Middle Lobe Artery. Solitary Aneurysm of the. A Case Report and Review of Solitary Peripheral. Pulmonary Artery Aneurysms

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1 Solitary Aneurysm of the Middle Lobe Artery A Case Report and Review of Solitary Peripheral Pulmonary Artery Aneurysms Jack Monchik, M.D., and Earle W. Wilkins, Jr., M.D. ABSTRACT A patient with a solitary peripheral pulmonary artery aneurysm successfully treated by operation is presented. This is the oldest patient in whom an aneurysm of this type has been removed by lobectomy, and the aneurysm is the first recorded in the right middle lobe. The literature on this entity is reviewed, and the diagnosis and etiology are discussed. Of 35 patients having a solitary peripheral pulmonary artery aneurysm, 21 died from rupture, an outcome which strongly supports the role of operation for this problem. The solitary peripheral pulmonary artery aneurysm is yet another entity to be considered in the differential diagnosis of the so-called coin lesion. Pulmonary angiography may be necessary to exclude multiple aneurysms. A neurysm of the pulmonary artery is a rare entity. Among 37,757 consecutive postmortem examinations reported in a review in 1939, not a single case was encountered [Z]. A review of the literature up to 1946 yielded 146 patients with aneurysm of the trunk and main right and left pulmonary arteries [5]. Aneurysms of the segmental pulmonary artery branches are even less common. (In this report the terms segmental or peripheral pulmonary artery refer to intrapulmonary arteries, excluding the main right and left pulmonary artery.) In 1961 Charlton and DuPlessis [4] reviewed the literature on multiple aneurysms of segmental branches of the pulmonary artery and found 30 cases. They excluded patients with Rasmussen s aneurysm related to tuberculosis cavities and mycotic aneurysms resulting from direct extension of a septic pulmonary focus. There have been several excellent reviews of aneurysm of the trunk and main pulmonary arteries [2, 51 and of multiple pulmonary artery aneurysms [4, 81, but there has been no published review of solitary peripheral pulmonary artery aneurysms. The purpose of this paper is to report a patient with a solitary peripheral pulmonary artery aneurysm, to discuss the diagnosis and etiological basis for these lesions, and to consider the implications for operative treatment. From the Department of Surgery, Harvard Medical School, and the General Surgical Services, Massachusetts General Hospital, Boston, Mass. Accepted for publication Oct. 22, Address reprint requests to Dr. Wilkins, Massachusetts General Hospital, Boston, Mass THE ANNALS OF THORACIC SURGERY

2 CASE REPORT: Solitary Perilbheral PA Aneurysm A FIG. 1. Posteroanterior (A) and lateral (B) roentgenograms of the chest showing a circumscribed solitaty nodule below the right hilus. A 6 1-year-old man entered the hospital because of a solitary pulmonary nodule noted on a routine chest roentgenogram (Fig. 1) at his place of work. He had smoked two packages of cigarettes per day until eight years before, when he had stopped completely. Night sweats and weight loss were denied. For the six months prior to admission he had noted intermittent bloody streaking of his sputum. For several years previous, he had had a cough productive of about one teaspoonful per day of yellow sputum. There was no dyspnea on exertion and no history of venereal disease. He had had a cholecystectomy and hemorrhoidectomy but no other serious illness or injury. On physical examination the blood pressure was 140/85 mm. Hg; pulse, 74 and regular; temperature, 98.6" F. There was no lymphadenopathy. The chest was clear to auscultation and percussion. The cardiac rhythm was regular, and no murmurs were present. Findings from the remainder of the physical examination were within normal limits. The hematocrit was 47%; white blood count, 6,750 with a normal differential: fasting blood sugar, 86; blood urea nitrogen, 11; calcium, 9.2 mg. per 100 ml.; phosphorus, 3.5 mg. per 100 ml.; and alkaline phosphatase, 2.8 Bodansky units. The serum Hinton test was normal. A chest roentgenogram revealed a 3 cm., well-defined, smooth, noncalcified nodule at the root of the right middle lobe. No pulsation was noted by fluoroscopy. The intravenous pyelogram was normal. The electrocardiogram was normal except for a slightly prolonged P-R interval of 0.21 second. B

