Hughes-Stovin Syndrome

September 14, 2005
Hesham Taha, MD
Hesham Taha, MD

,
Gamil Kostandy, MD
Gamil Kostandy, MD

,
David Rogers, MD
David Rogers, MD

,
Barry Kaplan, MD
Barry Kaplan, MD

A 22-year-old man complained of progressive shortness of breath and abdominal distention. Three years before, he had completed chemotherapy for Hodgkin's disease and had since been in remission. Recently, he had been treated for tonsillitis with oral antibiotics.

A 22-year-old man complained of progressive shortness of breath and abdominal distention. Three years before, he had completed chemotherapy for Hodgkin's disease and had since been in remission. Recently, he had been treated for tonsillitis with oral antibiotics.

The physical examination revealed edema of the lower extremities and ascites. A CT scan of the chest demonstrated multiple bilateral pulmonary aneurysms, which were confirmed by a pulmonary angiogram (A). CT scans of the abdomen showed thrombosis of the inferior vena cava (B) and the hepatic vein (C). Hughes-Stovin syndrome was diagnosed.

Pulmonary artery aneurysms are unusual,1 report Drs Hesham Taha and Gamil Kostandy of Brooklyn, NY, and Drs David Rogers and Barry Kaplan of Queens, NY. Most commonly, they are caused by an infection (eg, tuberculosis or syphilis) in association with structural cardiac abnormalities, such as congenital heart disease, and structural vascular abnormalities, such as Marfan syndrome and vasculitis.2

Drs Taha, Kostandy, Rogers, and Kaplan add that in 1959, Hughes and Stovin3 published the results of a study of four young men with a syndrome of pulmonary artery aneurysms and deep venous thrombosis. Since then, few cases of this syndrome have been described in young men. The exact cause of the disorder is not known, but it may be related to degenerative changes of the bronchial arteries or emboli infected with organisms of low-grade virulence that cause mycotic aneurysms. However, postmortem studies of the reported cases were unable to document infectious agents.

This patient was treated with anticoagulation and anti-inflammatory medications. The ascites and edema of the lower extremities improved. A follow-up CT scan of the chest showed spontaneous occlusion of the pulmonary aneurysms.

REFERENCES:1.Deterling RA, Clagett OT. Aneurysms of the pulmonary artery: review of literature and report of a case. Am Heart J. 1947;34:471-499.
2. Bartter T, Irwin R. Aneurysms of the Pulmonary arteries. Chest. 1988;94:1065-1075.
3. Hughes JP, Stovin PGI. Setmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest. 1959;53:19-27.