Meigs syndrome an unusual cause of dyspnea in

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Meigs syndrome: an unusual cause of dyspnea
in an emergency department
Servicio de Urgencias del Hospital Universitario Reina Sofía de Córdoba, Spain.

CORRESPONDENCE:                  Breathlessness is among the most common complaints mentioned to physicians in an
Rafael Calvo Rodríguez           emergency department. Respiratory and heart diseases are the most frequent causes.
Pasaje José Manuel Rodríguez     We report a case of dyspnea due to a rare disease, Meigs syndrome, or the presence of a
López, 7, 1º-1ª                  benign ovarian tumor with ascites and pleural effusion. Chronic abdominal hypertension
14005 Córdoba, Spain.            led to breathlessness, which was one of the most important presenting symptoms. The
                                 patient was initially diagnosed with decompensated heart failure. Later, Meigs syndrome
                                 was diagnosed and treated. [Emergencias 2010;22:206-208]
                                 Key words: Meigs syndrome. Dyspnea. Ovarian tumor.


Introduction                                                    ral effusion, hepatomegaly, no pain, ascites and
                                                                pretibial edema.
    Breathlessness is one the most common com-                      Chest X-ray showed right pleural effusion (Fig-
plaints mentioned to physicians in the emergency                ure 1), echocardiography showed non-dilated left
department (ED). We present a case with the rela-               ventricle with hyperdynamic systolic function,
tively rare Meigs syndrome (characterized by the                anomalous septal movement and characteristic
triad ascites, pleural effusion and a benign ovarian            signs of pericardial constriction, mitral filling rate
tumor) added to a highly unusual symptom of                     with relaxation deficits without alteration of
presentation: dyspnea. The case was diagnosed in                breathing movements. The ascending aorta was
our ED; it serves to highlight the clinical similari-           dilated. Pericardial and pleural effusion were mini-
ties this process may share with decompensated                  mal.
heart failure, and also the relevance even today of                 Chest computerized tomography (CT) scan
clinical symptoms for suspected diagnosis in the                showed no other significant findings.
ED.                                                                 She presented esophageal hiatus hernia with
                                                                gastric content, increased calibre esophagus with
                                                                wall thickening, and ascites. Endomyocardial biop-
Case report                                                     sy and right hemodynamic study were performed,
                                                                and the patient was diagnosed at discharge with
   A 64-year old woman with no history of inter-                diastolic heart failure by constrictive cardiomyopa-
est except previous hospitalization in the depart-              thy vs constrictive pericarditis.
ment of Cardiology of our hospital because of                       One month later she consulted the ED for
moderate effort dyspnea during one month with-                  worsening symptoms despite diuretic therapy. Pal-
out other clinical data. She also reported in-                  pation revealed an abdominal mass in the right ili-
creased abdominal girth and edema of the lower                  ac fossa, suggestive of ovarian tumor. Laboratory
limbs.                                                          tests (blood count and biochemistry including ba-
   Physical examination showed jugular engorge-                 sic blood amylase, AST, ALT, GGT and ALP) were
ment, hepatojugular reflux; semiology right pleu-               normal. The new chest X-ray was similar to the

206                                                                                        Emergencias 2010; 22: 206-208
                                                   MEIGS SYNDROME: AN UNUSUAL CAUSE OF DYSPNEA IN AN EMERGENCY DEPARTMENT


                                                                Figure 2. Abdominal MRI showing a right adnexal tumor,
Figure 1. Posteroanterior x-ray of the chest showing right      suggestive of ovarian fibroma. (asterisk).
pleural effusion.

