A 48-year-old Nigerian patient was in transit route to California via Bombay. He presented with haemoptysis of significant amount. He gave past history of recurrent haemoptysis for past 2 years. X-Ray chest showed mass in the left hilum. CT scan chest showed fusiform left pulmonary artery aneurysm in the middle portion (Fig. 1). Branches to lingular lobe and lower lobe were arising from the aneurysm. Pre-operative bronchoscopy revealed extrinsic compression of lingular and lower lobe bronchus with mucosal congestion and active oozing. 2-D Echocardiography ruled out valvular disease and left to right shunt. Serological tests for syphilis and HIV were negative. Although his arm span was significantly larger than his height, other features of Marfan’s syndrome were absent. There was no evidence of Systemic lupus erythematosus (SLE). CT Angio was reviewed by vascular surgeon for feasibility of aneurysmoplasty. With high risk consent left lower lobectomy and segmental resection of lingular lobe was done with excision of the aneurysm (Figs. 2,3). Postoperative course was uneventful. Histopathological study of aneurysmal wall revealed diminished and disarray of elastic tissue content. There was no vasculitis and pulmonary parenchyma was normal.

Pulmonary artery aneurysm (PAA) is a rare condition and its pathogenesis and clinical significance are not clearly understood. PAA secondary to pulmonary hypertension due to left to right shunt and congenital heart diseases has been reported.1,2 Giant PAA of the main trunk causes severe pulmonary regurgitation. None of the patients had intimal tearing, medial dissection or pulmonary artery rupture. Repair of the pulmonary valve suffices in such cases while repair of the aneurysm is not necessary. The timing of surgical intervention should be determined by change in the right ventricular size and function resulting into pulmonary regurgitation. Chen YF has reported the first successful aneurysmectomy and primary anastomosis of the defect of the main pulmonary artery.1 Aoyagi et al has reported PAA associated with atrial septal defect in mother and daughter.3 PAA has also been associated with collagen disorders like Marfan’s syndrome, Ehlers-Danlos syndrome and SLE.4 Vasculitis is the primary pathological feature of Behcet’s disease and in which PAA formation is rare but serious complication representing poor prognosis. Steroid therapy supplemented with Azathioprine may be a good choice for treatment of PAA in Behcet’s disease.5 Multiple aneurysms of pulmonary artery following recurrent septic pulmonary embolisms is also well known pathology.6 The relationship between size and location of the aneurysm, rate of diameter enlargement, pulmonary pressure and subsequent dissection or rupture remains undefined.7,8 Surgical management would be required in patients with dyspnoea on exertion, chest pain, haemoptysis or huge size of the aneurysm because conservative treatment will undoubtedly result in rupture with fatal outcome.7-9 Giant aneurysms are inherently unstable because any dilatation in turn increases the dilating force. This sequence is in accord with the law of Laplace, which states that the wall tension is directly proportional to intravascular pressure and radius of the vessel and is inversely related to the wall thickness. So that the thinner the vessel wall becomes the wall tension increases more and the vessel dilates more or even ruptures.10

Fig. 1 : CT scan showing pulmonary artery aneurysm in middle portion of left pulmonary artery.

In our case after diagnosing impending rupture of the middle portion of the pulmonary artery by CT scan and bronchoscopy, feasibility of local repair was ruled out because there were multiple branches to lingular and lower lobe arising from the aneurysm. With no option remaining resectional surgery was most appropriate.

Fig. 2 : Operative finding - Fusiform aneurysm of left pulmonary artery.	Fig. 3 : Operative specimen - Showing aneurysm eroding into the left lower lobe bronchus.

Book Review

Dermatology, Venereology and Leprology

By Dr. DM Thappa; Publisher : Elseveir; Second Edition.

This book is intended for undergraduates, post-graduates and general practitioners.

It has 405 pages and divided into well-laid out 40 chapters. They are well planned out and include basics of each diseases chapter 3 on phenomena, signs and tests is well thought off for undergraduate exams. schematic diagrams are clear and give out lot of information.

Clinical photographs at times are not very clear. One important aspect of medicine ie differential diagnosis of skin lesions which is not included under individual diseases. If this book is intended for general practitioners it also needs to discuss therapy in more detail. This book will be very useful for undergraduates preparing for the final MBBS examination. I am sure the author will keep the suggestion in mind and include them in next edition.

Dr. Deepak A Parikh
Skin specialist, Bombay Hospital