Childhood Pulmonary Alveolar Proteinosis

7 Petasnick JP . Radi ologic evaluation of aortic dissection. Radiology 1991; 180:297-305 8 Glower DD , Fann JI, Speier RH , Morrison I., White WD, Smith LR, et al. Com parison of m edical and surgical th erap y for un complicat ed descending aortic dissection. Circulation 1990; 82(supp l 4):39-46 9 Masuda Y, Yama da Z, Morooka N, Watanabe S, Inagaki Y. Prognosis of pati ent with medicall y treated aorti c dissection. Circulation 1991; 84(suppl 3):7-13 10 Bayer AJ, Ch ada JS, Farag RR, Path y MSJ. Changing pr esentation of myocard ial infarct ion with increasing old age . J Am Geriatr Soc 1986; 34:263-66 11 Miller PF, Sheps DS, Bragd on EE , Herbst MC, Dalton JL, Hinderli ter AI., et al. Aging and pain perc eption in ischemi c heart disease. Am Heart J 1990; 120:22-30 12 Tu cker MA, Andr ew MF , Ogle SJ, Davison JG. Age associated change in pa in thr eshold measured by transcut aneous neur onal electrical stim ulation. Age Aging 1989; 18:241-46

Childhood Pulmonary Alveolar Proteinosis* Extracorporeal Membrane Oxygenation With Total Cardiopulmonary Support During Bronchopulmonary Lavage Elizabeth M. Hurrion , M.B., Ch.B.; Gale A. Pearson , M.D.; and Richard K. Firmin , M.D .

Partial cardiopulmonary bypass with extracorporeal membrane oxygenation to allow bilateral bronchopulmonary lavage in pulmonary alveolar proteinosis has been described. However, this technique is complicated by a very low arterial POz and cardiovascular embarrassment. Total cardiopulmonary support avoids th ese problems and was successfully used in a 2V2-year-old girl. (Chest 1994; 106:638-40)

C ASE REPORT

A second child of healthy unrelated par ent s was born at 36 weeks' gestatio n weigh ing only 2.2 kg (tenth percentil e). Investigation of failure to thrive required severa l hospital admissions. At 2 yea rs of age , still without a definitive diagnosis, she became tachy pneic with a slight coug h. Th e cough worsened and 2 months later , hospital admission was arranged for incr eased d yspn ea . At th e tim e of hospit al admission, she was noted to be extreme ly thin, weigh ing only 8 kg (we ll below the third percentile). Mark ed pectu s excava tum and clubbing were noted, with cyanosis, tach ypn ea, and marked intercosta l and sub costal recession. Th er e wer e sca ttered fine cre pita tions on auscultation, and saturations with 28 perc ent oxyge n b y fac e mask wer e 80 to 94 perc ent. Results of cardiovascular exam ination were normal. Th e radi ographic a ppea rance was of bilateral reticulonodular shadow ing (Fig 1). Elect roca rdiog ram showed P pulmonale and right ventricular hyp ertroph y. High-resoluti on lung com puted tomography (CT ) revealed exte nsive consolida tion within both lungs, most ma rked posteriorly. Hi stologic exam ina tion of a percuta neous lun g biopsy specime n showed alm ost every alveolar space to be filled with a dense PAS-positiv e granular eosino philic mat er ial , consistent with a di agno sis of PAP. Silver sta ins did not reveal pn eumocystic organ isms and culture of th e biopsy speci men was negative. Suction ca the ter specim ens wer e negati ve for Pneu mocystis imm unofluorescence . Tr eat ment with an tibio tics and steroids was commenced which result ed in a sligh t im provem ent in satura tions. Ent er al nutrition via nasogas tric tube was com me nce d to give 1,340 kcal /d, resulti ng in a slight weight ga in. Despit e these improvem ents it was believed th at her sma ll size and degree of respiratory embarrassmen t would make unil at er al lavage via doubl e-lumen endotrac hea l intubation a high-risk pr ocedure. Th erefore, 6 weeks following hospit al ad m ission, in November 1992 , she was transferred to Groby Road Hospital for bronchopulmonar y lavage on full venoa rte ria l EC MO . Gene ra l anesthesia was induced and the pati ent was intubated and ven tilated . Biomedi cus ca nnulas wer e used to ca nnulate th e right inte rna l jugu lar vein (14F) and right com mon ca rotid art er y (12F). A chest radiograp h confirme d th e ca nnula positions and bypass was established uneventfull y. 14 Ven tilati on was ceased and pul monary lavage was ca rried out with pH-norm alized saline

BAL= bro nchoa lveola r lavage, ECMO =extracorporeal m embra ne oxyge nation; PAP=pulmonary alveo lar proteinosis

