An 11-year-old Croatian boy of Mediterranean origin with a history of asthma since childhood was admitted to our hospital for evaluation of difficult-to-control asthma during the previous six months. He complained of dyspnea on exertion and several asthma exacerbations, one of which required hospitalization the previous month. He had been treated with tapering doses of systemic corticosteroids for each exacerbation without improvement of chronic hypoxemia and symptoms. His current medications included: fluticasone and salmeterol 250μg/50μg twice daily, montelukast five mg once daily and salbutamol on an as-needed basis, which he currently used four times daily.
General patient history was positive on atopic diseases, as an infant he had atopic dermatitis with cow's milk allergy. He had also suffered from common epistaxis since childhood. At the age of six he was diagnosed with asthma because of a history of recurrent wheezing episodes and positive bronchoprovocation test. A skin prick test revealed sensitivity to house dust mite and grass pollen. Since the diagnosis of asthma had been established the patient was regularly followed-up by a pediatric pulmonologist and during the last couple of years his asthma was adequately controlled. He was symptom free, without prophylaxis and without the need for rescue medication. He complained of a poor appetite and frequent headaches two months prior to hospitalization.
At hospital admission, his general condition was slightly disturbed; he was hypoxic at rest (saturation level of oxygen in hemoglobin (SaO2) 92%), without improvement to oxygen supplemental therapy. Furthermore, there were discontinuous systolic murmurs (two out of six) along his left sternum, slightly decreased breath sounds on the right side of his lungs, two telangiectases on his left cheek and discrete telangiectases on his back; skin and visible mucous membranes were pale to cyanotic.
A body plethysmography showed forced vital capacity (FVC) 2.65L (79%), forced expiratory volume in one second (FEV1) 2.61L (97%), FEV1/FVC 95; airways resistance 0.23L (94%), expiratory airway resistance 0.38L (155%), airway conductance 4.28L (106%), residual volume 1.57L (156%), total lung capacity 4.26L (97%); post-bronchodilator studies FEV1 2.74L (increase of 5%); FVC 2.83L (increase of 5%). The diffusing capacity of his lungs for carbon monoxide (DLCO) was 61%. A bronchoscopy revealed compression of the superior lingular bronchus. A chest X-ray showed a homogeneous soft tissue mass 20×30mm at the left upper lobe of the left lung (Figure 1). Transthoracic echocardiography showed normal cardiac structures with no evidence of pulmonary hypertension. A computed tomography (CT) pulmonary angiogram showed a 35-mm arteriovenous malformation (AVM) within the patient's perihilar left upper lobe and a small AVM within the apical segment of the right lobe (Figure 2). The left perihilar AVM had a single feeding artery and a huge aneurism. On the right apical segment were two feeding arteries (Figures 3, 4).
Three-dimensional computed tomography angiography revealed a 35-mm vascular malformation within the perihilar left upper lobe with a single feeding artery originating from the pulmonary artery and a single draining vein.
A. Arteriovenous malformation and aneurism of the left pulmonary artery segmental branch. B. Status post-embolization of the left pulmonary artery segmental branch with arteriovenous malformation occlusion.
A. Arteriovenous malformation of the right pulmonary artery segmental branch in the upper lobe with two feeding arteries. B. Status post one arteriovenous malformation (AVM) feeding artery embolization of the right upper lobe. C. Status post second AVM feeding artery embolization in the right upper lobe.
Genetic analysis identified a mutation in the endoglin gene, typical for patients with hereditary hemorrhagic telangiectasia (HHT), also called Osler–Weber–Rendu syndrome.
Percutaneous transchateter embolization of both AVMs was performed with stainless steel coils (Figure 5). We used a transfemoral approach with selective catheterization of the feeding artery. We did not use an introducer catheter, and we only used a diagnostic catheter on the 0.035 hydrophilic guidewire. After we had achieved a secure position of the diagnostic catheter, we performed the embolization with stainless steel coils (Johnson-Johnson) whose official diameter was equal or 10% larger than the diameter of the feeding arteries. The control angiogram was performed with the same diagnostic catheter (Figures 3, 4). Post-embolotherapy, no residual flow was seen through the AVMs (Figure 6). The percutaneous pulse oxymetry saturation increased from 92% to 97% immediately on room air. His exercise tolerance improved and he was symptom free during the follow-up period of six months after the procedure. A magnetic resonance imaging (MRI) of the brain and cervical spine did not show any cerebral or cervical AVM. Further investigation revealed a positive family history: a cousin of the patient's father has a diagnosed brain AVM.
Chest X-ray with visible steels coils after embolotherapy of pulmonary arteriovenous malformations within the perihilar left upper lobe and small arteriovenous malformation within the apical segment of the right lobe.
J Med Case Reports. 2013;7(32) © 2013 BioMed Central, Ltd.