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Intralobar Pulmonary Sequestration Infected with a Mycobacterium of the Battey-Avium Complex
the fifth arches in man remains controversial, leading us to favor the theory of ductal origin of the pulmonary arteries with secondary ahsorption into the aorta . Successful correction of anomalous origin of the pul monary arteries has been carried out on 19 occasions." Although the surgical procedure undertaken in this case was feasible. the approach would have been different had the proper diagnosis been available. The posterolateral approach did not allow adequate visualization of the right pulmonary artery, and cross-damping the entire right ventricular outflow tract for the time required to complete an anastomosis was hazardous . The best exposure for total correction of this malformation would be via a hilateral fourth-interspace approach, transecting the sternum. A median sternotomy would compromise the exposure of the descending thoracic aorta and would he less suitable. The use of cardiopulmonary bypass would afford an increased margin of safety. Under any circumstances the defect is not compatible with prolonged survival, and early surgical correction is mandatory. REFERENCES
1, 2, 9 References available from author on request. 3 CucciCE, Doyle EF, Lewis EW Jr : Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 2B: 124, W64 4 Kauffman SL, Uao AC, Weblx-r CB, t't al : Origin of the right pulmonary artery from the aorta. Am J Cardiol 19:741, H)fj7 5 Wagenvoort CA, Xeufeld H:'I:, Birge RF, et al : Origin of the right pulmonary artery from the ascending aorta. Circulation 23 :84, 19(H 6 Pool PR, Vogel JHK, Blount se Jr: Congenital unilateral absence of a pulmonary artery. Am J Cardiel 10:706, 1962 7 Amhrus G : Congenital absence of the right pulmonary artery with bleeding into the right lung. J Tech Meth 15: 103, H).'36 8 O'Dell JE, Smith JC : Right pulmonary artery arising from ascending aorta. Am J Dis Child 105 :53, 1963
Intralobar Pulmonary Sequestration
A
patient with an intralobar pulmonary sequestration infected with a Mycobacterium of the Battey-avium complex is presented. This case illustrates possible routes of infection in intralobar pulmonary sequestrations and the difficulties of managing patients with Battey-avium disease. CASE HEPOHT
The patient was a 24-year-old white man who presented to the US Air Force Medical Center at Keesler Air Force Base, Mississippi, in March, W74 complaining of a persistent cough . He denied fever, chills, night sweats, weight loss, or exposure to tuberculosis. A history of repeated right-sided pneumonias was obtained, and documentation of a persistent right lower-lobe infiltrate on chest x-rav films existed since H)68. A previous intermediate-strength purified protein derivative of tuberculin (PPD) skin test was negative. Physical examination revealed an afebrile, slightly obese man . On auscultation of the lungs, crepitant rales were heard over the right posterior lung field. The remainder of the examination was unremarkable. Laboratory data showed a hematocrit reading of 46 percent and a white blood cell count of 9,200/cu mm with a normal different ial count. The blood glucose level was 92 mg/ 100 ml. The results of determinations for sweat chlorides, serum protein electrophoresis, and immunoglobulins were normal. On chest x-rav film a thin-walled Cyst in the right lower lohe was noted ' (Fig 1) . Multiple cystic areas in the right lower lobe were demonstrated hy tomography. An intermediate-strength PPD test was negative. A secondstrength PPD was positive with 14 mm of induration at 48 hours. The results of fungal serologic studies were negative, except for Histoplasma capsulatum "ew" and "VC" yeast phases at 1:16 and 1:8 dilution, respectively. Cultures of a transtracheal aspirate failed to grow bacteria or Mycoplasma. Rigid bronchoscopy revealed inflammatory changes in the right lower-lobe bronchus and its segmental bronchi. The results of hronchography and pulmonary function tests were normal. Direct smears of induced sputum demonstrated numerous acid-fast bacilli. Multiple sputum samples were sent to the Epidemiology Laboratory at Lackland Air Force Base, Texas, for identification of the organism. The patient was started on 300 mg of isoniazid, 2 gill (25 mg/kg ) of ethambutol, and 1 gm of streptomycin daily. He was discharged in late June 1974 after conversion of his SPUtUIll smears. Cultures subsequently grew an atypical Mycobacterium of the Battey-avium group. A sensitivity
Infected with a Mycobacterium of the Battey-Avium Complex* Lewis R. Mooney, M.D.; Joseph W . Brown, III, M.D .; and Robert L . Saunders, Jr ., M.D .