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ulmonary alveolar proteinosis (PAP) is a rare condition , particularly so in childre n, and is of unknown etiology .I-8Phospholipid mat eria l accumulates in alveolar spaces, impairing gas exchange and leading to arterial hypoxemia. Th e most effective treatment for PAP is bronchopulmonar y lavage, which at tempts to temporarily clear the alveoli of accumulated material and results in clinical remission, which may be tem porary2-4,7,9 or prolonged. I,5,8,10-13 We report a case of childhood PAP in which lavage was conducted on fu ll venoarteria l cardiopulmonary bypass with extracorporeal membrane oxygenation (ECM O). Imm ediate dr amatic clinical and radiologic improvement followed . To our know ledg e, this technique has not been reported pr eviously and yet offers significant advantagesover techniques currently employed . *F rom the Departmen t of Ped iatric Cardiothoracic Surg er y, Groby Road H ospital, Leicester , United Kingdom.

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FIGURE 1. Radiologic appearance at pres entation, showing bila teral reti cul onodular shadowing. Childhood Pulmonary Alveolar Proteinosis (Human et alj

FIGURE 2. Radiologic appearance following bronchopulmonary lavage showing dramatic improvement. solution until the effl uent fluid, initially pink and creamy, cleared. This required 10 L of saline solution, involved postural drainage and chest percussion, and took 5 h. The maximum ECMO requirement during the lavage procedure was a blood flow rate of 780 ml/min and a sweep rate of gas across the oxygenator of 1,750 L/min . Throughout the entire procedure, the patient was cardiovascularly stable and had excellent mixed venous blood gases. Following the lavage, 40 ml of artificial surfactant was instilled via the endotracheal tube, and ventilation was recommenced, but on minimal settings. The patient was allowed to rest overnight on ECMO to allowany residual fluid to be resorbed and to provide support during any deterioration of lung function. The flow and sweep were weaned as tolerated according to mixed venous oxygen saturations. The following morning, there was a minimal requirement for support. A postlavage chest radiograph showed a dramatic improvement (Fig 2). A trial off ECMO on modest ventilator settings resulted in an excellent arterial blood gas, and decannulation was performed. The vessels were repaired so that should the patient's condition deteriorate in the future, as is to be predicted from the natural history of this condition, further lung lavages on ECMO might be possible. The total time on ECMO was 29 h. The patient was extubated within a few hours of decannulation, and by the following morning was on room air with normal saturations and was transferred back to the referring hospital. Two months later, she was discharged home, still well on room air following a period of hospitalization to allow overnight nasogastric feeding. She was neurologically unimpaired despite carotid artery cannulation. DISCUSSION

In ad ult PAP, br onchopulmonary lavage ma y be carried out using a double-lumen endotracheal tube to allow ventilati on of one lun g during lavage of the other. 8,lO,ll ,13 However , thi s technique is not readily applica ble to children for two reasons. First, if a double-lumen tube is used in a small child, th e lun g is being ventilat ed via a tin y diame ter that ma y be insufficient for ad equate gas exchange, especially wh en respiratory function is Sign ifica ntly compromised. Second, double-lumen endotracheal tubes are not available for very small child re n. Thus bronch op ul-

