Alveolar Proteinosis* Lobar Lavage by Fiberoptic Bronchoscopic Technique Ber nard B. Brach , M .D .; [am es 1/ . Hurrell, M .D ., F .C .C.J' .; and Ken net h M . Moser , M .D ., F .C .C .I' .

Lavage limited to an isolated lobe was perform ed on multiplc occasions using a cuffed fihcroptic bronchoscope in a patient with alveolar protcinosis. Seq uential ventilationperfusion sci ntiphotosca ns were used to pre select and follow the functional behavior of the lavaged lobe, Lavage led to function al improvem ent. The technique of fiberoptic broncboscopic lobar la vag e is sim ple and ma y find application in patients in whom lavage of an entire lung ma y be hazardous.

T

h e b enefi ci a l use of lu n g la va ge in pul m on ary a lveola r p roteino sis h a s h een d emo n st rated in th e p ast. T o d a te , th ree t e ch n iqu es ha ve be en u sed : segmental irri g a tion via ca th ete r , I w hole lun g la v a g e via a h ro n cho sp irom e tric ca the te r ," an d ca the te r lobar la va g e in th e m a nn er o f F in ley." Th e pu rp o se o f thi s re port is tw o fol d : ( 1) to d escribe a te chnique of selective lob ar la v a g e usin g th e Iiber op tic b ron ch oscope , a n d ( 2) to rel at e th e seq uen tia l c hanges in pulmonary fu nction an d ven tilatio n-perfus ion sc in tip h o to sc a ns observed in a p atie nt w ith a lveolar p roteino sis in w hom thi s te chniq u e w as a p p lie d . CASE H EPO HT

T his 30 -yea r-old white ma n was first ad m itted to th e Un ive rsity Hosp ita l, San Diego, Calif, on Sep t 4, 1973 for ch ro nic alcoho lism a nd a lcoho l wi thdrawal. Durin g that ad m ission, a ches t x-ray film showed pa tch y uppe r lob e infiltrates ( F ig 1 ) . W ith the e xce p tion of a d u ll discomfort in th e a nte rior po rtion of th e ches t, the patie nt de nied any resp iratory sy mp toms. Th e pat ie nt had smoke d two packs of ciga re ttes per d ay for ten years a nd had wo rked, on occa sion , as a sandb las ter . Th e findings from ph ysical exa mina tion wer e norm a l; speci fica lly, th e lun gs wer e u n re ma rka ble. The pa tien t lef t th e hosp ita l before furt he r eva lua tion cou ld be ac complished . Th e patient was again admitted in Novem ber 1973 for alc oh ol wi thdrawal. A chest x-ra y film showed slight progression o f th e infiltrates. Once mor e, th e patie nt sig ned ou t of th e hosp ital. In Fe b rua ry 1974 , th e pati ent was a d m itted for a me tha q ua lone overdose . A che st x-ray film showed fu rther p rogr ession of th e patchy nodu lar reticu lar infil tra tes . Ph ysica l exam ina tion rev e a led nor mal vita l sig ns. C ya nos is and d ubbi ng we re ab sent. F in d ings from exa m ina tion of the ches t we re unre markab le, wit h th e e xce p tion of tubu la r b reath • F rom the Pul monar y D ivision, Un ive rsity Hospital, a nd th e Depa rt me nt of Med icine , Schoo l of Med icine , Un ive rsity of Ca lifornia, San Diego. T his p roject was su pported by i':a tio na l lI ea rt a nd Lun g Inst itute grants il L 14IG 9 and il L 00 134. Re p rin t req uests: Dr . M ose r, 225 ' Vest Di ck inson , San Di e go 92103

224 BRACH, HARRELL, MOSER

FI( ; U Il E 1. C hes t roe ntgenogram of pati e nt sho ws pat ch y infiltrate pri marily in uppe r lobe.