A 24-year-old man with an intralobar pulmonary sequestration infected with a Mycobacterium of the Batteyavium complex is presented and discussed. °From the Department of Medicine, US Air Force Medical Center, Keesler Air Force Base, \liSSisSifPi. The views expressed herein are those 0 the authors and do not necessarily reflect the views of the US Air Force or the Department of Defense. ReZlrint requests: Maior Mooney, HMR 2085, Keesler AFB, Bi oxi, Mississippi 39534
594 MOONEY, BROWN, SAUNDERS
«'lGURE
1. Chest x-ray film showing right lower-lobe infiltrate .
CHEST, 68: 4, OCTOBER, 1975
pulmonary sequestrations are discussed elsewhere. I·,. Proposed routes of entry for organisms infecting an intralobar pulmonary sequestration include entry via the normal bronchial sys tem .l-" direct spread from adjacent infected areas,' and hematogenous spread." In our patient, ca seating granulomas were identified in both the sequestered segment and in the res ected portion of the right lower lobe. This suggests that the organisms responsible for the granuloma formation were deposited in the sequestered segment either by lymphohematogenous di ssemination or by direct extension from the adjacent infected right lower lobe. Once it was documented that our patient had progressive disease caused by an organism of the Battey-avium complex, we followed the current consensus that the treatment of choice is multiple drug regimens and surgical resection of discretely involved lung (when possible) .6 . ; Limiting our drug regimen to three agents with in vitro effectiveness against the infecting strain should giv e maximum effectiveness and lessen the inconvenience, side effects, and toxicity of current five-drug and six-d ru g regimens. This case lends support to the theory that lymphohematogenous spread is one route whereby infection enters an intralobar pulmonary sequestration . It also illustrates th e problem of managing Battey-aviurn infections. FIGURE 2. Selective inject ion of feeding artery arising from abdominal area . Note notching as artery passes through diaphragm . report from National Jewish Hospital, Denver, revealed it to be resistant to isoniazid, sodium aminosalicylate, and streptomycin , but sensitive to ethionamide (15 .0}Lg/ml), rifampin (5.0}Lg/ml), and ethambutol (25 .0}Lg/ml). While on antituberculosis therapy, the patient clinically improved ; however, his right lower-lobe infiltrate remained unchanged. In September, 1974 the patient was readmitted for evaluation of the adequacy of his antituberculosis therapy, the persistent right lower-lobe infiltrate, and consideration for surgical resection of the right lower-lobe area . An aortic angiogram showed the right lower-lobe area was fed by an artery arising from the abdominal aorta (Fig 2) . Venous drainage was noted into the right pulmonary venous system . A diagnosis of intralobar pulmonary sequestration was made. The patient underwent right lower lobectomy for removal of the sequestered segment. Gross examination of the sequestered segment revealed it to be cystic without demonstrable bronchial connection. Microscopic examination revealed caseating granulomas to be present in the sequestered segment and the adjacent right lower lobe. Smears of the sequestered segment were negative for acid-fast bacilli; however, smears of the adjacent right lower lobe revealed rare acid-fast bacill i. Cultures of the surgical specimen were negative, The patient is currently doing well on therapy with ethionamide, rifampin, and ethambutol. He will be treated for 24 months . DISCUSSION
The diagnosis, the radiologic and embryologic findings, and the treatment of patients with intralobar
CHEST, 68: 4, OCTOBER, 1975
We wish to thank Dr. Harold A. Lvons for his valuable suggestions and comments. Ms. P. Mason provided secretarial help, and Ms. C. James assisted in library research. ACKjI;O\VLEDG~fEl':TS :
REFERENCES
