination of the thorax showed reduction of the vesicular murmur in the right pulmonary base. In the right flank a non-painful mass was palpable. [scielo.br]

  This patient had pleural effusion, renal involvement (abnormal creatinine, hematuria, and proteinuria), hemocyte decrease, hypocomplementemia, and raised antibody titer (ANA cytoplasm 1:1000 [+], anti-dsDNA antibody: 109.91 IU/ml, anti-SSA 114 [+++]). [journals.lww.com]

• Stroke

  Shen Y, Shi L, Nauman E, Katzmarzyk P, Price-Haywood E, Bazzano A, Nigam S and Hu G (2019) Inverse Association Between HDL (High-Density Lipoprotein) Cholesterol and Stroke Risk Among Patients With Type 2 Diabetes Mellitus, Stroke, 50:2, (291-297), Online [ahajournals.org]

  Overview Metabolic syndrome is a cluster of conditions that occur together, increasing your risk of heart disease, stroke and type 2 diabetes. [mayoclinic.org]

  DBS is associated with a small risk of stroke, infection, and the development of new neurological signs and symptoms. [blepharospasm.org]

  Friedhelm Hummel, EPFL 09:15 – 09:35 Personalizing neurorehabilitation after stroke: first insights from the AVANCER clinical trial Claudia Bigoni, EPFL 09:35 – 09:55 Interactions between the Human Primary Visual and Motor Cortices: a TMS study Stefano [hnp.fcbg.ch]

Workup of patients with Meigs syndrome is broad and involves a range of tests to detect abnormalities in laboratory values as well as imaging and procedural tests to localize and identify the tumors involved. A complete blood count is required and low hemoglobin should prompt additional investigations. It is mostly associated with iron deficiency anemia, thus the need to measure reticulocyte count, serum iron levels, ferritin and total iron binding capacity. Anemia prior to surgery is an emergency and can be immediately treated with blood transfusions. Iron therapy can be then administered postoperatively.

A basic metabolic profile is necessary prior to surgery and includes measurements of sodium, chloride, potassium, blood urea nitrogen, bicarbonate, creatinine and glucose serum levels. Prothrombin time is assessed to detect any possible coagulopathies and, if present, fresh frozen plasma or vitamin K injections are administered. Serum cancer antigen (CA-125), a marker for several tumors (particularly ovarian tumors) is usually elevated although its increase is not related to malignancy [6]. It can also be elevated in pleural and ascitic fluids but normal levels of CA-125 can also be present even in malignancy [7] [8]. 

Diagnostic imaging tests include a chest X-ray to detect any pleural effusions, an ultrasound of of the abdomen and the pelvis to identify ascites as well as ovarian masses and a computed tomography (CT) to rule out distant metastasis. The CT may additionally detect tumors in the ovaries, uterus, fallopian tubes or broad ligament masses [9].

Therapeutic and diagnostic procedures include a paracentesis or an aspiration of pleural fluids. These usually help in relieving symptoms and make it possible to distinguish malignant ascites from Meigs syndrome. Ascitic and pleural fluids are commonly transudative but they can also be exudative, and protein content is frequently elevated with ovarian cancer. Samples from the ascites and the pleural effusions are sent for laboratory tests to evaluate the levels of protein, glucose, amylase, cell counts and the presence of microorganisms.

• Right Pleural Effusion

  Right pleural effusion was found on admission to our hospital in October 2002. Continuous thoracic drainage obtained 500-2000 ml of fluid per day. [ncbi.nlm.nih.gov]

  Meigs syndrome is the constellation of ascites, ovarian fibroma and right pleural effusion. [1] Mnemonic FAR : Fibroma. Ascites. Right pleural effusion. References ↑ Novoa Vargas, A.; Tenorio González, F.; Gómez Pedroso, J.; Torres Martínez, A. [librepathology.org]

• Left Pleural Effusion

  Abstract An 11-year-old white girl presenting with fever, dyspnea, and cough demonstrated a left pleural effusion, ascites, and a suprapubic mass on physical and radiologic examinations. [jpedsurg.org]