soumis in th e le ft ap ex. La bora tory studies revealed a hem a tocri t rea di ng of 44 pe rce nt a nd a wh ite blood cell cou nt of 9,8 00/ cu rnm , with a no rma l d iffer ential cou nt. Pulmo nary fun ction results disclosed no signi fica nt abnorm a lit ies ( T able I ) . An ope n- lung biopsy was performed on Feb II , 1974 , th rough a right a n terior th oracotomy. Th e path ologic dia gnosis was a lveola r p rot e inosis. Se ve ra l mon ths la te r, th e pa tie nt develop ed a per sisten t nonp roduct ive cough and moderate exert iona l dys pnea . C hest a uscu lat ion rev ea led d iffuse fine ra les. Pu lmonary function studi es showed a loss in lu ng volum es, a decr em en t in flow rat es, and mild restin g hypox em ia ( T a ble I ) whi ch was considered past the time interval wh en pos tthoracoto my cha nge s would p e rsist .! Ve ntilation- perfusion scintip ho togra p h ic studi es in Ma y 1974 showe d several ma tched a rea s of hyp ope rfusion an d h yp oventila tion in th e upp e r and m id dle zones of both lu ngs. On la te ra l per fusion sca ns, pat chy areas of h yp op erfusion we re noted in the le ft upper lobe wi th lingu la r d istrib u tion ( Fi g 2 ). In conside ra tion of the pa tient's fun ctional decr em en t, he wa s admitted for lobar la vage via th e fibe rop tic bronchosco pe. M ethod Based on th e ventilatio n-perfusion stu dy , th e lef t upper lobe was se lec te d for lavage . Du ring the p rocedure, oxyge n was sup p lie d wi th a modi fied 60-per ce nt oxygen ma sk ( Ve ntimask ) by th e meth od describe d by Albe rtin i and associa tes.? A fibe ropt ic b ron ch oscope ( O lymp us mode l .5 BF2 ) wa s mo d ified with a 5 -mm tra cheostomy cuff ( Dit tm an ) with a le ngthe ned inflation line . Th e bronchosco pe was pass ed th rough th e nose via the modi fied oxyg en mask into the left uppe r-lob e bronchus . The ba lloon wa s th en inflated to sea l, and ap proximate ly 2,00 0 m l of room te mp e ratu re normal sa line solution ( p l l, 5 .5 ) was instilled via syringe in 50 to 100 ml a liq uots, a lte rn a ting la va ge a nd suc tio n. The e ntire p roce d ure lasted ap prox ima te ly 45 min ut es. Blood gas leve ls wer e monitored th rough ou t th e procedure . After th e lava ge, seria l blood gas de termi na tio ns wer e per form ed , a nd th e patie nt was mai nta ined on a GO per ce nt oxyge n mask un til th e a rteri al

CHEST, 69: 2, FEBRUARY, 1976

Tahl e l -Rl-suLt.• 01 Pulnionary Fu u etion S tu d ies

Dat e :\k as ur e men t '

Pred icted

2/ 6/74

,,) /10/ 74

/ 12/74 "

10/ 2.') / 7·1

VC , ml F HC , ml H V, 1111 TLC ,ml HV/TLC % FEF,s, ,, %, L/ sec l'aO, l'a CO, pll VD /VT % l'aO , with F lo, of 1.0

5,352 3,778 I,!!!!-I 7,-I711 35 > 3_00 > 80 38-42 7.:38-7.-12

4,6!l7 3,2 12 1,72!! 1l,-I211 211 :U iI 8:3 35 7.-111 28 % 50-1

4,24!! :3, \ ·1 \ ,-1 38 5,ll87

·1..1&1 :3,.') I.') 1,!l-1 :3 1l,-I07 :30 2.7\ 76

-1, 762 :3,.')-1 1,\ IlO 5,\128 20 2.110 7!) :15 7.-12

> 1150

?_ :J -

2.!!3 72 32 7.-1-1 3-1 % 551

:31 7.-15

' VC, Vital ca pac ity ; FR C , functio na l residual ca pac ity ; RV , residu al vo lume; TLC , tota l lun g ca pacity ; HV/ T LC % , ratio of residual volume to totu l luru; ca pac ity expressed as per cen t ; FE 10" •. 70% , mea n for ced expira tory flow du ring th e mid dl e half of th e forced vita l r-apiu- it y ; PaC O" a rte r ia l ca rb o n dioxid e te nsio n ; VD /VT % , rat io of ph ysiologic dea d-s pac e vo lume to t ida l vo lum e expresse d as pe rr-ent.; a nd F l o" fra ctional conce nt rat ion of oxygen in insp ired a ir. "Six week s uf'tr-r th ird lav uge. bl ood gas le vels had approach ed ba se line values. Ventilationperfusion sci ntip ho tosca ns we re obta ined imm ed iat el y following lava ge a nd 24 hours lat e r. Several lavages wer e d on e in d iffere nt lob es ove r th e ne xt several months. H ESULTS