Ranniger K, Valvassori GE : Angiograph tc diagnosis of intralobar pulmonary sequestration. Am J Roentgen 92 :540546, 1964 :2 Saegesser F, Besson A: Extralobar and intralobar pulmonary sequestrations of the upper and lower lobes: Report of 11 cases, including one case of scimitar syndrome. Chest 63 :69-73 , 1973 3 Gallagher PG, Lynch JP, Christian HJ : Intralobar bron chopulmonary sequestrations of the lung : Report of two cases and review of the literature. N Engl J Med 257 :643650, 1957 4 Garard FP, Lyons HA: Anomalous artery in intralobar bronchopulmonary sequestration : Report of two cases demonstrated by angiography. N En/otl J Med 259 :662-600, 1958 5 Pryce D: Lower accessory pulmonary artery with intralobar sequestration of the lung : Report of seven cases. J Path Bact 58 :457-467, 1946 6 Committee on therapy of the American Thoracic Society : Status of disease due to unclas sified Mycobacterium. Am Rev Resp Dis 87 :459-461,1963 7 Yeager H, Raleigh JW : Pulmonary disease due to Mycobacterium intracellularis. Am Rev Resp Dis 108:547-551 , 1973
INTRALOBAR PULMONARY SEQUESTRATION 595
1, 2, 9 References available from author on request. 3 CucciCE, Doyle EF, Lewis EW Jr : Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 2B: 124, W64 4 Kauffman SL, Uao AC, Weblx-r CB, t't al : Origin of the right pulmonary artery from the aorta. Am J Cardiol 19:741, H)fj7 5 Wagenvoort CA, Xeufeld H:'I:, Birge RF, et al : Origin of the right pulmonary artery from the ascending aorta. Circulation 23 :84, 19(H 6 Pool PR, Vogel JHK, Blount se Jr: Congenital unilateral absence of a pulmonary artery. Am J Cardiel 10:706, 1962 7 Amhrus G : Congenital absence of the right pulmonary artery with bleeding into the right lung. J Tech Meth 15: 103, H).'36 8 O'Dell JE, Smith JC : Right pulmonary artery arising from ascending aorta. Am J Dis Child 105 :53, 1963
Intralobar Pulmonary Sequestration
A
patient with an intralobar pulmonary sequestration infected with a Mycobacterium of the Battey-avium complex is presented. This case illustrates possible routes of infection in intralobar pulmonary sequestrations and the difficulties of managing patients with Battey-avium disease. CASE HEPOHT
The patient was a 24-year-old white man who presented to the US Air Force Medical Center at Keesler Air Force Base, Mississippi, in March, W74 complaining of a persistent cough . He denied fever, chills, night sweats, weight loss, or exposure to tuberculosis. A history of repeated right-sided pneumonias was obtained, and documentation of a persistent right lower-lobe infiltrate on chest x-rav films existed since H)68. A previous intermediate-strength purified protein derivative of tuberculin (PPD) skin test was negative. Physical examination revealed an afebrile, slightly obese man . On auscultation of the lungs, crepitant rales were heard over the right posterior lung field. The remainder of the examination was unremarkable. Laboratory data showed a hematocrit reading of 46 percent and a white blood cell count of 9,200/cu mm with a normal different ial count. The blood glucose level was 92 mg/ 100 ml. The results of determinations for sweat chlorides, serum protein electrophoresis, and immunoglobulins were normal. On chest x-rav film a thin-walled Cyst in the right lower lohe was noted ' (Fig 1) . Multiple cystic areas in the right lower lobe were demonstrated hy tomography. An intermediate-strength PPD test was negative. A secondstrength PPD was positive with 14 mm of induration at 48 hours. The results of fungal serologic studies were negative, except for Histoplasma capsulatum "ew" and "VC" yeast phases at 1:16 and 1:8 dilution, respectively. Cultures of a transtracheal aspirate failed to grow bacteria or Mycoplasma. Rigid bronchoscopy revealed inflammatory changes in the right lower-lobe bronchus and its segmental bronchi. The results of hronchography and pulmonary function tests were normal. Direct smears of induced sputum demonstrated numerous acid-fast bacilli. Multiple sputum samples were sent to the Epidemiology Laboratory at Lackland Air Force Base, Texas, for identification of the organism. The patient was started on 300 mg of isoniazid, 2 gill (25 mg/kg ) of ethambutol, and 1 gm of streptomycin daily. He was discharged in late June 1974 after conversion of his SPUtUIll smears. Cultures subsequently grew an atypical Mycobacterium of the Battey-avium group. A sensitivity
Infected with a Mycobacterium of the Battey-Avium Complex* Lewis R. Mooney, M.D.; Joseph W . Brown, III, M.D .; and Robert L . Saunders, Jr ., M.D .
