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Pulmonary Arteriovenous Malformations: Clinical and Radiological Presentation
Pulmonary Arteriovenous Malformations: Clinical and Radiological Presentation
T
wo girls (11 and 13 years old) with arteriovenous pulmonary malformations were treated in our institution; in both cases, the initial symptoms included exertional dyspnea and cyanosis. The signs and symptoms as well as results of laboratory tests and chest radiographs on admission are presented in the Table. The masses found on chest radiographs were further assessed by contrast-enhanced spiral computed tomography. Computed tomography identified both lesions as large pulmonary arteriovenous fistulas (PAVFs) (Figures 1 and 2). A small PAVF in the right upper lobe was also demonstrated in case 1 (Figure 3; available at www.jpeds.com). In both cases, underlying diseases such as hereditary hemorrhagic telangiectasia were excluded. The patients were treated with percutaneous coil embolization. This resulted in normalization of PaO2, RBC, and Hb values and subsequent resolution of clinical symptoms. Pulmonary arteriovenous fistulas are a rare clinical entity. The most common clinical presentation of large PAVFs in
Figure 1. Volume-rendering reconstruction of the contrastenhanced spiral computed tomography showing a large arteriovenous fistula in the left upper lobe (lingula).
Table. Results of the basic evaluation in the two presented patients Patient History
Case 1: 11-year-old girl
Case 2: 13-year-old girl
Exertional dyspnea for 4 years, medical and family history unremarkable
Exertional dyspnea for 2 years, migraine headaches, medical and family history unremarkable Symptoms Central cyanosis, finger Central cyanosis, finger clubbing, clubbing, growth no abnormalities on retardation (body heart and lung weight <3rd examination percentile) no abnormalities on heart and lung examination RBC, 6.02 T/L, Hct, Laboratory tests RBC, 6.26 T/L; Hct, 52.8%, 54.2%, Hb, 18.4 g/dL Hb, 18.8 g/dL, PaO2, 52 mm Hg PaO2, 55 mm Hg Chest radiograph Well-defined round Large, well-defined oval opacity in the midopacity in the midlower zone of the left upper zone of the lung (Figure 3) right lung (Figure 4) ECG, Echocardiography Normal Normal
children includes dyspnea, cyanosis, digital clubbing, and round or oval pulmonary mass on plain chest radiography (Figures 3, 4, 5, and 6; available at www.jpeds.com). Because PAVFs are associated with severe complications such as strokes, ischemic attacks, brain abscess, migraine headaches, seizures, hemothorax, hemoptysis or life-threatening hemorrhages, an early diagnosis and treatment is a key issue. The diagnosis of PAVFs is particularly essential in adolescent girls. This is because pregnancy may exacerbate PAVFs, and complications are more frequently observed in the last half of pregnancy.1,2 n
Katarzyna Grzela, MD, PhD Katarzyna Krenke, MD, PhD Marek Kulus, MD, PhD Department of Pediatric Pulmonology and Allergy
Rafa1 Krenke, MD, PhD Figure 2. Volume-rendering reconstruction of the contrastenhanced spiral computed tomography showing an arteriovenous fistula in the right upper lobe.
Department of Internal Medicine Pulmonology and Allergology Medical University of Warsaw Warsaw, Poland
References available at www.jpeds.com J Pediatr 2011;158:856. 0022-3476/$ - see front matter. Copyright ª 2011 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2010.12.015
856
Vol. 158, No. 5 May 2011
References 1. Gossage JR, Kanj G. Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 1998;158:643-61.
2. Faughnan ME, Thabet A, Mei-Zahav M, Colombo M, Maclusky I, Hyland RH, et al. Pulmonary arteriovenous malformations in children: outcomes of transcatheter embolotherapy. J Pediatr 2004;145:826-31.
Figure 3. Colored volume-rendering reconstruction of contrast-enhanced spiral computed tomography showing a large arteriovenous fistula in the left upper lobe (lingula) and a small arteriovenous fistula in the right upper lobe (two vessels feeding artery and draining vein are clearly visible).
Figure 4. Chest radiography: Well-defined round opacity in the mid-lower zone of the left lung.
Figure 5. Chest radiography: Large, well-defined oval opacity in the mid-upper zone of the right lung.