3 MONCHIK AND WILKINS The patient underwent a right thoracotomy in December, The lesion could not be located by palpation of the lung and mediastinum. Dissection in the major fissure demonstrated a 3 cm. pulsatile mass which proved to be an aneurysm of the right middle lobe artery. The aneurysm was resected by a right middle lobectomy (Fig. 2). The patient had an uncomplicated postoperative course and was discharged on the eleventh postoperative day. Microscopical sections of the aneurysm demonstrated destruction of the elastic tissue fibers in the media. No bacterial organisms were noted in the microscopical section. The middle lobe parenchyma was unremarkable. Review of the Literature A careful review of the literature reveals only 6 persons who have previously undergone successful operative removal of an aneurysm in a peripheral branch of the pulmonary artery (Table 1). Hohlweg [7] in 1948 presented the case of a 20-year-old man who had sustained a shell fragment injury to the chest fifteen days previously and who underwent left lower lobectomy for an aneurysm in a distal segmental artery. In 1953 MacKenzie and Clagett [ 1 11 successfully performed left upper lobectomy for an aneurysm of one of the arterial branches to the left upper lobe. The patient was a 28-year-old man with systolic pulmonary hypertension and a systolic murmur over the aneurysm. In 1955 Konhaus and Kunkel [lo] reported successful lobectomy in a 57-year-old asymptomatic woman for an aneurysm of a branch of the right pulmonary artery to the lower lobe. Her lesion had been found on a routine chest roentgenogram. Sancetta, Driscol, and Hackel FIG. 2. Right middle lobe with aneurysm dissected out; note how it branches into the parenchyma. 498 THE ANNALS OF THORACIC SURGERY

4 CASE REPORT: Solitary Peripheral PA Aneurysm TABLE 1. SUCCESSFUL RESECTIONS OF SOLITARY PERIPHERAL PULMONARY ARTERY ANEURYSMS Patient Author & Year Age&Sex Lobe Etiology of Aneurysm Hohlweg, , M LLL Shell fragment Mackenzie & Clagett, , M LUL Pulmonary hypertension Konhaus & Kunkel, , F RLL Unknown Sancetta et al., , F LLL Pulmonary hypertension Symbas & Scott, , M LLL Bullet wound Frater et al., , M RLL Mycotic infection Monchik & Wilkins, , M RML Unknown LLL = left lower lobe; LUL = left upper lobe; RLL = right lower lobe; RML = right middle lobe. [13] in 1958 reported a right lower lobectomy for an aneurysm of a branch of the pulmonary artery in an 11-year-old girl who had systolic pulmonary hypertension and a recent febrile illness with positive blood culture. Symbas and Scott [14] in 1963 described an aneurysm of a branch of the pulmonary artery to the left lower lobe in a 58-year-old man who had sustained a.32 caliber bullet wound in the left chest thirty-five years previously. He underwent successful resection by left lower lobectomy. Finally, Frater, Beck, and Schrire [6] in 1965 reported a 24-year-old man with deep vein thrombosis, recent febrile illness, and hemoptysis who had an aneurysm of one of the pulmonary artery branches to the right lower lobe. The aneurysm was SUCcessfully resected by right lower lobectomy. The factors related to the development of solitary peripheral pulmonary artery aneurysms are syphilis, tuberculosis, trauma, other mycotic infection, pulmonary hypertension, and congenital malformation. The literature on syphilitic disease of the pulmonary artery through 1909 was reviewed by Posselt [12]. Pulmonary artery lesions were often assumed to have a syphilitic basis because of a past history of syphilis or because luetic lesions were found in other organs. Actual gummas have been observed in several pulmonary artery aneurysms, but these were present only in the main pulmonary artery. Indeed, luetic pulmonary artery aneurysms were thought to occur only in the main pulmonary artery until Warthin [15] in 1917 demonstrated that syphilis could cause a solitary peripheral pulmonary artery aneurysm. He described a 37-year-old man with an aneurysm of a branch of the pulmonary artery to the left upper lobe. The patient had a history of penile chancre and skin rash, and death followed several episodes of massive hemoptysis. Postmortem examination showed spirochetes in the aneurysm wall when the wall was stained with Levaditi preparations. Tuberculosis has also been cited as an etiological factor in solitary segmental pulmonary artery aneurysms. An aneurysm in association with a chronic tuberculosis cavity has been known as a Rasmussen aneurysm. This type of aneurysm was fairly common before the decline in incidence of pulmonary tuberculosis. Auerbach [l] in 1939 found Rasmussen aneurysms in

