King Edward Memorial Hospital - Departments: Radiology - Case Of The Month May 2004

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Radiology

Case of the Month

Case No. :	65
Month :	May
Year :	2004
Contributor :	Dr. Amol Khandelwal

Other Cases

Radiological Findings

Discussion

CLINICAL PROFILE :

A 22-year-old man presented with breathlessness , fever and streaky hemoptysis since three days .There was no history of chest
pain. He had been diagnosed in 2002 as a case of Behcet's syndrome with pulmonary tuberculosis with pulmonary thromboembolism (chronic). Chronic pulmonary thromboembolism had been diagnosed on CT angiography in November 2002.
He was being treated with anti tuberculous drugs and was on Warfarin. He had stopped taking warfarin since last 1 year. On examination, his pulse rate was 122/min, BP- 140/90mmhg, Pallor ++, Periphral cynosis ++, Wheeze and creptitions were heard on auscultation. The INR was 1.89.

Plain chest radiographs, CT angiography and pulmonary angiograms were performed. These revealed an aneurysm of the right pulmonary artery as the cause of hemoptysis. Embolisation of the aneurysm was done using steel coils. However, the following day, the patient developed a cardiac arrest from which he could not be revived.

RADIOLOGICAL FINDINGS:

Chest Radiograph

Fig. 1

Fig. 1

There is bilateral hilar prominence and right paracardiac opacity with air bronchogram.

CT Angiohgram


Fig. 2	Fig. 3

Dilatation of the root of the main pulmonary artery.2.5 x 3 cm with an aneurysm of the descending branch of the right pulmonary
artery is seen. There is consolidation of the right middle lobe and apical segment of the lower lobe. Right-sided pleural effusion is apparent.

Pulmonary angiogram


Fig. 4	Fig. 5

Fig. 6

There is an aneurysm of the descending branch of the right pulmonary artery and thrombosis of the ascending branch of the right pulmonary artery. Endovascular Embolisation of the aneurysm was done using multiple coils (Cook) of sizes. Post embolisation angiogram revealed satisfactory occlusion of the aneurysm.

DISCUSSION:

Aneurysms of pulmonary artery (PAA) are uncommon but have a potentially fatal prognosis due to the risk of rupture and frequent presence of underlying pulmonary hypertension. Although the etiology and pathogenesis are not always clear, PAA are most commonly associated with congenital and acquired cardiovascular abnormalities accompanied by pulmonary hypertension. Most structural vascular abnormalities are degenerative and acquired- although isolated congenital vascular abnormalities are also associated with PAA. Infection is a common cause of acquired PAA. Both syphilis and tuberculosis, which were major causes of PAA in the past, are now quite rare.

Behcet's disease and Hugh-Stovin syndrome are forms of idiopathic vasculitis, which may be associated with PAA. Vasculitis due to Behcet's disease manifests as venous occlusions and varix formation, arterial occlusion and arterial aneurysm formation. Pulmonary involvement in Behcet's disease occurs in 5% of patients. Arterial involvement occurs in the late stages usually in young males and the presence of PAA indicates poor prognosis. Hugh-Stovin syndrome represents the combination of deep vein thrombosis and CNS involvement due to cerebral venous thrombosis and considered by many to be a variant of Behcet's disease. Blunt and penetrating trauma (e.g. injury from Swan-Ganz catheter) is a rare cause of PAA.

Clinical symptoms of PAA are nonspecific and reflect the underlying cause. Hemoptysis, abnormal chest radiograph, right to left shunt and CNS phenomena attributable to emboli without an obvious source are the four presentations that might raise suspicion for PAA. Depending on its location, PAA may present as a hilar or peripheral mass. Computed tomography is often the next diagnostic imaging procedure and helps not only in the detection of the vascular nature of the lesion, but also demonstrates its multiplicity, as also detecting any feeding vessels and secondary complications such as thrombosis.

Angiography still remains the gold standard of diagnosing PAA and is required especially in cases where embolotherapy is planned. Occlusion by detachable balloons or steel coils is the preferred mode of treatment of PAA.

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