Each procedure was we ll tolerat ed , a nd 110 com plication occ ur red . Co nsidera ble p rot ein aceou s mat eri al was removed durin g each lavage. Unrecovera ble lavage fluid

vari ed from 200 to 400 m\. The seq ue nce aft er eac h lavage was similar. A widening of the alveolar-arterial oxyge n pressur e differen ce lP( A-a) OJ occ ur red durin g the procedure a nd persisted for approximately five hours afte r lava ge (Table 2 ) . This was associa ted with immedi at e pos tlavage scin tipho tog raphs wh ich demonst rat ed a mark ed decrement in ventil ati on in th e lavaged area with a more mod est decrease in perfusion to that area ( Fig 3 ). T hus , lavage produced a "low ventila tion-pe rfus ion" zone. Thi s was associa ted with a radi ographic infiltrate and the wid en ed P ( A-a ) 0 ". However, by 24 hours after lava ge, the radiographic infiltrat e and wid en ed P ( A-a) 0 " had resolved , and th e ventil ation -perfusion scintipho togra phs dem onstra ted improvement in ventilation and sugges tive imp rovement in perfusion when compa red with the prelavage study. La vage was rep eat ed on thr ee occasion s over th e next several months . Sequential pulmonar y fun ction studies have shown an increm ent in lun g volum es and a rterial oxyge n pressure ( PaOJ (Table I ). Chest x-ray films ha ve shown littl e int erval chan ge. Clinically, the pati ent has noted a slight improvem ent over the last few months but still complains of mild d ysp nea on exertion. Ta h le 2-

2. Perfusion scintiplaotosca ns ( a ll four views) show decrem ent in perfusion ( arrows ) most prominent in upper and midd le lung zon es. FI G UlIE

CHEST, 69: 2, FEBRUARY, 1976

Artl'r inl IlloOfI Gu s Lerel« b ef or e , d uring: u sul a f t e r L a ru g e

T ime

Fl o?

PaO,

Pa CO,

p ll

P(A-a )O,

Before 8 A ~I

0.21

8-1

28

7.-17

38

During !! A ~I !!:30 A M

0.,')0 0.50

83 711

28 311

7.-17 7.-1\

2-15 2:35

Aft er 10 A ~I :3 I' ~I

0.50 0.21

82 711

-10

3,,)

7.37 7.-111

23,,) 3ll

* F'!Ot, Fracti on a l eoncentrat ion of oxyg en in inspired a ir.

ALVEOLAR PROTEINOSIS 225

FIGUHE 3. Posterior ventilation ( bottom) and perfusion ( to p) scans before ( left), immediately after (ce nter ) , and 24 hours after ( rig h t ) left lower lobe lavage. ~ostlav age scan demon:tra.tes marked decrement in ventilation and modera te decrease .in perfusion . At 24 hours, ventilation has improved and perfusion appears slightly improved compared to prelavage studies.

DISC USSI O N

Bronch opulmon ary lavage has been used in th erapy for alveo lar proteinosis," asth ma ,' a nd cys tic fibrosis' since Ham irez-H'' offere d it as an alt ern at ive to seg me n tal irrigati on in 1966. The pot ential hazard of seve re hypoxemia" and th e difficul ty of th e techniq ue (which involves tra ch eal divisi on via a Carlens br onchospiro metric tube, deg assing the lun g, 100-per cent oxygen b reathin g, a nd gene ral a nes thes ia) ha s limited its useful applica tion to a few medical cen ters and th e mor e adva nce d cases. In an effort to red uce th e probl em s of gas excha nge, Rogers and Tantam 10 sug ges te d specific guide lines for lavage with reduced filling and emp tyin g tim e. Seard et al II circumve nted th e p robl em s with unilateral lavage in child ren b y placing his patients on car diopu lmonary bypass and doin g bilater al lung lavages. An alt ernative to th ese pro cedures is selecte d lobar lavage. Using a technique of lob ar lava ge developed by Finlev Harri s e t al 3 recentlv reported successful man agem'e~t of moderately ad~anced alveo lar p rot ein osis. Unde r topi cal anes thes ia, a cuffed bronch ographic cathe te r is placed during fluoroscopi c examina tion so th at lav age can be perform ed in selec ted area s. A maximum volume of 1,000 ml wa s lava ged in 50-ml aliq uo ts. Fluoroscopic examina tion is a necessity in this method , since selec tive placem en t of a b ron chi al ca the te r is diffi-

