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A rare case of pulmonary arterio-venous malformation with recurrent anemia: Hereditary hemorrhagic telangiectasia.

Nataraju KT, Mukherjee T, Doddaiah RP, Nanjappa NG, Narasegowda L - Lung India (2015 Jul-Aug)

Bottom Line: Arteriovenous malformation (AVM) is a rare vascular anomaly of the lung, which manifests predominantly as dyspnea (due to right to left shunting) and paradoxical embolism.Hereditary Hemorrhagic Telangiectasia (HHT) being a rare genetic disorder is one of the most common causes of pulmonary arteriovenous malformation (PAVM).Here we report an interesting case of recurrent anemia in an elderly female, who was subsequently found to have multiple cutaneous and mucosal telangiectasias and a large pulmonary AVM.

View Article: PubMed Central - PubMed

Affiliation: Department of General Medicine, Kempegowda Institute of Medical Sciences, Bangalore, Karnataka, India.

ABSTRACT
Arteriovenous malformation (AVM) is a rare vascular anomaly of the lung, which manifests predominantly as dyspnea (due to right to left shunting) and paradoxical embolism. Hereditary Hemorrhagic Telangiectasia (HHT) being a rare genetic disorder is one of the most common causes of pulmonary arteriovenous malformation (PAVM). Here we report an interesting case of recurrent anemia in an elderly female, who was subsequently found to have multiple cutaneous and mucosal telangiectasias and a large pulmonary AVM.

No MeSH data available.


Related in: MedlinePlus

(a) Non-contrast CT chest showing a well-circumscribed serpiginous mass in the right lower lobe of the lung (b) Contrast CT chest showing contrast enhancement of the serpiginous mass (c) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein (d) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein
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Figure 3: (a) Non-contrast CT chest showing a well-circumscribed serpiginous mass in the right lower lobe of the lung (b) Contrast CT chest showing contrast enhancement of the serpiginous mass (c) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein (d) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein

Mentions: Investigations showed a hemoglobin of 5.3 gm/dl, total leucocyte count of 9100/cumm, with 84% neutrophils, and a platelet count of 1.79L/cumm. The packed cell volume (PCV) was 20.1 and the erythrocyte sedimentation rate (ESR) was 30 mm at the end of one hour and the peripheral smear showed microcytic hypochromic anemia. The chest x-ray (CXR) showed a right paracardiac mass [Figure 1]. Blood gas analysis showed hypoxemia (PO2 of 46.1 mmHg). The sputum Acid-Fast Bacilli (AFB) test was negative. Liver function tests were normal and the coagulation profile was also normal. The iron study showed low ferritin levels. In view of occult blood in stool, an upper gastrointestinal (GI) endoscopy was done, which showed multiple telangiectases in the pyriform fossa, esophagus, and gastric mucosa [Figure 2]. The colonoscopy was unremarkable. However, in view of the right paracardiac mass on chest x-ray, a CT thorax was done, which showed a well-defined serpiginous mass in the right lower zone suggestive of AV Malformation. A pulmonary angiogram was done, which confirmed the presence of large AV malformation in the right lung [Figure 3]. An ultrasound of the abdomen showed mild hepatomegaly. However, there was no intra-abdominal vascular malformation. A nasal endoscopy was unremarkable.


A rare case of pulmonary arterio-venous malformation with recurrent anemia: Hereditary hemorrhagic telangiectasia.

Nataraju KT, Mukherjee T, Doddaiah RP, Nanjappa NG, Narasegowda L - Lung India (2015 Jul-Aug)

(a) Non-contrast CT chest showing a well-circumscribed serpiginous mass in the right lower lobe of the lung (b) Contrast CT chest showing contrast enhancement of the serpiginous mass (c) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein (d) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4502207&req=5

Figure 3: (a) Non-contrast CT chest showing a well-circumscribed serpiginous mass in the right lower lobe of the lung (b) Contrast CT chest showing contrast enhancement of the serpiginous mass (c) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein (d) CT pulmonary angiogram showing large AV malformation arising from the right descending pulmonary artery, drained by an enlarged tributary of the pulmonary vein
Mentions: Investigations showed a hemoglobin of 5.3 gm/dl, total leucocyte count of 9100/cumm, with 84% neutrophils, and a platelet count of 1.79L/cumm. The packed cell volume (PCV) was 20.1 and the erythrocyte sedimentation rate (ESR) was 30 mm at the end of one hour and the peripheral smear showed microcytic hypochromic anemia. The chest x-ray (CXR) showed a right paracardiac mass [Figure 1]. Blood gas analysis showed hypoxemia (PO2 of 46.1 mmHg). The sputum Acid-Fast Bacilli (AFB) test was negative. Liver function tests were normal and the coagulation profile was also normal. The iron study showed low ferritin levels. In view of occult blood in stool, an upper gastrointestinal (GI) endoscopy was done, which showed multiple telangiectases in the pyriform fossa, esophagus, and gastric mucosa [Figure 2]. The colonoscopy was unremarkable. However, in view of the right paracardiac mass on chest x-ray, a CT thorax was done, which showed a well-defined serpiginous mass in the right lower zone suggestive of AV Malformation. A pulmonary angiogram was done, which confirmed the presence of large AV malformation in the right lung [Figure 3]. An ultrasound of the abdomen showed mild hepatomegaly. However, there was no intra-abdominal vascular malformation. A nasal endoscopy was unremarkable.

Bottom Line: Arteriovenous malformation (AVM) is a rare vascular anomaly of the lung, which manifests predominantly as dyspnea (due to right to left shunting) and paradoxical embolism.Hereditary Hemorrhagic Telangiectasia (HHT) being a rare genetic disorder is one of the most common causes of pulmonary arteriovenous malformation (PAVM).Here we report an interesting case of recurrent anemia in an elderly female, who was subsequently found to have multiple cutaneous and mucosal telangiectasias and a large pulmonary AVM.

View Article: PubMed Central - PubMed

Affiliation: Department of General Medicine, Kempegowda Institute of Medical Sciences, Bangalore, Karnataka, India.

ABSTRACT
Arteriovenous malformation (AVM) is a rare vascular anomaly of the lung, which manifests predominantly as dyspnea (due to right to left shunting) and paradoxical embolism. Hereditary Hemorrhagic Telangiectasia (HHT) being a rare genetic disorder is one of the most common causes of pulmonary arteriovenous malformation (PAVM). Here we report an interesting case of recurrent anemia in an elderly female, who was subsequently found to have multiple cutaneous and mucosal telangiectasias and a large pulmonary AVM.

No MeSH data available.


Related in: MedlinePlus