What is the best management approach for Meigs syndrome?

Management of Meigs Syndrome

Definition and Clinical Features

Meigs syndrome is a rare condition characterized by the triad of:

• Benign ovarian tumor (typically fibroma or fibrothecoma)
• Ascites
• Pleural effusion

All of these manifestations resolve spontaneously after surgical removal of the tumor 1, 2.

Diagnosis

Surgical resection of the ovarian mass is the definitive management approach for Meigs syndrome. 1, 2, 3

The diagnostic workup should include:

• Imaging studies:

  • Chest CT scan to confirm pleural effusion
  • Abdominal/pelvic ultrasound or MRI to identify the ovarian mass
  • Evaluation for ascites

• Laboratory tests:

  • Serum CA-125 (often elevated but not diagnostic)
  • Other tumor markers like HE-4 may be elevated 1
  • Complete blood count (anemia may be present) 3

• Fluid analysis:

  • Thoracentesis to rule out malignancy or infection
  • Paracentesis if significant ascites is present

Management Algorithm

1. Initial Assessment:

   • Evaluate severity of symptoms (dyspnea, abdominal distention)
   • Assess hemodynamic stability
   • Determine if emergency intervention is needed

2. Preoperative Management:

   • Thoracentesis for symptomatic relief of severe dyspnea 2, 4
   • Correction of anemia if present 3
   • Optimization of respiratory status

3. Definitive Treatment:

   • Surgical removal of the ovarian tumor (gold standard) 1, 2, 3, 4, 5
   • Options include:
     • Laparoscopic approach for smaller tumors 3
     • Laparotomy for larger tumors or when malignancy is suspected
     • Unilateral salpingo-oophorectomy or ovarian mass excision depending on patient age and tumor characteristics

4. Postoperative Care:

   • Monitor for resolution of pleural effusion and ascites (typically resolves within days after tumor removal) 1, 2
   • Follow-up imaging to confirm resolution
   • Monitor tumor markers for normalization 1

Special Considerations

• Elevated tumor markers: CA-125 and HE-4 may be significantly elevated, mimicking ovarian malignancy. This should not deter surgical management, as these markers normalize after tumor removal 1.

• Differential diagnosis: Must be distinguished from malignant ovarian tumors with peritoneal and pleural metastases 2, 4.

• Associated conditions: Rare cases may present with pericardial effusion 5 or hemolytic anemia 3.

• Surgical approach: Laparoscopic surgery is feasible and safe even for large tumors when performed by experienced surgeons, offering faster recovery and shorter hospitalization 3.

Prognosis

The prognosis of Meigs syndrome is excellent with appropriate surgical management:

• Complete resolution of ascites and pleural effusion typically occurs within days after tumor removal 1, 2, 5
• Tumor markers normalize within months 1
• No recurrence is expected as the underlying tumor is benign 1, 2

Pitfalls to Avoid

1. Misdiagnosis as malignancy: Elevated CA-125 and presence of effusions may lead to incorrect diagnosis of advanced ovarian cancer 1, 4.

2. Inappropriate neoadjuvant chemotherapy: Some patients receive unnecessary chemotherapy due to suspicion of malignancy 1.

3. Misdiagnosis as heart failure: Pleural effusion and edema may be mistaken for cardiac conditions 4.

4. Delayed diagnosis: Focusing only on the effusions without investigating the underlying ovarian mass can delay proper treatment 2, 5.