226 BRACH, HARRElL, MOSER

cult.!" Even in ade q ua te ly anes the tized patients, our expe rience indic a tes th at la vage stimula tes coug hing, which usually dislod ges th e ca thete r. Repl acem ent of th e ca the te r in to th e selec ted bro nch us can be a ma jor problem. Select ed lavag e wit h isolation of th e involved lob es is easily accomplishe d by direct visual placem ent of th e fiberoptic broncho scope and inflation of the a ttac he d cuff. Repl acem ent after a tussive exp ulsion is readil y accomplished. A lavage volume of 1,500 ml to 2,000 ml ca n be deliv er ed in 50 to 100-ml aliq uo ts witho ut difficulty. Fo r th e a nxious pati ent, intravenous administration of dia zepam ma y be useful . If desired, additional cont rol of th e airway ma y be ob ta ine d by passin g the fiberoptic b ron ch oscop e via an endo trache al tub e. The pati ent sho uld be given high-fl ow oxyge n b y mask to assure ade q uate oxygena tion durin g and afte r th e proce d ure. Preliminar y ve ntila tion- pe rfusion scintiphotoscans are useful in defining th e a re as which ar e most compromised . This assures th at areas whi ch are supporting gas excha nge rem ain un compromised during lava ge. Scintiphotoscans also are of value in following th e postlava ge even ts. A gr eater decrem ent in ventilation than in blo od flow to th e lav aged lun g zon e, with th e crea tion of a low ven tilation-pe rfusion zone, was dem onstrat ed afte r lavage several tim es in thi s pati ent. A con tra la ter al shift in distribution of perfusion also was dem onstrated . Other

CHEst 69: 2, FEBRUARY, 1976

authors have attributed this shift in blood flow to an increased alveolar pressure in the fluid-filled lung. lO However, in our patient, the shift was noted at a time when the lung was not filled with fluid. It appears likely that the perfusion shift we observed was a response to regional alveolar hypoxia, related to the postlavage decrement in ventilation.Pr'" Sequential ventilation-perfusion scintiphotographic studies also were useful in documenting restoration of gas exchange prior to lavage of an additional area. The functional impairment in the patient described was initially modest, and the improvement noted could be ascribed to spontaneous improvement as well as to the lavages performed. However, the most important aspect of the lavage sequence described was the ease and safety with which such focal lavage was applied to this patient. This suggests its possible extension to other clinical situations in which lavage would be helpful, but in which a temporary decrease in the gas-exchange function of an entire lung would not be acceptable. REFERENCES