5 MONCHIK AND WILKINS 4 to 5% of postmortem examinations of patients who died with chronic pulmonary tuberculosis. Kidd [9] in 1884 reported 26 cases of pulmonary artery aneurysm in 230 patients dying of chronic pulmonary tuberculosis. In 17 of these 26 patients, fatal hemoptysis resulted from aneurysm rupture. The peripheral aneurysm was single in 22 of the 26 patients, and most of the aneurysms occurred in small or medium cavities. Trauma is one of the rarer causes of solitary peripheral pulmonary artery aneurysm. Symbas and Scott [14] in 1963 were able to find only 4 previous cases in addition to their own. Three of these 5 resulted from gunshot wounds of the chest. Of the other 2, 1 was caused by a war injury of undescribed type and the other perhaps resulted from blunt trauma. In 2 of the 3 patients with gunshot wounds, a peripheral pulmonary artery aneurysm developed within twenty days of injury. Hemoptysis was present in both of these patients, and 1 died of intrabronchial hemorrhage. The third patient with a gunshot wound had no symptoms of aneurysm for twenty-six years. This patient had also been treated for syphilis in the interim. Two of the 3 patients with aneurysms secondary to gunshot wounds had resection of their aneurysm by lobectomy. Although mycotic pulmonary artery aneurysms are usually multiple, they may present as solitary peripheral pulmonary artery aneurysms. In 1967 Kauffman, Lynfield, and Hennigar [8] reported 22 cases of mycotic pulmonary aneurysm, 4 of which were single and peripheral. Aneurysms caused by tuberculosis, syphilis, and trauma were excluded from this study, and the 22 patients were divided into three groups: (1) those with congenital heart disease, (2) those with recurrent infections or thrombophlebitis (Hughes-Stovin syndrome), and (3) those without congenital heart disease or known thromboembolism. Of 9 patients with pulmonary artery aneurysm associated with congenital heart disease, only 1 had a solitary peripheral aneurysm. At postmortem examination all these patients had bacterial endocarditis, and formation of the aneurysm may have been secondary to embolization from the endocarditis. The patient with the solitary peripheral pulmonary artery aneurysm was a 22-year-old woman whose aneurysm was located in a branch of the pulmonary artery to the left lower lobe. She died of a wasting illness with severe dyspnea of twelve months duration. At postmortem examination a patent ductus arteriosus with endocarditis on the ductus, on the aortic valve, and in the proximal pulmonary artery was found. The heart was enlarged, and although she had evidence of active pulmonary tuberculosis, there was no cavitary disease. There was no evidence at postmortem examination of rupture or leakage from the aneurysm. Eight patients with peripheral pulmonary artery aneurysm associated with recurrent thromboembolism have been reported. Two of these patients had solitary peripheral pulmonary artery aneurysms. All 8 patients were young males between 10 and 35 years old. The earliest symptoms were 500 THE ANNALS OF THORACIC SURGERY

6 CASE REPORT: Solitary Peripheral PA Aneurysm those of peripheral thrombophlebitis. A febrile illness occurred in almost all these patients. Cough, dyspnea, and hemoptysis were common. Blood cultures were negative in 6. The disease was prolonged and terminated in rupture of the aneurysm in 1 of the 2 patients with a solitary lesion. It is interesting to note that all 6 patients with multiple mycotic aneurysms in this group died of rupture of a pulmonary aneurysm. The final group in the series of mycotic aneurysms reported by Kauffman, Lynfield, and Hennigar [8] consisted of 5 patients without congenital heart disease or thromboembolism. One of these patients had a solitary peripheral aneurysm. Because this patient had an unusual form of pulmonary hypertension and because histologically the aneurysm did not appear mycotic, this patient is discussed under the etiological category of pulmonary hypertension. Pulmonary hypertension has an interesting relationship to solitary segmental pulmonary artery aneurysms. Two instances of solitary peripheral pulmonary artery aneurysm have been associated with an unusual type of pulmonary hypertension. Sancetta, Driscol, and Hackel [ 131 described an ll-year-old girl who presented with an acute febrile illness thought to be encephalitis. Staphylococcus aureus was grown from several blood cultures. A right middle and lower lobe infiltrate was noted on chest roentgenogram. With gradual clearing of the infiltrate, a right perihilar density became apparent. Cardiac catheterization revealed a pulmonary artery (PA) pressure of 90/ 15 mm. Hg and no evidence of intracardiac shunt. A right lower lobectomy was performed for a peripheral pulmonary artery aneurysm which histologically was not mycotic. Repeat cardiac catheterization three years later showed no evidence of progression of her systolic pulmonary hypertension, and she has remained asymptomatic. MacKenzie and Clagett [ 1 13 described a similar finding in a 28-year-old dentist. At age 5 he had been found to have a systolic murmur that was heard both over the left side of the chest and in the interscapular area. There was no cyanosis. He had no symptoms. Chest fluoroscopy showed a pulsatile mass in the left upper lobe. Arterial oxygen studies at rest and during exercise were normal, excluding an arteriovenous fistula unless the shunt were very small. Cardiac catheterization showed no intracardiac shunt. The PA pressure was 60/10 mm. Hg. He underwent left upper lobectomy, and the solitary aneurysm was successfully resected. Postoperatively the murmur remained essentially unchanged. These 2 patients differ in three respects from those with idiopathic primary pulmonary hypertension: (1) the diastolic pressure was normal; (2) there was no evidence of progression of pulmonary hypertension; and (3) the small muscular arteries and arterioles were essentially normal histologically. It is interesting that both of these unusual cases of pulmonary hypertension were associated with a solitary peripheral pulmonary artery aneurysm. In a careful review of the literature, we could find no patients in

