Different strokes for different folks
A 42-year-old woman presented with acute onset of dyspnea, chest pain, and fever of 10 days duration. She had insignificant past medical record. She was apparently healthy prior to 2 weeks. Her illness started after exposure to large amount of hay dust, which led to episode of sneezing. She developed acute onset of breathing difficulty and dry cough after that episode. She received treatment at local hospital with no symptomatic benefit. Her condition continued to deteriorate; she had new onset of dyspnea and started having fever. So, she was referred to our hospital for further management. She denied any history of hemotpysis, bleeding tendency, epistaxis, telengectasis, immobilization, hypertension, and catamenial hemoptysis. She was not on any medications like antiplatelets or anticoagulants. She denied any history of recent trauma or surgery. Family history was not contributory.\nThe patient was restless and anxious. Vital signs were as follows: Temperature: 37.8°C; heart rate: 114 beats/min; blood pressure: 102/60 mm Hg; respiratory rate: 30 breaths/min, and oxygen saturation: 95% while breathing on 10 L of oxygen. There was no clubbing, cyanosis, lymphadenopathy, edema of extremities, mucosal or vascular abnormalities, (telangiectasia) or visible thyroid swelling. Respiratory system examination was consistent with left-sided pleural effusion. The abdomen was soft with no appreciable organomegaly. The patient was alert and oriented with no neurologic deficits.\nLaboratory findings included the following: Hemoglobin: 7.73 g/dL; hematocrit: 23%; white blood cell count: 30.5 × 103/mm3; 88% neutrophils; platelet count: 811 × 103/mm3; creatinine level: 0.7 mg/dL; total bilirubin 2.98 mg/dL; alanine aminotransferase: 52.4 IU/L; aspartate amino transferase: 82.3 IU/L; alkaline phosphatase: 95.5 IU/L; prothrombin time with international normalized ratio: 14.9/14.60/1.04 s, activated partial thromboplastin time: 40.5/32.2 s. Chest radiography demonstrated left opaque hemithorax with contralateral mediastinal shift [].\nDiagnostic pleural aspiration revealed hemorrhagic fluid with hematocrit of 18%. Diagnosis of spontaneous hemothorax was made. (Serum hematocrit: 23%). Emergency multidetector computed tomography (MDCT) chest was done which confirmed left-sided hemothorax (high attenuation) with underlying collapsed left lung. It also showed a 2 × 1 cm, tortuous, tubular structure visualized in left lingular lobe suggestive of pulmonary arteriovenous malformation/venous varix []. She was subjected to left posterolateral thoracotomy. Left lingular lobe showed two nodules of approximately 3 × 2 cm in size; left lingular lobe was friable warranting lobectomy. Intercostal chest drain was inserted following the procedure. Entire specimen was sent for histopathology, which showed multiple large and small vascular spaces with variable smooth muscle layer, with absence of elastic lamina, suggesting arteriolization of veins. The histopathology findings were consistent with PAVM [].\nPostoperative period was uneventful and she was successfully discharged on 8th postoperative day. Genetic testing for hereditary hemorrhagic telangiectasia was suggested for her as well as her family members.