I McLaughlin JS, Ramirez RJ: Pulmonary alveolar proteinosis. Am Rev Respir Dis 89:745, 1964 2 Ramirez-R J: Pulmonary alveolar proteinosis. Arch Intern Med II9:147, 1966 3 Harris JO, Castle JR, Swenson EW, et al: Lobar lavage: Therapeutic benefit in pulmonary alveolar filling disorders. Chest 65:655-659, 1974 4 Gorlin R, Knowles J, Storey C: Effeets of thoracotomy on pulmonary function. J Thorac Surg 34:242,1957 5 Albertini RE, Harrell JH II, Moser KM: Management of arterial hypoxemia induced by fiberoptic bronchoseopy. Chest 67:134-136,1975 6 Ramirez-R J: Alveolar proteinosis: Importance of pulmonary lavage. Am Rev Respir Dis 103:666, 1970 7 Rogers RM, Tantam KR: Bronchopulmonary lavage: A "new" approach to old problems. Med Clin North Am 54:617-629, 1970 8 Rausch DC, Spock A, Kylstra JA: Lung lavage in cystic fibrosis. Am Rev Respir Dis 101:1006, 1970 9 Smith JD, Millen JE, Safar P, et al: Intrathoracic pressure, pulmonary vascular pressure and gas exchange during pulmonary lavage. Anesthesiology 33:401-405, 1970 10 Rogers RM, Tantam KR: Hemodynamic response of the pulmonary circulation to bronchopulmonary lavage in man. N Engl J Med 286:1230,1972 II Seard C, Wasserman K, Heimlich EM: Simultaneous bilateral lung lavage using partial cardiopulmonary bypass. Am Rev Respir Dis 101:877, 1970 12 Haberman PB, Green JP, Archibald C: Determinants of successful selective tracheobronchial suctioning. N Engl J Med 289:1060-1063,1973 13 Lopez-Majano V, Wagner HN, Twining RH, et al: Effects of regional hypoxia on the distribution of pulmonary blood flow in man. Circ Res 18:550, 1966 14 Fowler KT, Read J: Effect of alveolar hypoxia on regional distribution of pulmonary blood flow. J Appl Physiol 14:244-250, 1963

CHEST, 69: 2, FEBRUARY, 1976

Melioidosis Complicated by Pericarditis* Martin]. Raff, M.D.;"" Nelson Lamkin, lr., M.D.;t John Braun, M.D.;:j: and Patricia Barnwell§

A case of acute and recrudescent melioidosis complicated by pericarditis and pericardial effusion is described. The potential for the appearance of future cases in the United States and the necessity for physicians to remain aware of this potential diagnosis are discussed.

I

n 1912 Whitmore and Krishnaswami- first described the pathologic features of a "disease somewhat resembling but really easily distinguishable from glanders." It was recognized from postmortem findings of "the ill-nourished, neglected, wastrels of the town [Rangoon]." Stanton and Fletcher- suggested the name "melioidosis" (Greek, "a resemblance to the distemper of asses") and were the first to make the diagnosis antemortern." The term, melioidosis, encompasses a wide variety of clinical infections ranging from latent to acute systemic disease. The appearance of pericarditis and pericardial effusion with melioidosis is a distinctly unusual complication. CASE REPORT

A 19-year-old man developed fever and cough in November 1971, three months following his arrival in South Vietnam. A chest x-ray film revealed a right upper lobe infiltrate. Fever persisted during the next six weeks, and the cough became productive of one cup of yellow sputum per day. The right upper lobe infiltrate became more pronounced, accompanied by formation of a 2-cm thin-walled cavity. On Jan 7, 1972, the patient complained of midsternal chest pain unrelated to respiration, and his electrocardiogram demonstrated first-degree atrioventricular block and ST-segment elevation with decreased T-wave amplitude in leads 1,2, aVF, and V4 to V6. A pericardial friction rub, most pronounced along the left sternal border, became audible four days later. Skin tests with histoplasmin and purified protein derivative of tuberculin (PPD) were negative. Sputum cultures grew Pseudomonas pseudomallei. Tetracycline therapy at a dosage of 3 gm per day orally was begun on Jan 10. A chest x-ray film on Jan 13 revealed an enlarged cardiac shadow, minimal infiltrate, and persistence of the cavity in the right upper lobe. The patient responded well to therapy, with resolution of both clinical and electrocardiographic signs of pericarditis, return of cardiac shadow to normal size, clearing of infiltrate, and diminution in the size of the lung abscess. Convalescent serum studies on Feb 8 revealed a complement fixation titer of 1:512 and a hemagglutination titer of 1:2,560 for antibodies to melioidosis. A chest x-ray film taken on Feb 9 "From the Department of Medicine, University of Louisville School of Medicine, Louisville, and Ireland Army Hospital, Ft. Knox, Kentucky. ""Chief, Section of Infectious Diseases, University of Tennessee School of Medicine, Memphis. i'Fellow, Section of Allergy and Immunology. tFirst Year House Officer, Department of Medicine, University of Alabama in Birmingham. §Research Associate in Infectious Diseases. Reprint requests: Dr. Raff, University of Louisville, Department of Medicine, Louisville 40201

MELIOIDOSIS COMPLICATED BY PERICARDITIS 227