                                                                completely known, it is believed that pleural effu-
previous one (Figure 1). Electrocardiogram                      sion is due to the passage of peritoneal ascites flu-
showed no alterations and arterial blood gas                    id to the pleural cavity by lymphatic vessels or
analysis showed pO2 71 mmHg, pCO2 36 mmHg                       congenital defects of the diaphragm, which are
and pH 7.35. Abdominal ultrasound showed a                      most common on the right side2. Ascites appears
solid, right ovarian tumour. We consulted the de-               as a result of fluid secretion by the tumour or va-
partment of gynecology for suspected Meigs syn-                 soactive substances related with them3 (inflamma-
drome. The study was completed with abdominal                   tory cytokines, fibroblast growth and vascular en-
MRI (Figure 2) which showed a tumour occupy-                    dothelial       factor 4 ).  The     term     Meigs
ing the right upper pelvic region, of adnexal ori-              pseudosyndrome is used when the tumour is ma-
gin, suggestive of ovarian fibroma. Intravenous                 lignant5,6. In 1981, Bast et al7 associated the pres-
urography showed uretero-vesical compression by                 ence of elevated serum CA-125 with ovarian carci-
the tumour without signs of infiltration.                       noma. Although elevated serum CA-125 in
    After a preoperative diagnosis of ovarian fibro-            postmenopausal women with adnexal masses, as-
ma ovarian, exploratory laparotomy revealed a                   cites and pleural effusion is highly suggestive of
right ovarian mass with benign macroscopic fea-                 malignant ovarian tumour, in 1989 Jones et al.8
tures, and the patient underwent hysterectomy                   reported a case of Meigs syndrome with elevated
with bilateral oophorectomy.                                    CA-25. Peritoneal irritation, pleural effusion and
    After surgical treatment, there was a progres-              ascites production may contribute to increased
sive clinical improvement with disappearance of                 levels of serum antigen CA-1252,9. The diagnosis is
hydrothorax, ascites and normalization of serum                 primarily by preoperative ultrasound and abdomi-
CA 125 antigen and alfafetroprotein (initially                  nal CT. Treatment is surgical in all cases9. The di-
high) in the following weeks. The pathology re-                 agnosis is confirmed when, after removing the
port on the surgical specimen was ovarian fibro-                primary tumor, the ascites and pleural effusion re-
ma.                                                             solve and serum levels of CA-125 normalize11 in
                                                                subsequent weeks 12. The presentation of Meigs
                                                                syndrome with dyspnea symptoms has been de-
Discussion                                                      scribed once before13.
                                                                    Dyspnoea may be explained by pleural effu-
    Meigs syndrome was first described by Meigs                 sion of a certain magnitude, or abdominal hyper-
and Cass in 19371. It is characterized as a benign              tension (ABHT) secondary to the progressive in-
ovarian tumor (fibroma, tecoma, granulose cell                  crease in volume of the pelvic mass and ascites13.
tumours and Brenner tumour), together with as-                      ABHT is shown by the increase in abdominal
cites and pleural effusion, which resolves after sur-           content volume, which is initially compensated by
gical removal of the ovarian tumor. Pleural effu-               elasticity of the abdominal wall but ultimately this
sion is right sided in 70% of cases and bilateral in            is insufficient to maintain pressure in the normal
20%; sometimes, when it is massive, dyspnea is                  range (<12 mmHg) 14,15. There are two forms of
the alarm signal. Although its pathogenesis is not              ABHT: acute (abdominal trauma, hemoperi-

Emergencias 2010; 22: 206-208                                                                                       207
R. Calvo Rodríguez et al.

