during the following 24 to 48 b with total clearing of endotracheal tube blood in three days. On the fifth da~ extubation was accomplished. The patient was discharged on a regimen of prednisone, 1.5 mw1cg daily, and orally administered cyclophosphamide, 2.5 mWkg three times weekly. Cyclophosphamide therapy was continued for 14 months and altemate-day dosage of prednisone was given for 26 months. Chemoprophylaxis with cotrimoxazole was used during the prednisone therapy. He also remained on a milkfree diet. The disease has remained in remission without further medications for approximately five years. DISCUSSION

The etiology ofprimary pulmonary hemosiderosis remains unknown. An immunologic basis is supported by observations of improvement in some patients receiving immunosuppressive therapy. Improvement in acute symptomatology follOwing administration of corticosteroids was reported by Browning and Houghton in 1956, j although an epidemiologic survey of 30 children with this disease concluded that corticosteroid therapy did not alter the long-term course or prognosis. 3 Other reports have suggested that addition of azathioprine appeared to produce remission after corticosteroids failed to do so. ".5 To our knowledge, the only reported cases of primary pulmonary hemosiderosis treated with cyclophosphamide are found in the European literature. Apparent cyclophosphamide-induced remission ofprimary pulmonary hemosiderosis was reported in a 4-year-old girl6 and a 13-year-old boy7 after corticosteroid therapy failed. Others have reported improvement of acute symptoms with a combination ofprednisone and cyclophosphamide the~ 8-10 O'Donohue reported a patient with the original diagnosis of primary pulmonary hemosiderosis, but with many features suggestive of Wegener's granulomatosis, showing remission on two occasions with cyclophosphamide thera~ 11 As documented by clinical status and P(A-a)Oj values, our patient worsened during azathioprine and corticosteroid therapy but promptly improved after a single dose of cyclophosphamide. This scenario occurred two additional times with the patient's condition changed from one ofsevere ventilatory failure requiring 100 percent oxygen and high ventilator pres.sures to rapid discontinuation of mechanical ventilation after resumption of cyclophosphamide therap~ Improvement was noted on all three occasions within 24 to 48 h ofcyclophosphamide treatment and remission has been maintained for nearly five years. While primary pulmonary hemosiderosis has a remitting nature, the timing of clinical improvement in this case strongly suggests a beneficial response to cyclophosphamide. The finding of autoimmune hemolysis associated with primary pulmonary hemosiderosis has been reported. II This patients hemoglobinuria rapidly improved and was attributed to this etiology. Cyclophosphamide possesses potent antiin8ammatory and immunosuppressive actions. It inhibits both humoral and cell-mediated reactions through unknown mechanisms. Possible side effects include leukopenia, thrombocytopenia, hemorrhagic cystitis, interstitial pulmonary fibrosis, sterility and secondary malignan~ We propose that this child's response to cyclophosphamide was due to the drug's antiinflammatory and immunosuppressive properties. Its use appeared to be life-saving in this 980

patient and without adverse effects, although the bearing on the long-term outcome is less clear. ~ report this case for its potential value to English-speaking physicians. Due to the frequency and severity of side effects associated with cyclophosphamide, we believe that its use should be limited to life-threatening exacerbations of primary pulmonary hemosiderosis which have not responded to traditional therap~

ACKNOWLEDGMENTS: The authors thank TImothy Hallberg, M.S., for graphics assistance and Mrs. Mary Bell and Mrs. Ruth Traub for secretarial assistance. REFERENCES

