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.
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 [
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%). Patient's level of consciousness rapidly deteriorated and a petechial rash was noted in the deltopectoral triangle and left eye conjunctiva. There was no evidence of sepsis and blood cultures remained sterile. Biological tests revealed the presence of a coagulopathy (prothrombin time: 46%; platelet count: 67,000/mm3; fibrinogen: 1.5 g/dL) but haemoglobin level was preserved (10.8 g/dL). A vasopressor support was initiated and hypoxemia persisted despite a 100% FiO2 and a PEEP challenge (PaO2/FIO2: 110). Abdominal ultrasound and bedside chest X-ray were unremarkable. Transesophageal echocardiography (TEE) ruled out a traumatic disruption of the aorta and depicted a normal heart. A contrast study excluded a patent foramen ovale but disclosed a massive anatomical shunt of the pulmonary vasculature (Figure
Pulmonary arteriovenous fistulae associated with the Rendu-Osler-Weber disease. The contrast study performed during a transesophageal echocardiographic examination depicted a massive opacification of the left atrium a few beats after the opacification of the right atrium through the left and right pulmonary veins (a), but no patent foramen ovale, and a subsequent massive opacification of the aortic arch (b). Pulmonary angiography confirmed the presence of multiple arteriovenous fistulae in the two lungs ((c) and (d), arrows), the largest being located in the left inferior lobe (thick arrow). These lesions were subsequently excluded from the pulmonary circulation by serial percutaneous transcatheter embolizations. LA: left atrium; La: left atrial appendage; PV: pulmonary vein; Ao: aortic arch.
In the present case, the Rendu-Osler-Weber disease was fortuitously identified in a trauma patient secondary to severe complications related to large pulmonary anatomic shunts. These complications led to a sustained hypoxemia under ventilator and have presumably facilitated cerebral fat embolism with secondary neurological compromise. Noticeably, the two complications had an abrupt onset and developed concomitantly once the patient had been stabilized.
Unexplained (absence of radiographic infiltrates), sustained (negative recruitment maneuvers) hypoxemia under ventilator should raise the diagnosis of an anatomical shunt, mostly intracardiac (i.e., patent foramen ovale) or rarely intrapulmonary [
Although fat embolism is challenging to recognize in ventilated trauma patients, our patient met all major clinical criteria [
Only 5 to 15% of patients with the Rendu-Osler-Weber disease exhibit anatomical shunt of the pulmonary vasculature [
This work was performed in Medical-Surgical ICU, Teaching Hospital of Limoges, Limoges, France.
All authors declare having no personal or financial conflict of interests.