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Joseph Junewick, MD FACR
over 7 years ago
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Osler-Weber-Rendu

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Vascular
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
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History

10 year old male


Case Images


Diagnosis

Osler-Weber-Rendu

Findings

CT – Coronal reformatted postcontrast images of the chest show multiple large (measuring 5.1 × 4.2 × 4.0 cm in the right lower lobe) pulmonary arteriovenous malformations. Note the enlargement of the involved pulmonary arteries and pulmonary veins.

MR – Postgadolinium axial and coronal T1 images demonstrate left frontal and parietal vascular malformations likely involving the leptomeninges and cortex.

Discussion

Osler-Weber-Rendu (hereditary hemorrhagic telangiectasia) is a systemic fibroblastic dysplasia of all vessels related to a mutation in activin receptor-like kinaase or endoglin (growth factors). Typically patients manifest with recurrent epistaxis and gastrointestinal bleeding.

Over 70% of patients with pulmonary arteriovenous malformations have Osler-Weber-Rendu syndrome whereas about 30% of patients with Osler-Weber-Rendu syndrome have pulmonary arteriovenous malformations. Pulmonary arteriovenous malformations manifest with hypoxia, dyspnea and decreased exercize capacity. Since pulmonary arteriovenous malformations lack a capillary bed, there is a high incidence of transient ischemic attacks and stroke. Pulmonary arteriovenous malformations tend to be unilateral and more often in the lower lobes. Pulmonary arteriovenous malformations are more common in females (except in the newborn period) which may be related to estrogen effect.

While the vascular malformations of hereditary hemorrhagic telangiectasia are congenital, usually they are not recognized until adulthood. Intracranial arteriovenous malformations may result in seizure or hemorrhage. Hepatic arteriovenous malformations may result in high output congestive heart failure. Patients with Osler-Weber-Rendu syndrome require antibiotic prophylaxis for surgical and dental procedures.

Reference

Remy J, Remy-Jardin M, Wattinnet L, Deffontaines C. Pulmonary arteriovenous malformations: Evaluation with CT of the chest before and after treatment. Radiology (1992); 182: 809-816.



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