1 Heiner D. Primary pulmonary hemosiderosis with sensitivity to cow milk. In Chemic ~ Kendig EL eds. Disorders of the respiratory tract in children. Philadelphia: WB Saunders Co, 1990:498-506 2 Browning JR, Houghton JD. Idiopathic pulmonary hemosiderosis. Am J Med 1956; 20:374-82 3 Chryssanthopoulos C, Cassimos C, Panagiotidou C. Prognostic criteria in idiopathic pulmonary hemosiderosis in children. Eur J Pediatr 1983; 140(2):123-25 4 Steiner B, Nabrady J. Immunoallergic lung purpura treated with azathioprine. Lancet 1965; 1:140-41 5 Yeager H, Powell D, Weinberg R. Idiopathic pulmonary hemosiderosis: ultrastructural studies and response to azathioprine. Arch Intern Med 1976; 136:1145-49 6 Carnelli ~ Biraghi ~ Zurlo MG, Rossi MR, Parziani ~ Efficacy of treatment with cyclophosphamide in a case of idiopathic pulmonary hemosiderosis. Minerva Pediatr 1979; 31:3893-3900 7 Garatti M, Parravicini C, Meroni PL, Giulotto ~ Rusconi R, Carnelli ~ Idiopathic pulmonary hemosiderosis: report of a case with a favorable response to cyclophosphamide the~ Pediatr Med Coo 1983; 5:95-98 8 Lapena S, Blanco A, Solis ~ Linares ~ Gomez S, Hemanz-Sanz JL. Risk factors in the outcome of idiopathic pulmonary hemosiderosis. An Esp Pediatr 1985; 23:5-11 9 Bagnato L, Grilli C, Portioli ~ Biella C, Carnelli ~ Long-term evaluation ofimmunosuppressive therapy in pediatric pulmonary hemosiderosis. Pediatr Med Chir 1986; 8:671-74 10 Einhorn S, Sulcenik S, Einhorn M, Keynan A. The value of the combination cyclophosphamide-prednisone in the treatment of idiopathic pulmonary hemosiderosis. Ann Med Intern 1987; 138:144-45 11 O'Donohue WJ. Idiopathic pulmonary hemosiderosis with manifestations of multiple connective tissue and immune disorders. Am Rev Respir Dis 1974; 109:473-79 12 Rafferty JR, Cook MIC. Idiopathic pulmonary haemosiderosis with autoimmune haemolytic anaemia. Br J Dis Chest 1984; 78:282-85

Implantable cardloverter Defibrillator Infection causing Constrictive Pericarditis· Arnold H. Kauanoffi M.D.; Charles B. Lemn, M.D.; Christopher R. C. Wyndham, M.D.; and 1Awr8nce J Mills, M.D. *From the Department of Medicine, Presbyterian Hospital of

Dallas. Repnnt requests: Dr. Kaaaanoffi 7150 Greenville, Dolltu 75231

Implantable C8rdIcMt1er DefibriIator Infection (Kasunolf et aI)

A case of severe constrictive pericarditis resulting from an iodoIent plftldomorttJa caeruginotG inCectioo or the automatic internal eardiac de6brilLdor is cIescribed. Total esplanatioa the device was attempted after nine months but was unsuccessful because of dense adbesioas under the piteb electrodes. The patient subsequently developed clinical and hemodynamic 6ndings of constrictive pericarditis and a second desperate attempt to remove the patches resulted in operative death. Diagnostic modalities Cor detecting inCection the AICD are reviewed. As soon as there is inCection imoIving any component, the entire lead system and pulse generator sbould be removed. (Chat 1992; 102:960-63)

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A1CD automalic impIaDtabie cardiac de8brillator

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I

he automatic implantable cardiac defibriUator has shown remarbble efficacy in decreasing the incidence of sudden cardiac death in patients with drug-resistant malignant ventricular arrhythmias. Major complications have been relatively uncommon but include mechanical and sensing problems, generator erosion through the overlying skin, the short life of the current units, and sepsis. Infection, as a complication usually involves the subcutaneous generator pocket, but has the potential of spreading to the mediastinum and patch electrodes. A single case ofconstrictive pericarditis has been reported, 1 but occurred as a result of a noninfected foreign body reaction which produced dense adhesions and crumpling of the defibrillating patches and surrounding pericardium. The following report describes severe constrictive pericarditis resulting from an indolent prolonged Pseud0rn0n08 aerugioosa infection. CASE REPORT

A 69-yeaN)!d man, with an inferior myocardial infarction 16 years before. suffered canliac arrest with documented ventricular fibrillation. He was successfully defibrillated. There was no evidence of acute myocardial infarction. Cardiac catheterization subsequently revealed an ejection fraction of 50 percent with inferior akinesis, total occlusion of the right coronary artery, and a 30 to 40 percent mid left anterior descending lesion. An electrophysiology study revealed long runs of inducible ventricular tachycardia. An AlCD was implanted using two large flexibletitanium mesh silicone rubber patch electrodes on the posteriorlateral left venbicle and the antero-basal right venbicle. The generator was positioned in the left posterior rectus sheath below the left costal margin. Two weelcs later, a subcostal wound infection appeared. PJeudo. monas aerugtflOllJ was cultured. and ceftazidime and tobramycin were administered and drains inserted in the generator pocket. An indium scan showed no uptake in the incision or pericardial space. A prolonged coone of ciprofloxacin was given. Nine months later, be developed a draining sinus of the wound. Pseudomonas was again cultured, a new subcutaneous pocket was created, and a six-week course of ceftazidime was given. Nevertheless, subcutaneous infection appeared in the second subcutaneous pocket and because of this, an attempt was made to remove the entire A1CD device. The generator was removed through a subcostal incision, but an attempt to remove the patches via a left thoracotomy was unsuccessful because of adhesions under the patches. A twoweek coone of aztreonam was given, and amiodarone was begun. Five weelcs later. the patient presented with a one-week history of increasing dyspnea on exertion. increasing abdominal girth, and peripheral edema. He had been afebrile. On examination, he

