The Rendu-Osler-Weber Disease Revealed by a Refractory Hypoxemia and Severe Cerebral Fat Embolism

The Rendu-Osler-Weber disease is a genetic disease which may lead to severe hemorrhage and less frequently to severe organ dysfunction. We report the case of a 22-year-old patient with no personal medical history who was involved in a motorcycle accident and exhibited severe complications related to large arteriovenous pulmonary shunts during his ICU stay. The patient developed an unexplained severe hypoxemia which was attributed to several arteriovenous shunts of the pulmonary vasculature by a contrast study during a transesophageal echocardiographic examination. The course was subsequently complicated by a prolonged coma associated with hemiplegia which was attributed to a massive paradoxical fat embolism in the setting of an untreated femoral fracture. In addition to hemorrhagic complications which may lead to intractable shock, arteriovenous malformations associated with the Rendu-Osler-Weber disease may involve the pulmonary vasculature and result in unexpected complications, such as hypoxemia or severe cerebral fat embolism in high-risk patients. 1. Introduction Although epistaxis is usually the first symptom of the Rendu-Osler-Weber disease, the severity of vascular lesions and related organ dysfunctions increases with age [1, 2]. We report the case of a young blunt trauma patient whose disease was revealed by life-threatening complications related to large pulmonary arteriovenous shunts. 2. Case Report A 22-year-old motorcyclist without medical history was involved in a violent head-on collision. The patient was initially conscious; he had no motor deficit and no hemodynamic or respiratory compromise. Contrast-enhanced body CT scan ruled out a head trauma but disclosed multiple facial fractures, a mandibular fracture, a rounded opacity in the left pulmonary base consistent with an arteriovenous shunt, a hepatic contusion, a fractured left iliac crest, and a closed fracture of the right femoral diaphysis. The patient was promptly referred to the operating suite where his facial wounds were sutured and a maxillomandibular fixation was placed. The femoral osteosynthesis was postponed due to unstable hemodynamics, and the right lower limb was immobilized with a traction. Hemodynamics were stabilized through the transfusion of red cells and plasma units. The patient was admitted to the ICU with normal blood pressure (120/75？mmHg), body temperature (36.7°C), and blood oxygenation (SpO2: 99%). An abrupt hypotension occurred (60/35？mmHg) in conjunction with sinus tachycardia (130？bpm), hyperthermia (40°C), and marked oxygen desaturation (SpO2: 81%).