Radiological Category Thoracic Chest Principal Modality 1 CT

Case History 16 y/o female, currently no pulmonary symptoms, history witheld. (Ignore the fact

Test Your Diagnosis Which one of the following is your choice for the appropriate

Findings and Differentials Findings: Multiple AVM’s as evidenced by nodules with feeding arteries and

Discussion Pulmonary AVM’s are abnormal pulmonary arterial to venous communications w/o intervening capillary bed.

Discussion Clinical manifestations of PAVM’s include asymptomatic dz, exertional dyspnea/cyanosis, hemoptysis, paradoxical embolic phenomena

References Cottin, V, et al. Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Series

Diagnosis Pulmonary arteriovenous malformations in Hereditary Hemorrhagic Telangiectasia, aka Osler-Weber-Rendu disease.

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Radiological Category: Thoracic Chest Principal Modality (1): CT Principal Modality (2): General Radiography Case Report #0381 Submitted by: Andrew Tenn, M. D. Faculty reviewer: Emma Ferguson, M. D. Date accepted: 05 March 2007

Case History 16 y/o female, currently no pulmonary symptoms, history witheld. (Ignore the fact that she has a vp shunt- not relevant to case)

Radiological Presentations

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. • Septic emboli • Pulmonary metastatic disease • Osler-Weber-Rendu • Wegener’s granulomatosis

Findings and Differentials Findings: Multiple AVM’s as evidenced by nodules with feeding arteries and draining veins. RLL avm has been previously coiled. Patient also has a VP shunt for unrelated hydrocephalus. Differentials for multiple bilateral pulmonary nodules (few enough to count) • Mets • Wegener’s granulomatosis • AVM’s • Septic emboli • In AIDS, Kaposi’s Sarcoma, NHL

Discussion Pulmonary AVM’s are abnormal pulmonary arterial to venous communications w/o intervening capillary bed. 80% or more of PAVM’s are associated with Osler-Weber-Rendu or Hereditary Hemorrhagic Telangiectasia, an autosomal dominant disease of telangiectasias of skin, mucosal surfaces, and avm’s in various visceral organs or brain. Can also be idiopathic, or related to hepatopulmonary syndrome. Simple avm has 1 feeding artery and 1 draining vein; complex has 2 or more arterial feeders.

Discussion Clinical manifestations of PAVM’s include asymptomatic dz, exertional dyspnea/cyanosis, hemoptysis, paradoxical embolic phenomena (cerebral abscess or infarcts, periph emboli), high-output CHF. HHT has myriad other manifestations due to numerous avm’s elswhere, such as epistaxis, gi bleeds, Fe-deficient anemia, seizures, etc… Embolization of PAVM’s is indicated if: asymptomatic patient w/ avm’s of larger diameter than 3 mm, due to risk of paradoxical embolism; any avm’s amenable to embo if there are cardiovascular or embolic effects related to shunting Embolization is with coiling, or for larger avm’s detatchable balloons. Particulate embo material is just a bad idea. . .

References Cottin, V, et al. Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Series of 126 Patients. Medicine (Baltimore) Volume 86(1), January 2007, pp 1 -17 Muller NL, Fraser RS, Colman NC, Pare PD. Radiologic Diagnosis of Disease of the Chest. W. B. Saunders Co. , 2001.

Diagnosis Pulmonary arteriovenous malformations in Hereditary Hemorrhagic Telangiectasia, aka Osler-Weber-Rendu disease.