Bacterial Endocarditis in a Patient with Marfan's Syndrome

dyspnea. although nonspecific, is the most common presentation for tracheal tumors.1.2 Tracheal tumors are rare; the Mayo Clinic reported one tracheal tumor to every 75 to 180 pulmonary tumors. 2 A tracheal tumor must be considered as a possibility in any patient with recent onset of wheezing which is unresponsive to bronchodilator therapy. The study of these patients is complicated by the fact that frequently the routine radiographic views of the chest are read as normal. Nevertheless, in one series, 13 of 53 patients had a demonstrable tracheal abnormality on routine chest films; another series reported only nine of 20 examinations to be diagnostic initially. 1 There are two reasons for the early misinterpretation. The trachea is often obscured, and physicians, including radiologists, may be unfamiliar with tracheal tumors and may not carefully examine the area. Further noninvasive studies which can increase the yield of pathologic findings are overpenetrated anteroposterior views and tracheal tomograms. Tomographic studies are especially helpful in defining the extent of both intraluminal and possible soft-tissue spread. In the series of Houston et al, 2 tracheal tomograms showed positive findings in 16 of 18 patients, whereas 13 of those 16 patients had normal routine posteroanterior and lateral films. The definitive diagnosis is established by bronchoscopic examination, which provides both a pathologic specimen and information on the exact location and amount of intraluminal involvement. The latter is important in planning the surgical manipulation of the endotracheal tube. The use of a second tube inserted in the main-stem bronchus obviated the need for extracorporeal circulation. In general, cytologic examinations are of little value, but they are helpful in epidermoid carcinoma.2 Histologically, the most common tracheal tumor is epidermoid carcinoma, followed by adenoid cystic carcinoma. Mucoepidermoid carcinoma of the trachea, reported by Larson et al 3 in 1965, is extremely rare, and there are only a few case reports. 4 •6 These tumors are known to occur in the salivary glands 6 and in the major bronchi,T-10 where they form along with carcinoid adenoid cystic carcinoma and the bronchial "adenoma" tumor group. The mucoepidermoid lesions usually are smooth, rounded tan-colored masses with intact overlying mucosa which are histologically a composite of mucinproducing glandular elements and epidermoid cells with intercellular bridging.a The lesions are believed to arise from the ducts of the tracheobronchial glands, and the most frequent location on the posterolateral aspect of the trachea corresponds to the larger number of mucous glands located there. 2 Based on the experience with bronchial mucoepidermoid carcinoma, there are two natural courses that the tumor can follow; one is gradual expansion over a period of many months with local infiltration and obstruction, and the other is transformation of the epidermoid element into frank high-grade metastasizing epidermoid carcinoma with a rapid downhill course. 6 •7 Adequate treatment seems to rely on complete surgical extirpation;

784 MEHL, KRONZON, ZIMMERMAN

these tumors are radioresistant, and endoscopic diathermy leads to repeated local recurrences and high morbidity.2 The prognosis in these tumors is difficult to assess, due to their rarity. The patient of Larson et al 3 was alive and without recurrence four years after surgery. The extent of local infiltration, which would govern resectability and affect surgical mortality, would be important. In addition, on the basis of the experience with mucoepidermoid carcinoma of the bronchus, those patients having tumors with a dominance of anaplastic epidermoid elements will probably do poorer than those patients having tumors with more benign-appearing mucous and epidermoid cells.8 - 11

REFERENCES 1 Karlan MS, Livingston PA, Baker DC Jr: Diagnosis of tracheal tumors. Ann Otol Rhino! Laryngol 82:790, 1973 2 Houston HE, Payne WS, Harrison EG, et al: Primary cancer of the trachea. Arch Surg 99: 132, 1969 3 Larson RE, Woolner LB, Payne WS : Mucoepidermoid tumor of the trachea. J Thorac Cardiovasc Surg 50:131, 1965 4 Trentini GP, Palmieri B: Mucoepidermoid tumor of the trachea. Chest 62:336-338, 1972 5 Heilbrunn A, Crosby IK: Adenocystic carcinoma. and mucoepidermoid carcinoma of the tracheobronchial tree. Chest 61:145-149, 1972 6 Stewart FW, Foote FW, Becker WF: Mucoepidermoid tumor of the salivary glands. Ann Surg 122:820, 1945 7 Dowling EA, Miller RE, Johnson IM, et al: Mucoepidermoid tuinors of the bronchi. Surgery 52:600, 1962 8 Meckstroth CV, Davidson HB, Kress GO : Muco-epidermoid tumor of the bronchus. Dis Chest 40:652-656, 1961 9 Weiss L, Ingram M: Adenomatoid bronchial tumors. Cancer 14:161, 1961 10 Turnbull AD, Huvos AG, Goodner JT, et al: Mucoepidermoid tumors of bronchial glands. Cancer 28:539, 1971 11 Payne WS, Ellis FH, Woolner LB, et al: The surgical treatment of cylindroma (adenoid cystic carcinoma) and mucoepidermoid tumors of the bronchus. J Thorac Cardiovasc Surg 38:709, 1959

Bacterial Endocarditis in a Patient with Marfan' s Syndrome• Eehoeardiographie Features Sydney]. Mehl, M.D.; Itzhak Kronzon, M.D.; and

David Zimmerman, M.D.

