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Question

A $60$ year old male with Marfan's syndrome comes to emergency department with severe generalised tearing pain localised to chest anteriorly and inter scapular region, sweating and weakness. On examination, his BP is $200/140$ mm Hg. X ray chest shows widening of superior mediastinum. ECG shows sinus tachycardia. What is the most likely diagnosis?

The correct answer is
Dissection of aorta

Diagnosis of Aortic Dissection in Marfan Syndrome

This case involves a 60-year-old male with known Marfan syndrome presenting with acute, severe symptoms. Key findings must be correlated to determine the most likely diagnosis.

Analyzing Patient Presentation

  • Patient History: 60-year-old male with Marfan syndrome. Marfan syndrome is a significant risk factor for aortic dissection due to connective tissue weakness.
  • Symptoms: Severe generalized tearing pain, localized to the chest anteriorly and radiating to the interscapular region. This type of pain is highly suggestive of aortic dissection. Associated symptoms include sweating and weakness.
  • Vital Signs: Blood pressure is extremely high ($200/140$ mm Hg), which can be both a cause and a consequence of aortic emergencies.
  • Radiological Findings: Chest X-ray shows widening of the superior mediastinum. This is a classic sign indicating potential aortic pathology, such as dissection or aneurysm.
  • ECG: Sinus tachycardia is noted, a non-specific finding common in acute distress or pain.

Evaluating Differential Diagnoses

  • Acute pulmonary embolism: While causing chest pain and tachycardia, the characteristic 'tearing' pain and widened mediastinum are less typical.
  • Dissection of aorta: This diagnosis aligns perfectly with the patient's history of Marfan syndrome, the classic tearing chest pain radiating to the back, severe hypertension, and the widened mediastinum on X-ray.
  • Acute myocardial infarction: Typically presents with crushing chest pain, and ECG changes are usually expected. Widened mediastinum is not a feature.
  • Acute pericarditis: Often causes sharp, pleuritic chest pain, and ECG typically shows diffuse ST elevation and PR depression. Widened mediastinum is not associated.

Conclusion

The combination of Marfan syndrome, severe tearing chest pain radiating to the back, significant hypertension, and widening of the superior mediastinum strongly points towards Dissection of aorta as the most likely diagnosis.

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Important Questions from General Clinical Conditions

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    2. Narcotic drugs
    3. Iatrogenic-Post RT/Surgery
    4. Hypothyroidism
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