Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

26 November 2007

Haemorrhagic pulmonary arteriovenous malformations (PAVMs) in a pregnant woman with Rendu-Osler-Weber syndrome treated successfully with embolization – case report

Beata Gryglicka, Irena Węgrzyn-Szkutnik, Janusz Milanowski, Elzbieta Czekajska-Chehab, Andrzej Drop, Tomasz Jargiełło, Malgorzata Szczerbo-Trojanowska

Case Rep Clin Pract Rev 2007; 8:300-305 :: ID: 559625


Background: The Rendu-Osler-Weber syndrome, also known as hereditary haemorrhagic telangiectasia (HHT) is a rare (1 in 10 000) hereditary autosomal dominant disorder caused by the mutation in one of two different genes on chromosome 9 or 12. The syndrome is characterized by dermal and mucosal telangiectases and by arteriovenous fi stulas that are mainly present in the lungs and the central nervous system (CNS), and less commonly in the liver. The major cause of morbidity and mortality due to this disorder is the presence of multiorgan arteriovenous malformations and the associated haemorrhages that may accompany them. In the Rendu-Osler-Weber disease, the bleedings are caused by degenerative changes in the capillaries lacking the elastic muscular-fi brous layer and hyaline lesions.
Case Report: A 19-year-old 5-month pregnant patient was admitted to the Department of Pneumonology because of a pain in the right chest that caused diffi culties in breathing. The physical examination and chest X-ray revealed the presence of fl uid in the right pleural cavity; 500 ml of bloody fl uid was released. The history revealed the Rendu-Osler-Weber syndrome and the arteriovenous fi stula in the right lung diagnosed at the Children Health Centre in 1997. After the consultation with gynaecologists, surgeons and radiologists, multislice computed tomography of the lungs was performed. The examination showed a large arteriovenous fistula with bleeding to the pleural cavity. The patient was decided to be treated by pulmonary embolization. The selective embolization of the artery feeding the vascular malformation resulted in the complete, effective closure of the fi stula.
Conclusions: The presence of one disease (the Rendu-Osler-Weber syndrome and PVAMs) obliges to search for another disorder. The follow-up examinations should be performed in patients with the Rendu-Osler-Weber syndrome every 5 years or more often (e.g. in pregnancy or before planned pregnancy). Due to frequent complications in Rendu-Osler-Weber syndrome patients in pregnancy, CT and angiography should be performed, and embolization should be considered, if necessary. At present embolization is regarded the gold standard of PVAM treatment.

Keywords: Rendu-Osler-Weber Syndrome, PAVM, embolization



19 May 2022 : Case report  Serbia

Bilateral Emphysematous Pyelonephritis Associated with COVID Pneumonia: A Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.936370  

17 May 2022 : Case report  USA

High-Frequency Oscillatory Ventilation for Refractory Hypoxemia in Severe COVID-19 Pneumonia: A Small Case ...

Am J Case Rep In Press; DOI: 10.12659/AJCR.936651  

13 May 2022 : Case report  Japan

A case of exacerbation of haloperidol-induced rhabdomyolysis following the onset of COVID-19

Am J Case Rep In Press; DOI: 10.12659/AJCR.936589  

13 May 2022 : Case report  USA

Acute Disseminated Encephalomyelitis After SARS-CoV-2 Vaccination

Am J Case Rep In Press; DOI: 10.12659/AJCR.936574  

In Press

26 May 2022 : Case report  USA

Cardiac Tamponade as Initial Presentation of Systemic Lupus Erythematous in Third-Trimester Pregnancy

Am J Case Rep In Press; DOI: 10.12659/AJCR.936273  

25 May 2022 : Case report  Mexico

Acute Mitral Regurgitation Due to Chordae Tendineae Rupture: A Rare Presentation of Cardiac Amyloidosis

Am J Case Rep In Press; DOI: 10.12659/AJCR.936545  

25 May 2022 : Case report  USA

Pleural Effusion as the Initial Presentation of Gastric Adenocarcinoma: A Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.935434  

24 May 2022 : Case report  USA

Adalimumab Mitigates Lumbar Radiculopathy in a Case of Ankylosing Spondylitis

Am J Case Rep In Press; DOI: 10.12659/AJCR.936600  

Most Viewed Current Articles

23 Feb 2022 : Case report  USA

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

17 Feb 2022 : Case report  Oman

Myocarditis, Pulmonary Hemorrhage, and Extensive Myositis with Rhabdomyolysis 12 Days After First Dose of P...

DOI :10.12659/AJCR.934399

Am J Case Rep 2022; 23:e934399

06 Dec 2021 : Case report  Brazil

Lipedema Can Be Treated Non-Surgically: A Report of 5 Cases

DOI :10.12659/AJCR.934406

Am J Case Rep 2021; 22:e934406

19 Feb 2022 : Case report  USA

A Rare Case of Coronavirus Disease 2019 Vaccine-Associated Cerebral Venous Sinus Thrombosis Treated with Me...

DOI :10.12659/AJCR.935355

Am J Case Rep 2022; 23:e935355

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923