A patient with Madan's syndrome and subacute bacterial endocarditis is presented. Ec:hocardiograpbic studies demonstrated dilatation of the aortic root, prolapse of the posterior leaftet of the mitral valve, and the appearance of shaggy echoes on the anterior mitral leaftet, of the kind previously described as representing bacterial vegetations. °From the Department of Medicine, New York University School of Medicine, New York. Reprint requests: Dr. Kronzon, 560 First Avenue, New York City 10016

CHEST, 70: 6, DECEMBER, 1976

s syndrome, an inherited disorder of connecM arfan' tive tissue, commonly associated with involve-

is ment of the aortic root and mitral apparatus.1 An increased susceptibility to bacterial endocarditis, particularly on the mitral valve, has been noted. 2 • 3 We report a patient with Marfan's syndrome and tl-hemolytic streptococcal endocarditis, in whom echocardiographic examination revealed dilatation of the aortic root, bolosystolic mitral valvular prolapse, and the constant finding of multiple, irregular shaggy echoes on the anterior mitral leaflet, of the kind previously reported in association with valvular bacterial vegetations.• CASE REPoRT

A 26-year-old man was admitted to University HospitBl with a two-week history of fever. A diagnosis of Marfan's syndrome had been made when the patient was ten years old, at which time he was found to have ectopia lentis and a characteristic body habitus. Aheart murmur was first detected when the patient was 17 years old. There was no history of rheumatic fever, and the patient was without cardiac symptoms. He was well witil two weeks prior to admission when he insidiously developed fever as high as 39.5°C ( 103°F) accompanied by shaking chills and malaise.

On physical examination, the patient was tall ( 193 em [ 6 ft 4 in]) and thin but in no acute distress. The blood pressure was 100/60 mm Hg, the pulse was 120 beats per minute, the respiratory rate was 18/min, and the temperature was 38.9•C (102•F). Ectopia lentis, high-grade myopia, and a high arched palate were noted. The jugular and carotid pulsations were normal. There was scoliosis of the thoracic spine. The chest was clear. The apical impulse was forceful in the fifth intercostal space in the midclavicular line and was accompanied by a systolic thrill. The heart sounds were normal, and there were no elides. A grade 4/6 holosystolic blowing murmur was maximally audible at the apex, ·with wide radiation. The splenic tip was easily palpable just below the left costal margin. The extremities were long and thin, with an arm span greater than the height, hyperextensible joints, and arachnodactyly. There were no peripheral stigmata of endocarditis. The hematocrit reading was 36 percent, and the white blood cell count was 8,900/cu mm, with 59 percent neutrophils, 18 percent bands, 20 percent lymphocytes, and 3 percent monocytes. The erythrocytic sedimentation rate was 60 mm/hr. The chest x-ray film was normal, and the electrocardiogram was unremarkable. Five blood cultures dnlwn on the day of admission grew ,s-hemolytic streptococci, and the patient was started on a four-week course of intravenously administered penicillin (20 million units/day) and a twoweek course of intramuscularly administered streptomycin ( 1 gm/day). The patient's fever promptly abated, and the blood became sterile. The erythrocytic sedimentation rate gradually fell, and the hematocrit reading rose slowly.

FiGURE 1. Echocardiogram showing aortic root ( Ao). Aortic internal dimension is dilated ( 4.0 em). LA, Left atrium; and AoV, aortic valve.

REsULTS

The echocardiograms revealed a dilated aortic root measuring 4.0 em at the end of diastole (normal, less than 3.7 cm 6 ) and a normal aortic valve (Fig 1). There was holosystolic prolapse of the posterior mitral valvular leaflet; and despite different gain settings, transducer positions, and angulations, there were multiple, irregular shaggy echoes constantly appearing on the anterior mitral valvular leaflet (Fig 2) . These features were found both early in the patient's hospitalization and on the day of discharge. DISCUSSION

In a recent review of the echocardiographic features of Marfan's syndrome, either dilatation of the aortic root or mitral valvular prolapse (usually of the holosystolic variety) or both features were found in 97 percent of the patients. 8 While aortic insufficiency or dissection or both are regarded as the major cause of death in this syndrome, 1 acute surgical emergencies involving the mitral valvular· apparatus have also been described. 8 Bacterial endocarditis is also a well-recognized complication of

METHODS

Echocardiographic examination was performed several

times during the patient's hospitalization, and a commercially

available echocardiograph coupled to a fiberoptic strip-chart recorder was used. Continuous scans were obtained from the . apex to the aortic root and left atrium, using various gain , settings, transducer positions, and angulations.

CHEST, 70: 6, DECEMBER, 1976

FiGURE 2. Echocardiogram of mitral valve. Note holosystolio prolapse of valve (solid arrow) and shaggy multiple echoes from anterior mitrallea8et, representing bacterial vegetations

(open arrow).