  Uterine leiomyoma causing massive ascites and left pleural effusion with elevated CA 125: A case report. J Obstet Gynecol Res 2000; 26 (4): 283-287 Giannakopoulos K, Giannakopoulou CH, Matalliotakis I, Neonaki M, Papanicolaou N, Koumantakis E. [jmscr.igmpublication.org]

  Uterine leiomyoma causing massive ascites and left pleural effusion with elevated CA 125: A case report. J Obstet Gynaecol Res 2000 Aug;26(4):283–7. [ CrossRef ] [ Pubmed ] [ijcasereportsandimages.com]

  Uterine leiomyoma causing massive ascites and left pleural effusion with elevated CA 125: A case report. J Obstet Gynecol Res 2000; 26 (4): 283-287 12. Dunn JS Jr, Anderson CD, Method MW, Brost BC. [ispub.com]

• Pleural Effusion on Chest X-Ray

  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. [clinmedjournals.org]

  effusion in chest X-ray (Figure 1) nor ascites or mass in abdominopelvic ultrasonography. [ovarianresearch.biomedcentral.com]

• Ovarian Mass

  Figure 2 Macroscopic pictures of the ovarian mass specimen. (A) Uncut (firm, solid mass). (B) Cross-section of the specimen. [doi.org]

  BACKGROUND: Adnexal masses are not uncommon in patients with breast cancer. Breast cancer and ovarian malignancies are known to be associated. [ncbi.nlm.nih.gov]

• Pleural Effusion

  Surgical removal and chemotherapy against the ovarian cancer resulted in disappearance of the ascites and pleural effusion, establishing a diagnosis of pseudo-Meigs’syndrome. [jstage.jst.go.jp]

  […] accumulated as pleural effusion. [bmcpulmmed.biomedcentral.com]

  Origin of pleural effusion The etiology of pleural effusion is unclear. Efskind and Terada et al theorize that ascitic fluid is transferred via transdiaphragmatic lymphatic channels. [emedicine.medscape.com]

  Symptoms related to pleural effusion include dyspnea, dry cough, and pleurisy. The pleural effusions are usually right-sided, even though the left and bilateral effusions are possible. [ncbi.nlm.nih.gov]

• Pleural Exudate

  When the protein concentration > 3.0 g/dL was applied as a criterion of pleural exudate, 88.8% (80/90) of effusions were classified as exudates. [ncbi.nlm.nih.gov]

  We reported a 38-year-old obese woman who was admitted for massive right-side pleural effusion. The analysis of the pleural fluid revealed an exudate with lymphocyte predominance and an increased adenosine deaminase (ADA) level (49 IU/L). [tmu.pure.elsevier.com]

  When pleural fluid protein concentration > 3.0 g/dL was applied as a criterion of pleural exudate, 88.8% (80/90) of effusions were classified as exudates. [journals.lww.com]

Surgery is the cornerstone of treatment as Meigs syndrome is a benign condition that usually fully resolves after resection of the tumor [10]. Surgical treatment starts with an exploratory laparotomy and a frozen section of the tumor is sent for pathological analysis. If the pathology report indicates absence of malignant features, the surgeon can proceed with a salpingo-oophorectomy or an oophorectomy, although the choice of the procedure depends on the age and reproductive status of the woman. Premenopausal women usually undergo a unilateral salpingo-oophorectomy, while postmenopausal women can undergo a bilateral salpingo-oophorectomy with total hysterectomy or a bilateral or unilateral salpingo-oophorectomy. A wedge resection of the ovaries along with a unilateral salpingo-oophorectomy is the treatment of choice in prepubertal girls.

An ovarian mass that is associated with elevated levels of CA-125 is malignant until proven otherwise. Malignancy is ruled out with negative cytology, benign histological reports and absence of any peritoneal implantation. It is critical to consult a gynecologic surgeon and a gynecologic oncologist during the treatment process.

Non-surgical treatment with thoracentesis and paracentesis can additionally help in relieving symptoms. 

Meigs syndrome is a benign disease with an excellent prognosis if adequately diagnosed and treated, even when some features resemble more ominous malignant disorders. Patients with Meigs syndrome also tend to have similar life expectancy to patients in the general population. Fertility is usually not affected if the ovaries are still functional. It is important to note that the resolution of ascites and pleural effusions after surgery is a defining characteristic of the disease.