monary lavage in children ha s been carr ied out by eithe r a modified double-lumen technique using br onchosco py7,9,15 or by massive bil at er al sim ultaneous lavag e using a singl e-lumen tube. 13,16 However, bronchopulmonar y lavage (whethe r in adults or children ) has two pot ential problems. First, sever e hypox emia during the tech nique is common. l ,2,3,5,12,17.20 This is gr eat est during drainage of th e lavage fluid wh en pulmonar y perfusion is restored to th e nonventilated collapsed lung. Also, overspill of lavage fluid into the ventilated lung is an important complication that will wor sen hypoxemia; Thus, th e lavage must be conducted rapidly, a nd the wet lungs ventilated immedi ately afte r th e lavage to avoid neurologic sequela e. Second, card iovascular inst ability is well recognized and is mainly manifest as systemic hypotensi on .i' It ma y be du e to pulmonary hyperten sion wh en the lun g is filled with fluid . Th er e have been a numbe r of rep orts of th e use of partial ca rd iopulmo nary byp ass during lavage.!" In thi s technique, one third of th e card iac output is div erted to an extraco rporeal oxygenator during unilater al or bil at eral lung lavage. But while thi s may over come the technical difficulties in pe rformi ng lun g lavage in small childre n, it does not entirely avoid the above -me ntioned problems of hypoxemia and cardiovascular instability. Th e use of prolonged full ECM O support to tr eat sma ll child ren with PAP does avo id such com plica tions. We are awa re of three other cases (in th e United Stat es) in which thi s techn iqu e has been used successfully (pe rsonal commun ica tion R.H. Bartlett, 1992 ). Th e main ad vantages are norma l arte rial oxyge na tion an d card iovascul ar stability during lavage. Furthermore, th e technique allows prolonged support to avo id imm edi at e revent ilati on of the wet lun gs postlavage. Intrapulmonar y fluid ca n th er efore be allowed to disper se while arteri al oxyge na tion is maint ain ed . Th e clinica l and radi ologic improvem ent seen in thi s pat ient seemed to be mor e dramatic than that rep ort ed in most accounts that see only a mod est improvement ove r 48 h followed by a gra dua l improvem ent during the ensuing weeks.1·3,7,lO,ll Althoug h we ca nnot extr apolat e fr om one case, if thi s technique does allow more thorough lavage, this could be of significant clinical benefit in allowing longer time peri ods bet ween lavages and red uci ng th e propen sity for supe radde d inf ection. If th e vessels are rep air ed following ECMO decannulation, this technique ca n be rep eat ed as the child's clinical condition di ct at es. Childhood PAP was pr eviously th ought of as unif ormly fatal. 2l How ever , spontaneo us remission, recognized for a long tim e in th e adult disease,8,13,22,23 is now bein g reported . 13,24 If disease regr ession is a possibilit y, then improved techniques of bronchopulmonary lava ge ma y allow longer remissions in the meantime. If thi s is the case, ven oarterial ECMO ma y be the safest and most effective option, ulti mat ely offering the gr eatest hop e to these child re n. ACKNOWLEDGMENTS: With thanks to Dr. P. H. Weller, Birmingham Childrens Hospital, for referring the patient, and to Tim Postle, Southampton, for analyzing the lavage fluid. R EFERENCES

Seard C, Wasserman K, Benfield jll , Cleveland RJ, Costley DO, Heimlich EM. Simultaneous bilateral lung lavage (alveolar CHEST 1 106/ 2 1 AUG UST, 1994

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washing}using partial ca rdiopulmona ry bypass. Am Rev Respir Dis 1970; 101:877-84 2 Mazyck EM, Bon ner JT, Herd H M, Symb as I'N. Pulmonary lavage for childhood pu lmonary alveolar protein osis. J Ped iatr 1972; 80:839-42 3 Allose MD, H icks RE, Ed wards MW . Extracorporea l membran e oxygenation during bronchopulmonary lavage. Arch Surg 1976; 1]] :]]48-53 4 Cooper JD , Du ffin J, Glynn MFX, Nelems JM, Teasdale S, Scott AA. Combination of membrane oxygenator support and pulmonary lavage for acut e respira tory failure. J T horac Cardiovase Surg 1976; 71:304-08 5 Fr eedm an AI' , Pelias A, Johnston RF, Goel II', Hakki I-Il, Oslick T, et al. Alveolar prot einosis lung lavage using pa rtial card iopulmonary bypass. T horax 1981; 36:543-45 6 Hiratzka LF , Swan DM, Rose EF , Ahrens Re. Bilat era l simultaneous lung lavage utilizing membran e oxygena tor for pu lmon ar y alveolar pr oteinosis in a 8-month-old infan t. Ann Thorac Surg 1983; 35:313-17 7 Mahut B, de Blic J, Le Bourgeois M, Beringer A, Chevalier JY, Schein mann P. Parti al and massive lung lavages in an infant with severe pulmonary alveolar proteinosis. Pediatr Pulmonol 1992; 13:50-3 8 Prakash UBS, Barham SS, Ca rpe nter H A, Dines DE, Marsh HM. Pulmona ry alveolar ph ospholipopro teinosis: experie nce with 34 cases and a review. Mayo Clin Proc 1987; 62:499-518 9 Moazam F, Schmid t JH , C hesrown SE, Graves SA, Saude r RA, Dru mmond J. Total lung lavage for pu lmonary alveolar prot einosis in an infant without the use of ca rdiopulmonary bypass. J Pediatr Surg 1985; 20:398-401 10 Ramir ez-R] . Alveolar prot einosis: importance of pulm onary lavage. Am Rev Respir Dis 1971; 103:666-78 ]] Roger s RM, Levin DC, Gr ay BA, Moseley L W. Physiologic effects of bronchopulmonar y lavage in alveolar pro teinosis. Am Rev Respir Dis 1978; ]]8:255-64 12 Sund erland W A, Campbell RA, Edwa rds MJ. Pulmonar y alveolar proteinosis and pulmonary cryptococcosis in an adolescent boy. J Ped iatr 1972; 80:450-56 13 Karim an K, Kylstra JA, Spock A. Pulm onar y alveolar prot einosis: prospecti ve clinical experience in 23 patient s for 15 yea rs. L ung 1984; 162:223-31 14 Bartlett RH . Extrac orporeal life sup port for cardiopulmonary failure. Curr Prob Surg 1990; 27:621-705 15 McKen zie B, Wood RE, Bailey A. Airway managem ent for unilatera l lung lavage in child ren. Anesthesiology 1989; 70:550-53 16 Spock A, Kylstra J, Lan ning C, Camporesi EM. Lung lavage in two infan ts with alveolar pro teinosis. Pediat r Res 1983; 17:390A 17 Rogers RM, Szidon j P, Shelburne J, Ne igh JL, Shuman JF, Tantum KIt Hemodynamic response of th e pulmonary circulation to bronc hopulmonary lavage in man. N Engl ] Med 1972; 286:1230-33 18 Wasserman K, Blan k N, Fletcher G. Lun g lavage (alveolar washing ) in alveolar prot einosis. Am J Med 1968; 44:6]]-17 19 Smith JD, Millen JE , Safa r 1', Robin E D. Intrathoracic pressure, pu lmonar y vascular pressures and gas exchange during pul monar y lavage. Anesthesiology 1970; 33:401-05 20 Rami rez RJ. Bronchopul monary lavage-new techni qu es and observations. Dis Chest 1966; 50:581-88 21 Colon AR, Lawrence RD , Mills SD, O 'Connell EJ. Child hood pulmonary alveolar pr otein osis (PAP): report of a case and review of the literatur e. AJDC 1971; 121:481-85 22 Davidson JM, Macleod WM . Pulmonary alveolar prot einosis. Br J Dis Chest 1969; 63:13-28 23 Ram irez RJ. Pulmonary alveolar prot einosis. Arch Intern Med 1967; ]]9:147-56 24 Pau l K, Muller KM, Op permann HC, Nu tzen adel W . Pulmo-