FICURE 1. Preoperative roentgenogram showing normal heart size, a large right pleural effusion, and marked crumpling of the patch electrodes. showed marked jugular venous distension, ascites, tender hepatomegaly with a hepatojugular reflux, and diminished breath sounds at the left lung base. Chest x-ray 111m revealed interstitial left lower lobe pneumonia. Gallium and lung scans were negative, and there was no Significant change in oxygen diffusion. An ecbocardiogram was normal. but poor visualization of the apex was noted. The MUGA scan showed posterior and septal wall motion abnormality CHEST I 102 I 3 I SEPTEMBER. 1992

981

FICURE 2. Thick, densely 8brotic pericardial sac measuring 10 to 12 mm, adhering to both ventricles. with an ejection fraction of 45 percent, but no evidence of right ventricular malfunction. A stress tbalIium test disclosed reversible septal and inferoposterior perfusion defects. The patient was given furosemide fur diuresis, and discharged. Six weeks later, the patient was again readmitted in severe right heart failure. The blood pressure was 12CV70 mm Hg without pulsus paradoxus and the patient was again afebrile. Marked jugular venous distention, hepatojugular re8ux, decreased breath sounds with egophony at the right base, and a tender liver were noted. The chest x-ray 81m (Fig 1) showed diminution of the lower left lobe in81trate, but a right pleural effusion was noted. Distortion of the epicardial patches was apparent. Thoracentesis removed 1,600 ml of straw-colored 8uid which subsequently cultured Pseudomonas. The patient was given a diuretic. The possibility of patch-related oonstrictive pericarditis was raised, and a cardiac catheterization was perfurmed. The cardiac output was 3.5 Umin, with equalization of the diastolic pressures after volume expansion with llespan, producing a "dip and plateau" pattern in the right ventricular tracing. The ventriculogram showed marked deformity of the two patches, extension of the heart shadow beyond the epicardium, and markedly reduced apical 6I\ing and deformity. There was 100 percent occlusion of the proximal right ooronary artery and 100 percent occlusion of the distal left anterior descending coronary artery. Pericardiectomy with patch removal was attempted on cardiopulmonary bypass. The patches were surrounded by gross pus and dense adhesions, but were removed with difficulty. Myocardial function, however, did not return, and the patient died. At postmortem examination, the heart weighed 580 g. The remaining pericardium (Fig 2) measured 10 to 12 mm in thiclmess and was densely 8brotic, covering the surface of both ventricles. Microscopic sections revealed chronic in8ammation of the thick pericardium with focal necrosis, surrounded by granulation tissue, lymphocytes, and plasma cells. The in8ammatory reaction beneath the epicardia1 surface was minimal. An old posterior myocardial infarction was seen. There was bilateral lower lobe pneumonia. Pseudomonas was cultured from the patch specimens. DISCUSSION

The pathologic consequences of the AlCD have been described in relatively few patients. Singer et aJs examined 25 autopsy cases. Acute pericarditis occurred in all patients with resultant pericardial adhesions and progressive localized fibrosis. The frequency of defibrillations resulted in contraction band necrosis or vacuolar cytoplasmic clearing and loss of monocytes, but the observed changes were