The causative mechanisms responsible for pleural effusions in Meigs syndrome remain unknown and seem to be independent of the amount of ascites. Samanth and Black propose that the ascitic fluid is directly secreted from the tumors and report that ovarian tumors only larger than 10 cm and with a myxoid component are associated with ascites [7]. On the other hand, Efskind and Terada suggest that pleural effusions result from the passage of fluid in the lymphatic channels through the diaphragm. Other mechanisms that are thought to be involved include the presence of mechanical pressure on the lymphatic channels and vessels, hormonal activation and tumor torsion. 

Meigs syndrome is a rare condition and is very uncommon before the age of 40 [5]. It becomes more frequent with age although some studies report teratomas and cystadenomas in prepubertal girls. The incidence of ovarian tumors is more elevated in women with higher socioeconomic status. It increases in the third decade of life and keeps on progressively increasing after menopause, with an average of 50 years of age. Ovarian fibromas represent 2% to 5% of tumors removed surgically, with Meigs syndrome occurring in 1% to 2% of them. Ascites occurs in 10-15% of women with ovarian fibromas while hydrothorax is associated with larger lesions and has an incidence of 1%. Life expectancy in Meigs syndrome is similar to the general population after surgical removal of the tumor and the risk of progression from fibroma into fibrosarcoma is 1%.

The pathophysiology underlying ascites remains uncertain. It is hypothesized that contact of the peritoneal surface with a hard mass such as an ovarian solid tumor stimulates peritoneal fluid production. The development of ascites can also be caused by a secretion of mediators that increase capillary permeability such as activated complements, histamines and fibrin degradation products.

The fluids that collect within the pleura and the peritoneum can either be transudative or exudative. Both pleural and ascitic fluids tend to have a similar composition. It is usually tumor size and not its histological features that determines the presence and consistency of pleural effusions and ascites.

There are no current preventive measures for Meigs syndrome.

Meigs syndrome is defined by the simultaneous presence of a solid ovarian tumor along with ascites and pleural effusions. In the majority of cases, the tumor involved is a fibroma. A diagnosis of Meigs syndrome can only be established after a complete exclusion of other potential malignant causes [1]. Pseudo-Meigs is a similar condition that involves benign tumors other than fibromas. These tumors are not limited to the ovaries and may be present in the uterus or fallopian tubes and may also consist of mature teratomas, struma ovarii or ovarian leiomyomas. Some cases involve ovarian or gastrointestinal metastatic tumors [2] [3] [4].

The underlying etiological and physiological mechanisms are still not completely elucidated. Some hypothesize that pleural effusions result from the passage of ascitic fluid through transdiaphragmatic channels. On the other hand, ascites is thought to be caused direct pressure from the ovarian tumor over the peritoneum. Neurochemical mediators such as histamine, fibrin degradation products and activated complements are also involved. These increase vascular permeability and ultimately lead to fluid extravasation within the peritoneum.

Incidence and prevalence of Meigs syndrome are low, with the condition occurring in only 1 to 2% of all cases with ovarian fibromas. The latter constitute 2 to 5% of ovarian malignancies treated surgically. 

Patients with Meigs syndrome usually present with symptoms that are related to the ascites and the pleural effusions such as weight gain, fatigue, shortness of breath or abdominal swelling. Physical exam shows tachypnea, tachycardia, decreased breath sounds and tactile fremitus. Abdominal exam reveals fullness and dullness over the flank area because of the presence of ascites. Diagnosis is established after performing a biopsy and ruling out all malignant causes. Although a paracentesis or a pleural aspiration can provide symptomatic relief, surgical resection of the tumor is the cornerstone of the treatment. The type of surgery performed depends on the age of the patient as well as her reproductive status.