A 24-year-old man with an intralobar pulmonary sequestration infected with a Mycobacterium of the Batteyavium complex is presented and discussed. °From the Department of Medicine, US Air Force Medical Center, Keesler Air Force Base, \liSSisSifPi. The views expressed herein are those 0 the authors and do not necessarily reflect the views of the US Air Force or the Department of Defense. ReZlrint requests: Maior Mooney, HMR 2085, Keesler AFB, Bi oxi, Mississippi 39534
594 MOONEY, BROWN, SAUNDERS
«'lGURE
1. Chest x-ray film showing right lower-lobe infiltrate .
CHEST, 68: 4, OCTOBER, 1975
pulmonary sequestrations are discussed elsewhere. I·,. Proposed routes of entry for organisms infecting an intralobar pulmonary sequestration include entry via the normal bronchial sys tem .l-" direct spread from adjacent infected areas,' and hematogenous spread." In our patient, ca seating granulomas were identified in both the sequestered segment and in the res ected portion of the right lower lobe. This suggests that the organisms responsible for the granuloma formation were deposited in the sequestered segment either by lymphohematogenous di ssemination or by direct extension from the adjacent infected right lower lobe. Once it was documented that our patient had progressive disease caused by an organism of the Battey-avium complex, we followed the current consensus that the treatment of choice is multiple drug regimens and surgical resection of discretely involved lung (when possible) .6 . ; Limiting our drug regimen to three agents with in vitro effectiveness against the infecting strain should giv e maximum effectiveness and lessen the inconvenience, side effects, and toxicity of current five-drug and six-d ru g regimens. This case lends support to the theory that lymphohematogenous spread is one route whereby infection enters an intralobar pulmonary sequestration . It also illustrates th e problem of managing Battey-aviurn infections. FIGURE 2. Selective inject ion of feeding artery arising from abdominal area . Note notching as artery passes through diaphragm . report from National Jewish Hospital, Denver, revealed it to be resistant to isoniazid, sodium aminosalicylate, and streptomycin , but sensitive to ethionamide (15 .0}Lg/ml), rifampin (5.0}Lg/ml), and ethambutol (25 .0}Lg/ml). While on antituberculosis therapy, the patient clinically improved ; however, his right lower-lobe infiltrate remained unchanged. In September, 1974 the patient was readmitted for evaluation of the adequacy of his antituberculosis therapy, the persistent right lower-lobe infiltrate, and consideration for surgical resection of the right lower-lobe area . An aortic angiogram showed the right lower-lobe area was fed by an artery arising from the abdominal aorta (Fig 2) . Venous drainage was noted into the right pulmonary venous system . A diagnosis of intralobar pulmonary sequestration was made. The patient underwent right lower lobectomy for removal of the sequestered segment. Gross examination of the sequestered segment revealed it to be cystic without demonstrable bronchial connection. Microscopic examination revealed caseating granulomas to be present in the sequestered segment and the adjacent right lower lobe. Smears of the sequestered segment were negative for acid-fast bacilli; however, smears of the adjacent right lower lobe revealed rare acid-fast bacill i. Cultures of the surgical specimen were negative, The patient is currently doing well on therapy with ethionamide, rifampin, and ethambutol. He will be treated for 24 months . DISCUSSION
The diagnosis, the radiologic and embryologic findings, and the treatment of patients with intralobar
CHEST, 68: 4, OCTOBER, 1975
We wish to thank Dr. Harold A. Lvons for his valuable suggestions and comments. Ms. P. Mason provided secretarial help, and Ms. C. James assisted in library research. ACKjI;O\VLEDG~fEl':TS :
REFERENCES
Ranniger K, Valvassori GE : Angiograph tc diagnosis of intralobar pulmonary sequestration. Am J Roentgen 92 :540546, 1964 :2 Saegesser F, Besson A: Extralobar and intralobar pulmonary sequestrations of the upper and lower lobes: Report of 11 cases, including one case of scimitar syndrome. Chest 63 :69-73 , 1973 3 Gallagher PG, Lynch JP, Christian HJ : Intralobar bron chopulmonary sequestrations of the lung : Report of two cases and review of the literature. N Engl J Med 257 :643650, 1957 4 Garard FP, Lyons HA: Anomalous artery in intralobar bronchopulmonary sequestration : Report of two cases demonstrated by angiography. N En/otl J Med 259 :662-600, 1958 5 Pryce D: Lower accessory pulmonary artery with intralobar sequestration of the lung : Report of seven cases. J Path Bact 58 :457-467, 1946 6 Committee on therapy of the American Thoracic Society : Status of disease due to unclas sified Mycobacterium. Am Rev Resp Dis 87 :459-461,1963 7 Yeager H, Raleigh JW : Pulmonary disease due to Mycobacterium intracellularis. Am Rev Resp Dis 108:547-551 , 1973
INTRALOBAR PULMONARY SEQUESTRATION 595