Figure 6. Colored volume-rendering reconstruction of contrast-enhanced spiral computed tomography showing an arteriovenous fistula in the right upper lobe. 856.e1
T
wo girls (11 and 13 years old) with arteriovenous pulmonary malformations were treated in our institution; in both cases, the initial symptoms included exertional dyspnea and cyanosis. The signs and symptoms as well as results of laboratory tests and chest radiographs on admission are presented in the Table. The masses found on chest radiographs were further assessed by contrast-enhanced spiral computed tomography. Computed tomography identified both lesions as large pulmonary arteriovenous fistulas (PAVFs) (Figures 1 and 2). A small PAVF in the right upper lobe was also demonstrated in case 1 (Figure 3; available at www.jpeds.com). In both cases, underlying diseases such as hereditary hemorrhagic telangiectasia were excluded. The patients were treated with percutaneous coil embolization. This resulted in normalization of PaO2, RBC, and Hb values and subsequent resolution of clinical symptoms. Pulmonary arteriovenous fistulas are a rare clinical entity. The most common clinical presentation of large PAVFs in
Figure 1. Volume-rendering reconstruction of the contrastenhanced spiral computed tomography showing a large arteriovenous fistula in the left upper lobe (lingula).
Table. Results of the basic evaluation in the two presented patients Patient History
Case 1: 11-year-old girl
Case 2: 13-year-old girl
Exertional dyspnea for 4 years, medical and family history unremarkable
Exertional dyspnea for 2 years, migraine headaches, medical and family history unremarkable Symptoms Central cyanosis, finger Central cyanosis, finger clubbing, clubbing, growth no abnormalities on retardation (body heart and lung weight <3rd examination percentile) no abnormalities on heart and lung examination RBC, 6.02 T/L, Hct, Laboratory tests RBC, 6.26 T/L; Hct, 52.8%, 54.2%, Hb, 18.4 g/dL Hb, 18.8 g/dL, PaO2, 52 mm Hg PaO2, 55 mm Hg Chest radiograph Well-defined round Large, well-defined oval opacity in the midopacity in the midlower zone of the left upper zone of the lung (Figure 3) right lung (Figure 4) ECG, Echocardiography Normal Normal
children includes dyspnea, cyanosis, digital clubbing, and round or oval pulmonary mass on plain chest radiography (Figures 3, 4, 5, and 6; available at www.jpeds.com). Because PAVFs are associated with severe complications such as strokes, ischemic attacks, brain abscess, migraine headaches, seizures, hemothorax, hemoptysis or life-threatening hemorrhages, an early diagnosis and treatment is a key issue. The diagnosis of PAVFs is particularly essential in adolescent girls. This is because pregnancy may exacerbate PAVFs, and complications are more frequently observed in the last half of pregnancy.1,2 n
Katarzyna Grzela, MD, PhD Katarzyna Krenke, MD, PhD Marek Kulus, MD, PhD Department of Pediatric Pulmonology and Allergy
Rafa1 Krenke, MD, PhD Figure 2. Volume-rendering reconstruction of the contrastenhanced spiral computed tomography showing an arteriovenous fistula in the right upper lobe.
Department of Internal Medicine Pulmonology and Allergology Medical University of Warsaw Warsaw, Poland
References available at www.jpeds.com J Pediatr 2011;158:856. 0022-3476/$ - see front matter. Copyright ª 2011 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2010.12.015
856
Vol. 158, No. 5 May 2011
References 1. Gossage JR, Kanj G. Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 1998;158:643-61.
2. Faughnan ME, Thabet A, Mei-Zahav M, Colombo M, Maclusky I, Hyland RH, et al. Pulmonary arteriovenous malformations in children: outcomes of transcatheter embolotherapy. J Pediatr 2004;145:826-31.
Figure 3. Colored volume-rendering reconstruction of contrast-enhanced spiral computed tomography showing a large arteriovenous fistula in the left upper lobe (lingula) and a small arteriovenous fistula in the right upper lobe (two vessels feeding artery and draining vein are clearly visible).
Figure 4. Chest radiography: Well-defined round opacity in the mid-lower zone of the left lung.
Figure 5. Chest radiography: Large, well-defined oval opacity in the mid-upper zone of the right lung.
Figure 6. Colored volume-rendering reconstruction of contrast-enhanced spiral computed tomography showing an arteriovenous fistula in the right upper lobe. 856.e1