Meigs syndrome is defined by the presence of an ovarian tumor along with an accumulation of fluid in the abdomen (ascites) and effusions around the lungs (pleural effusions). The ovarian tumor involved in Meigs syndrome is usually a fibroma, a benign tumor. Cases that involve other types of ovarian tumors, such as a thecoma, cystadenoma or a granulosa cell tumor, are called pseudo-Meigs syndrome. The mechanisms underlying the development of Meigs syndrome are still not well understood, although it is thought that the ascites and the pleural effusions result from inflammatory reactions triggered by the tumor. Most women affected are older than 40, with the risk further increasing with age. Very rare tumors in prepubertal girls have also been reported.

Patients with Meigs syndrome usually present with symptoms related to the ascites and the pleural effusions. These range from shortness of breath, fatigue and swelling in the abdomen to weight gain and irregular menses. Physical exam is important for the diagnosis of Meigs syndrome. Dullness to percussion of the chest, decreased breath sounds and decreased tactile fremitus (transmitted sounds in the lungs) can indicate pleural effusions. Careful abdominal and pelvic examinations may detect a tumor beneath the ascites. Ascites usually manifests with fullness and dullness in the flank and abdominal areas.

Laboratory and imaging tests are broad and aim to detect the ovarian tumor as well as to characterize the ascitic and pleural fluids. Routine blood tests such as a complete blood count, liver function tests and assessment of proteins in the blood are performed. In addition, a specific marker called CA-125 is measured and is usually more elevated in ovarian cancer. A urinalysis can also be performed to detect proteins in the urine. Imaging tests such as an ultrasound, a CT scan or an MRI of the abdomen and pelvis can reveal the ascites and confirm the presence of the tumor. A chest X-ray may also detect the pleural effusions.

Procedural tests such as aspiration of pleural and ascitic fluids can relieve the bothering symptoms as well as provide samples of the fluid that may be sent for further analyses. Cytologic tests on these samples help in distinguishing Meigs syndrome from frank malignant conditions.

Surgery is the cornerstone of treatment and the disease commonly resolves after the ovarian mass is removed. The type of surgery depends on the age and reproductive status of the women. A wedge section of the ovaries is usually performed in girls before the age of pubery whereas women of reproductive age undergo a resection of the ovary and associated fallopian tube (salpingo-oophorectomy). Women who are in the menopausal period can additionally undergo a full removal of the uterus, both ovaries and the fallopian tubes (bilateral salpingo-oophorectomy with hysterectomy).

1. Riker D, Goba D. Ovarian mass, pleural effusion, and ascites: revisiting meigs syndrome. J Bronchology Interv Pulmonol. 2013 Jan; 20(1):48-51. 
2. Dunn JS Jr, Anderson CD, Method MW. Hydropic degenerating leiomyoma presenting as pseudo-Meigs syndrome with elevated CA 125. Obstet Gynecol. 1998 Oct; 92(4 Pt 2):648-9. 
3. Iavazzo C, Vorgias G, Sampanis D, et al. Meig's or Pseudomeig's syndrome? Bratisl Lek Listy. 2007; 108(3):158-60.
4. Schmitt R, Weichert W, Schneider W, Luft FC, Kettritz R. Pseudo-pseudo Meigs' syndrome. Lancet. 2005 Nov 5; 366(9497):1672.
5. Agaba EI, Ekwempu CC, Ugoya SO, et al; Meigs' syndrome presenting as haemorrhagic pleural effusion. West Afr J Med. 2007 Jul-Sep; 26(3):253-5.
6. Moran-Mendoza A, Alvarado-Luna G, Calderillo-Ruiz G, et al. Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature. Int J Gynecol Cancer. 2006 Jan-Feb; 16(1):315-8
7. Jones OW, Surwit EA. Meigs syndrome and elevated CA 125. Obstet Gynecol. 1989 Mar. 73(3 Pt 2):520-1.
8. Meigs JV, Cass JW. Fibroma of the ovary with ascites and hydrothorax: with a report of seven cases. Am J Obstet Gynecol. 1937; 33:249-267.
9. Lanitis S, Sivakumar S, Behranwala K, et al. A case of Meigs syndrome mimicking metastatic breast carcinoma. World J Surg Oncol. 2009 Jan; 22: 7-10.
10. Samanth KK, Black WC. Benign ovarian stromal tumors associated with free peritoneal fluid. Am J Obstet Gynecol. 1970 Jun 15; 107(4):538-45.