confined to the tissue under the patch electrodes. Constrictive pericarditis was not seen. The case of reactive constrictive pericarditis with distortion of the patches described by Almassi et all is the only case of its kind we could find in the literature. Constrictive pericarditis was discovered 15 months postoperatively. There was no associated infection. Histologic findings re. vealed fibrous tissue with multinucleated giant cells, and the patient recovered after pericardial stripping. Our case illustrates an unsuccessful attempt to manage infection without early removal of the entire AlCD device. Because there was nothing to suggest mediastinal extension of the infection (negative gallium and indium scans), it was decided to treat the infection with antibiotics, changing of the power generator, and relocating the site of implantation. This attempt is not without precedent. Taylor et aJ3 successfully treated a Staphylococcus QUf'ftU and Serratia marcescens infected generator pocket with debridement followed by pocket irrigation with antibiotics, parenteral antibiotics, and long-term oral antibiotics. The authors pointed out that infected aortic grafts and prosthetic valves have been removed from an infected bed and then replaced. Likewise, infected cardiac pacemakers have been treated without removal of the devices.• Wunderly et ala commented on eight patients who developed wound infections. Infections treated with antibiotics alone, or pocket explantation failed to heal in four out of five patients. Total removal of the entire device, however, was uniformly successful. They concluded, as have others, that when there is infection within the generator pocket, the entire lead system, pads and generator should be explanted. Furman,· in an editorial comment, stressed that "a seemingly 'small' or inconsequential hardware infection should not be treated as such; it should be treated as the beginning of the end of that implant in that patient." Early recognition ofinfection is important since continued tissue ingrowth into the screen-like architecture ofthe patch electrodes makes removal increasingly difficult without causing epicardial damage. 3 In our patient, after the initial attempt at electrode patch removal was unsuccessful, there remained a matrix for continued infection and eventual constrictive pericarditis. Goodman et al' reported seven patients with AlCD infections. As bas been reported by others, the clinical presentation is frequently indolent rather than septic in nature. The pericardial infection was localized to the area underneath or adjacent to the patch resulting in distortion of the patches. In the group with pericardial infection (five patients), fluid was found beneath all ten patches at surgery, and in eight patients, there was frank pus. The authors concluded that a distorted or crumpled patch on a roentgenogram associated with erythema or pain around the generator pocket strongly suggests pericardial infection, and that a CT scan is accurate in recognizing fluid adjacent to the patch. This case report represents the first report ofconstrictive pericarditis as a complication of patch infection, and again stresses the need to explant infected or potentially infected patches at the first sign of device infection. Delay makes patch removal more difficult, and serious consequences may follow.

Table l-Clulnga in Median Frequency.

ACKNOWLEDGMENT: Or. Susan Miner provided excellent pathological assistance and advice, and Cathy Harrell gave secretarial assistance.

Inspiration

REFERENCES

1 Almassi GH, Chapman PO, Troup PC, Wetherbee IN, Olinger GN. Constrictive pericarditis associated with patch electrodes of the automatic implantable cardioverteNiefibrillator. Chest 1987; 92:369-71 2 Singer I, Hutchins GM, Mirowski M, Mower MM, Veltri E~ Guarnieri ~ Pathologic findings related to the lead system and repeated defibrillations in patients with the automatic implantable cardioverteNlefibrillator. }ACC 1987; 10:382-88 3 18ylor RL, Cohen OJ, Widman LE, Chilton RJ, 01\ourke RA. Infection of an implantable cardioverter defibrillator: management without removal of the device in selected cases. PACE 1990; 13:1352-63 4 Furman ~ Hiller AJ, Playforth RH, Bryant LR, Trinkle JK. Infected pennanent cardiac pacemaker, management without removal. Ann Thorac Surg 1972; 14:54-58 5 Wunderly 0, Maloney J, Edel T, McHenry M, McCarthy PM. Infections in implantable cardioverter-defibrillator patients. PACE 1990; 13:1360-64 6 Furman S. From the editor: Implantable cardioverter de6brillator infection. PACE 1990; 13:1351 7 Goodman LR, Almassi GH, Troup PJ, Gurney JW: Veseth-Rogers J, Chapman PO. Complications of automatic implantable cardioverter defibrillators: radiographic, Cl: and echocardiographic evaluation. Radiology 1989; 170:447-52

Tracheal Sounds in Upper Airway Obstructlon* Hans ItJsterkamp, M.D.; and Ignacio Sanchez, M.D.t A boy with subglottic narrowing secondary to laryngotracheitis presented with noisy breathing. Acoustic measurements of tracheal sounds at standardized air 80ws correlated weD with the clinical course and with spirometric assessments. This indicates the potential value of respiratory sound characterization in patients with upper airway obstruction. (Chat 1992; 102:963-65)

A. cute upper airway obstruction commonly presents with .t1 abnormal respiratory sounds. Inspiratory stridor is a

well recognized clinical sign, but stenosis of the upper airways may also lead to greater respiratory noise that lacks the musical quality of stridor. We report measurements of tracheal sounds in a boy with noisy breathing because of acute laryngotracheitis and subglottic stenosis. We .found the sound spectral characteristics at given airflows to correlate well with changes in maximum inspiratory and expiratory Bows during the course of the illness. Objective characterization of tracheal sounds may provide a noninvasive and effort-independent method to assess therapeutic effects in upper airway obstruction. CASE REPORT

This 81h-year-old boy who was previously weD had acute onset of *From the Department of Pediabics and Child Health, Section of Re~irol~ University of Manitoba, Winnipeg, Canada. t Fell