Different strokes for different folks.

A 42-year-old woman, presented to our emergency room with chief complaints of acute onset of dyspnea, left-sided chest pain, and respiratory distress. On arrival, she had blood pressure of 90/50 mm Hg, respiratory rate of 40/min, and oxygen saturation of 95% breathing on 10 L oxygen. She was in significant respiratory distress. On examination, she was found to have diminished breath sounds on the left side of chest. Her chest radiograph showed left massive pleural effusion causing contralateral shift of mediastinum. Diagnostic pleural aspiration was done; results were consistent with hemothorax. Her multidetector computed tomography chest showed features of left-sided hemothorax (high attenuation) along with a 2 cm-enhancing lobulated opacity in the left lingular lobe, suggestive of pulmonary arteriovenous malformation (PAVM). She was successfully treated with surgical resection of PAVM.

INTRODUCTION

Pulmonary arteriovenous malformation (PAVM) is an abnormal communication between pulmonary arteries and veins. Approximately, 30-90% of PAVM are caused by autosomal dominant genetic disorder, hereditary hemorrhagic telangiectasia (HHT), also known as Osler—Weber--Rendu disease. It was first described in 1897.[1] Clinical manifestations of PAVM can be subtle and this condition may remain undiagnosed until complications arise in adulthood. Spontaneous, massive hemothorax is a life-threatening complication of PAVM. Rapid identification and appropriate treatment can be lifesaving. We present a case of a young woman, who presented with spontaneous hemothorax, secondary to rupture of PAVM.

CASE REPORT

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.

Physical examination

The 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.

Laboratory and radiographic findings

Laboratory 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 [Figure 1].

Figure 1

Chest X-ray showing left-sided massive pleural effusion with contralateral shift of mediastinum

Treatment and clinical course

Diagnostic 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 [Figure 2]. 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 [Figure 3].

Figure 2

MDCT chest showing left massive pleural effusion with a 2 × 1 cm, tortuous, tubular structure in left lingular lobe and two small nodules

Figure 3

Microscopic image of lobectomy specimen of lung (hematoxyline-eosin, original ×10), showing arteriolization of veins with surrounding areas of hemorrhage

Postoperative 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.

DISCUSSION

Hemothorax is defined as pleural fluid with hematocrit of more than 50% of the peripheral blood hematocrit.[1] The causes of hemothorax include malignancies, anticoagulant medications, vascular ruptures (aortic dissection, arteriovenous malformations) endometriosis, pulmonary infarctions, adhesions with pneumothorax, and hematologic abnormalities such as hemophilia.[1] PAVM is direct communication between pulmonary artery and veins without intervening capillary bed.[234] Hemothroax is rare but fatal complication of PAVM. Hemothorax can be presenting feature of PAVM in 1-3% of cases. A total of 30-70% of PAVM are caused by an autosomal dominant disorder, HHT, also known as Osler—Weber--Rendu disease.[5] In largest series of PAVM of 143 cases, in five cases, the presenting feature of PAVM was hemothorax.[2] In the same series, the authors found that 50% of reported cases were in pregnant women.[2] Pregnancy is a risk factor for hemothorax in patients with PAVM.[1-4] MDCT angiography (MDCTA) is the investigation of choice for patients presenting with hemothorax.[1] Transcatheter embolization is treatment of choice for patients of PAVM presenting as hemothorax.[6] However, surgical resection of PAVM is indicated in patients who have intrapleural rupture of PAVM.[7] Our patient presented with significant hypoxia and septic shock. She was not fit for transcatheter embolization and was directly taken up for surgical correction. In case of hemothorax secondary to vascular anomaly, the anomaly should be corrected prior to removal of fluid; as tamponade effect provided by hemothorax, if removed, can lead to catastrophic bleed.[8]

CONCLUSION

PAVM should be considered in differential diagnosis of spontaneous hemothorax. In case of undiagnosed hemothorax, MDCTA is the investigation of choice. In case of vascular anomalies causing hemothorax, it is advisable to treat anomalies prior to doing thoracocentesis, as tamponade effect provided by hemothorax, if removed, may cause catastrophic bleed.