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nar y alveolar lipoproteinosis in a seven-year-old girl. Acta Paediatr Scand 1991; 80:477-81

Bronchogenic Carcinoma in Patients Seropositive for Human Immunodeficiency Virus* Shawn D. Aaron , MD .; Ellen Warn er, M D .; and Jeffrey D. Edelson, M D ., F.C.C.P.

Th e purpose of this report is to describe an as sociati on between bronchogenic carcinoma and HIV. Three HIVseropositive patients are described who developed bronchogenic can ce r (two large cell, one ad enocarcinoma) before developing an AIDS-defining illn ess. A critical review of th e literature revealed 22 other patients in which th e association of HIV infection and lung cancer is reported. Th ese patients are charact erized by a relati vel y young age at di agn osis (median, 43 years) and pr evalence of th e ade nocarc inom a subtype (13 of 25 patients). Twenty of 21 patients h ad a hi story of smoking. Among 21 patients for whom data wer e available, 6 patients (28 percent) had AIDS at time of diagnosi s of lung cancer while 11 patients (55 percent) did not have AIDS or AIDS-related com plex at diagnosis. (Che st 1994; 106:640-42)

I AZT =zidov udi ne I

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atients inf ect ed with H IV frequently develop pulmonar y com plications.l -S Althoug h lung ab normalities in AIDS usually result fr om pu lmonary inf ect ion, ma lignan cies such as Kaposi's sarcom a and non-Hodgkin 's lymph om a occur with markedl y increase d frequ en cy in AIDS patients.i'-" Lun g ca ncer, however, has not classically been associa ted with HI V infecti on. Th is repo rt det ails three cases of HI V-infected pa tients who developed bronchoge nic ca rcinoma. O ther published repor ts are reviewed , sugges ting a causal association be tween HI V infection and lung can cer. CASE R EPOR TS C ASE

1

A 50-year -old HI V-positive, bisexual man presente d in Novem ber 1991 with a 3-m on th history of an inter mittent rightsided chest and shoulder pain. He had a 30-pa ck-yea r smo king history and had been taking zido vudi ne (AZT) for 4 years. He had a CD4 count of 240 in Oct ober 1991. Th er e was no history of lym ph adenopat hy or AIDS-defining illness. Th e patien t reported a 4.5-kg weigh t loss over the previous 8 mon ths but had no fever , cough, d yspnea, or hem optysis. Ph ysical exam ination revealed *F rom the Dep artm ent of Medi cin e, St. Michael's Hospit al, University of Toro nto, Ont ari o, Ca nada . Re print requests: Dr. Edelson , SI. Michael's Hospital, 30 Bond Street , Toront o, Onta rio, Canada M5B l W 8 Bronchogenic Carcinoma in HIV+ Patients (Aaron , Warner, Edelson)