toneum, acute pancreatitis, peritonitis, and post-             many organs and systems, where the symptom
operative complications) and chronic develop-                  causing the visit may initially lead to erroneous
ment over months or years (pregnancy, abdomi-                  suspected diagnosis.
nal tumours, ascites, etc.)14,15. Both forms can lead
to abdominal compartment syndrome (intra-ab-
dominal pressure       20 mmHg), a surgical emer-              References
    The most accurate diagnosis of ABHT is based                1 Meigs JV, Cass JW. Fibroma of the ovary with asictes and hydrotho-
                                                                  rax: With a report of seven cases. Am J Obstet Gynecol.
on a combination of clinical findings and monitor-                1937;33:249-67.
ing intravesical pressure, although for obvious rea-            2 Agranoff D, May D, Jameson C, Knowles GK. Pleural effusion and a
                                                                  pelvic mass. Postgrad Med J. 1998;74:265-7.
sons most of the time this is made on a clinical                3 Abad A, Cazorla E, Ruiz F, et al. Meigs’ syndrome with elevated CA
basis alone. The development of chronic ABHT                      125: case report and review of the literature. Eur J Obstet Gynecol.
                                                                  Reprod Biol 1999;82:97-9.
may be suspected by the presence of collateral                  4 Abramov Y, Anteby SO, Fasouliotis SJ, Barak V. The role of inflamma-
cava-cava venous flow: flow from the inferior vena                tory cytokines in Meigs’ syndrome. Obst Gynecol 2002;99:917-9.
                                                                5 Meigs JV. Fibroma of the ovary with ascites and hydrothorax; Meigs’
cava is compressed into the superior vena cava,                   syndrome. Am J Obstet Gynecol. 1954;67:962-85.
through the internal mammary and epigastric                     6 Kazanov L, Ander DS, Enriquez E, Jaggi FM. Pseudo-Meigs’ Syndro-
                                                                  me. Am J Emerg Med. 1998;16:404-5.
veins. Voiding frequency indicates progressive de-              7 Bast RC, Feeney M, Lazarus H, Nadler LM, Colvin RB, Knapp RC. Re-
crease of bladder function caused by direct com-                  activity of menoclonal antibody with human ovarian carcinoma. J
                                                                  Clin Invest. 1981;68:1331-7.
pression by intrapelvic masses and slow increase                8 Jones OW, Surwit EA. Meigs syndrome and elevated CA 125. Obstet
of abdominal pressure13. In this patient, these clin-             Gynecol. 1989;73:520-1.
ical data were not observed, but uretero-vesical                9 Niloff JM, Knapp RC, Schaetzl E, Reynolds C, Bast RC. CA 125 anti-
                                                                  gen levels in obstetric and gynecologic patients. Obstet Gynecol.
compression shown by the urological study sup-                    1984;64:703-7.
ports this diagnosis.                                          10 Santangelo M, Battaglia M, Vescio G, Sammarco G, Gallelli G, Vetere
                                                                  A, et al. Meigs’ syndrome: its clinical picture and treatment. Ann Ital
    Thus, ABHT by ovarian mass and concurrent                     Chir. 2000;71:115-9.
ascites in Meigs syndrome is, in some cases such               11 Moran Mendoza A, Alvarade Luna G, Calderillo Ruiz G, Serrano Ol-
                                                                  vera A, Lopez Graniel CM, Gallardo Rincon D. Elevated CA 125 level
as that described here, the cause of dyspnea in                   associated with Meigs’ syndrome: case report and review of the lite-
these patients, although more often it is due to                  rature. Int J Gynecol Cancer. 2006;16 Supl 1:315-8.
                                                               12 Peparini N, Matteo FM, Silvestri A, Caronna R, Chirletti P. Abdominal
pleural effusion, which in our case was minimal.                  hypertension in Meigs’ syndrome, Eur J Surg Oncol. 2008;34:938-42.
Meigs syndrome should therefore be considered                  13 Malbrain MLNG, Cheatham ML, Kirkpatrik A, et al. Results from the
                                                                  international conference of experts on intra-abdominal hypertension
as one of the rare causes of dyspnea that may                     and abdominal compartment syndrome. I. Definitions. Intensive Ca-
present in the ED.                                                re Med 2006;32:1722-32.
                                                               14 Ivatury RR. Abdominal compartment syndrome: a century later, isn’t
    In our opinion, this case emphasizes the im-                  it time to accept and promulgate? Crit Care Med. 2006;34:2494-5.
portance of comprehensive vision by the ED                     15 Kimball EJ, Rollins MD, Mone MC, Baraghoski GK, Johnszon C, Day
                                                                  ES, et al. Survey of intensive care physicians on the recognition and
physician and thorough workup of patients, espe-                  management of intraabdominal hypertension and abdominal com-
cially in cases with signs and symptoms involving                 partment syndrome. Crit Care Med. 2006;34:2340-8.

Síndrome de Meigs como causa inusual de disnea en el servicio de urgencias
Calvo Rodríguez R, Aparicio Sánchez J, Frías Tejederas G, García Olid A, Montero Pérez FJ, Jiménez Murillo L
La disnea es uno de los síntomas con el que más frecuentemente se enfrenta el urgenciólogo. Las causas respiratorias y
cardiacas son las más frecuentes. Se presenta un caso de disnea que resultó ser originada por una causa bastante infre-
cuente. Un caso de síndrome de Meigs (presencia de un tumor ovárico benigno acompañado de ascitis y derrame
pleural), con hipertensión abdominal crónica que motivó que la disnea fuera uno de los síntomas de presentación más
relevantes. La paciente fue inicialmente diagnosticada de insuficiencia cardiaca descompensada, y posteriormente se
llegó al diagnóstico y tratamiento definitivos. [Emergencias 2010;22:206-208]

Palabras clave: Síndrome de Meigs. Disnea. Tumor ovárico.

208                                                                                               Emergencias 2010; 22: 206-208

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