BACTERIAL ENDOCARDms AND MARFAN'S SYNDROME 785

this syndrome 2 •1 and in a recent study was responsible for two deaths among 54 cases of Marfan's syndrome in whom the cause of death was determined. 7 Dillon et al• reported the echocardiographic features of valvular bacterial vegetations, whose existence was documented by surgical or postmortem examination. Lee and associates' have recently described the echocardiographic appearance of bacterial vegetations on the tricuspid valve, which were later found on the surgically extirpated specimen. Although we do not have anatomic confirmation of the presence of bacterial vegetations in our patient, there can be no doubt as to the diagnosis of endocarditis, and the irregular shaggy echoes noted on the anterior mitral valvular leaflet are very comparable to the echoes presented by Dillon et al• and by Lee et al; 11 however, it is unclear how specific this echocardiographic appearance is for active endocarditis, and it is entirely possible that healed endocarditis may give similar echocardiographic findings. Our patient's echocardiograms show the two major cardiovascular features of the Marfan's syndrome, dilatation of the aortic root and mitral valvular prolapse, along with shaggy echoes of the kind described with bacterial vegetations, representing a third cardiovascular feature of the syndrome, the occurrence of bacterial endocarditis.

1 McKusick VA: The cardiovascular aspects of Marfan's syndrome: A heritable disorder of connective tissue. Circulation 11:321, 1955 2 Bowers D, Um DW: Subacute bacterial endocarditis and Marfan's syndrome. Can Med Assoc J 866:445, 1962 3 Wunsch MC, Steinmetz EF, Fisch C: Marfan's syndrome and subacute bacterial endocarditis. Am J Cardiol15:102, 1965 4 Dillon JC, Feigenbaum H. Konecke LL, et al: Echocardiograpbic manifestations of valvular vegetations. Am Heart J 86:698, 1973' 5 Feigenbaum H : Echocardiography. Philadelphia, Lea and Febiger, H172 6 Brown OR, DeMotts H, Kloster FE, et al: Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: An echocardiographic study. Circulation 52:651, 1975 7 Murdoch JL. WalJcer BA, Halpern BL, et al: Life expectancy and cause of death in the Marfan's syndrome. N Engl J Med 286 :804, 1972 8 Simpson JW, Nora JJ, McNamara DG: Marfan's syndrome and mitral valve disease: Acute surgical emergencies. Am Heart J 77:96, 1969 9 Lee CC, Ganguly SN, Magnisalis X:, et al: Detection of tricuspid valve vegetations by echocardiography. Chest 66:432-433, 1974

788 ZVETINA, NATHAN, FACEN

Pulmonary Tuberculosis following Successful Treatment of Pulmonary Infection with Mycobacterium lcansasii* ]ames R. Zvetina, M.D., F.C.C.P.; Norrnan Nathan, M.D.; and Harold Facen, M.D.

A case of pulmonary tuberculosis foUowiug successful treatment of pulmonary iafection with Mycobacterium lumsasii Is presented. The immunizing effect of an iafection with M 1umsasii and other nonspecific immune factors are discussed.

I

nfection with Mycobacterium tuberculosis and M kansasii simultaneously is infrequently observed. Only seven such cases have been observed on the tuberculosis wards of our hospital over a 20-year period since 1955, and they were not included in a study of patients with pulmonary mycobacteriosis due to M kansasii, since it was not known which infection occurred first. Moreover, from Runyon's 1 observations and from our own experience, it is rare for pulmonary tuberculosis to occur after adequate treatment of pulmonary mycobacteriosis due to M kansasii alone. Animals with infections due to mycobacteria other than M tuberculosis have been shown to possess increased resistance to a subsequent infection with M tuberculosis. 2-6 Between 1955 and 1975, a total of 225 patients with pulmonary disease due to M kansasii alone have been observed at Veterans Administration Hospital, Hines, Ill. With few exceptions, all patients were initially treated in an environment of high infectivity. Among these 225 patients, there was only one instance of pulmonary tuberculosis that developed following infection with M kansasii.

CASE

REPoRT

A 39-year-old machinist was found to have an infiltrate in the left upper lobe on a routine chest roentgenogram m February of 1969 (Fig 1) . All sputum samples were positive on culture for M kansasl.i. The patient received three months of therapy with isonazid through a local tuberculosis clinic and was subsequently lost to follow up. On Oct 30, 1970, the patient was admitted to Hines Veterans Administration Hospital for excision of a local large ulcerative carcinoma of the scalp. A chest roentgenogram on admission revealed a fibronodular lesion involving the left apex and infraclavicular region. Cultures of the patient's sputum grew M kansasil on several occasions. Therapy' with streptomycin, isoniazid, and p-aminosalicylic acid was instituted. An initial indirect drug-susceptibility test subsequently showed the organisms to be completely resistant to isoniazid (51-'g/ml), to p-aminosalicylic acid (251-'g/ml), and to ethambutol ( 101-'g/ml) and streptomycin ( 101-'g/ml) . •From the Medical Service, Veterans Administration Hospital, Hines, Ill. Reprint requests: Dr. Zvetina, VA Hospltal, Hines, Illlnais 60141

CHEST, 70: 6, DECEMBER, 1976