7 MONCHIK AND WILKINS TABLE 2. INCIDENCE OF RUPTURE RESULTING IN DEATH IN SOLITARY PERIPHERAL PULMONARY ARTERY ANEURYSMS No. Cause of Aneurysm No. of Patients No. Dead from Rupture Undergoing Resection Syphilis Tuberculosis Trauma Mycotic infection Systolic pulmonary hypertension Congenital lesion (?idiopathic) Total whom segmental pulmonary artery aneurysm was associated with the more usual form of idiopathic pulmonary hypertension. Those solitary peripheral pulmonary artery aneurysms for which no other etiological cause is apparent have been classified as congenital. The patient presented in this paper and the one described by Konhaus and Kunkel [lo] may well have had congenital aneurysms. In the absence of previous chest films, this consideration is at best speculative. Perhaps it would be more appropriate to call the aneurysms idiopathic. Comment Table 2 divides the solitary peripheral pulmonary artery aneurysms by etiology in showing the incidence of rupture. The data, illustrating the high incidence of rupture in association with infection and trauma, strongly support operative removal of the aneurysms in these patients. The diagnosis of peripheral pulmonary artery aneurysm should be considered in a patient with a chest roentgenogram showing a solitary pulmonary nodule, particularly if the patient s symptoms fit into one of the above etiological categories. Calcification was not seen in the patients reviewed. Chest fluoroscopy should be done to determine if pulsation is present. If an aneurysm is suspected, a thorough search must be made for other pulmonary artery aneury:.*,.. because of the high incidence of multiple aneurysms. A pulmonary angiogram provides the definitive evidence. By injecting contrast material into the right atrium or pulmonary artery, Calenoff [3] was able to localize multiple mycotic peripheral artery aneurysms in 2 patients. The aneurysms opacified with filling of the pulmonary artery and remained filled through the venous phase. The slow emptying of contrast material from the aneurysms during the venous phase is probably secondary to the deficiency of elastic tissue in the aneurysm. Thus the pulmonary angiogram provides both a definitive diagnosis and the demonstration of multiple aneurysms. 502 THE ANNALS OF THORACIC SURGERY

8 References CASE REPORT: Solitary Peripheral PA Aneurysm Auerbach, 0. Pathology and pathogenesis of pulmonary arterial aneurysm in tuberculous cavities. Am. Rev. Tuberc. 39:99, Boyd, L. J., and McGauack, T. H. Aneurysm of the pulmonary artery: A review of the literature and report of two cases. Am. Heart J. 18:562, Calenoff, L. Multiple mycotic pulmonary artery aneurysms. Am. J. Roentgenol. Radium Ther. Nucl. &fed. 91:379, Charlton, R. W., and DuPlessis, L. A. Multiple pulmonary artery aneurysms. Thorax 16:364, Deterling, R. A., and Clagett, 0. T. Aneurysm of the pulmonary artery: Review of the literature and report of a case. Am. Heart J. 34:471, Frater, R. W., Beck, W., and Schrire, V. Syndrome of pulmonary artery thrombi and peripheral venous thrombi. J. Thorac. Cardiovasc. Surg. 49:330, Hohlweg, E. Operativ geheiltes traumatisches Aneurysma einer Lungarterie. Chirurg 19:373, Kauffman, S. L., Lynfield, J., and Hennigar, G. R. Mycotic aneurysms of the intrapulmonary arteries. Circulation 35:90, Kidd, P. Unusual cases of pulmonary aneurysms. Trans. Pathol. SOC. London 35:98, Konhaus, C. H., and Kunkel, P. A., Jr. Aneurysm of a pulmonary artery: Report of a case in which treatment was surgical. Ann. Surg. 142:997, MacKenzie, D. A., and Clagett, 0. T. Unusual aneurysm of a pulmonary artery: Report of a case in which treatment was surgical. J. Thorac. Surg. 25:524, Posselt, A. Die Erkrankungen des Lungenschlagader. Ergeb. Allg. Pathol. 13:298, Sancetta, S. M., Driscol, T., and Hackel, D. B. Primary pulmonary systolic hypertension associated with aneurysm of the pulmonary artery. Am. Heart J. 55:607, Symbas, P. N., and Scott, H. W., Jr. Traumatic aneurysm of the pulmonary artery. J. Thorac. Cardiovasc. Surg. 45:645, Warthin, A. S. Syphilis of the pulmonary artery. Am. J. Syph. 1:693, VOL. 17, NO. 5, MAY, )