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3. Mumtaz MA, Schwartz RH, Grundfast KM, Baumgartner RC. Tuberculosis of the middle ear and mastoid. Pediatr Infect Dis 1983;2:234-6. 4. MacAdam AM, Rubio T. Tuberculous otomastoiditis in children. Am J Dis Child 1977;131:52-6. 5. Jacobs JC, Li SC, Ruzal-Shapiro C, Kiernan H, Parisien M, Shapiro A. Tuberculous arthritis in children. Clin Pediatr 1994;33:344-8. 6. Garrido G, Gomez-Reino JJ, Fernandez-Dapica P, Palenqu EL, Priento S. A review of peripheral tuberculous arthritis. Semin Arthritis Rheum 1988;18:142-9. 7. Davidson PT, Horowitz I. Skeletal tuberculosis. Am J Med 1970;48:77-84. 8. Wolfgang GL. Tuberculosis joint infection. Clin Orthop 1978; 136:257-63. 9. Berney S, Goldstein M, Bishko F. Clinical and diagnostic features of tuberculous arthritis. Am J Med 1972;53:36-42. 10. Haygood TM, Williamson SL. Radiographic findings of extremity tuberculosis in childhood: back to the future? Radiographics 1994;14:561-70. 11. Ellis ME, E1-Ramahi KM, A1-Dalann AN. Tuberculosis of peripheral joints: a dilemma in diagnosis. Tuber Lung Dis 1993; 74:399-404.

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12. Rasool MN, Govender S, Naidoo KS. Cystic tuberculosis of bone in children. J Bone Joint Surg (Br) 1994;76:113-7. 13. Papavasiliou VA, Petropoulos AV. Bone and joint tuberculosis in childhood. Acta Orthop Scand 1981;52:1-4. 14. Starke JR, Correa AG. Management of mycobacterial infection and disease in children. Pediatr Infect Dis J 1995; 14:55-70. 15. Ad Hoc Committee of the Scientific Assembly on Microbiology, Tuberculosis, and Pulmonary Infections. Treatment of tuberculosis and tuberculosis infection in adults and children. Clin Infect Dis 1995;21:9-27. 16. Starke JR, Jacobs RF, Jereb J. Resurgence of tuberculosis in children. J Pediatr 1992; 120:839-55. 17. Weis SE, Slocum PC., Blais FX, et al. The effect of directly observed therapy on the rates of drug resistance and relapse in tuberculosis. N Engl J Med 1994;330:1179-84. 18. Jeang MK, Fletcher EC. Tuberculous otitis media. JAMA 1983 ;249:2231-2. 19. Skolnik PR, Nadol J, Baker AS. Tuberculosis of the middle ear: review of the literature with an instructive case report. Rev Infect Dis 1986;8:403-10. 20. Windle-Taylor P, Bailey CM. Tuberculous otitis media: a series of 22 patients. Laryngoscope 1980;90:1039-44.

Complications associated with severe invasive streptococcal syndrome Vicki L. M o n t g o m e r y , MD, a n d Denise Bratcher, DO From the Depaffment of Pediatrics, Division of Pediatric Critical Care Medicine, and the Department of Pediatrics, Division of Pediatric Infectious Diseases, University of Louisville, Louisville, Kentucky

Group A l~-hemolytic streptococcal sepsis may cause life-threatening disease. We describe a child with severe invasive streptococcal syndrome in whom severe respiratory failure and pulmonary pneumatoceles required extracorporeal life support. Physicians should be aware of the full spectrum of pathologic changes and life-threatening complications caused by group A l~-streptococcus. (J Pediatr 1996; 129:602-4) CASE

REPORT

A 21-month-old girl came to the emergency department with fever, tachypnea, grunting, intercostal retractions, and lethargy. She had decreased oral intake and periods of unresponsiveness. She was admitted to the pediatric intensive care unit. Vital signs on admission revealed a temperature of 39 ° C, heart rate of 180 beats]rain, respiratory rate of 70 breaths/rain, and blood pressure of 99/50 mm Hg. Nasal flaring, grunting, and intercostal retractions were present. No breath sounds were audible in the left hemithorax. Coarse rhonchi were auscultated in the fight side of the Submitted for publication Feb 21, 1996; accepted May 24, 1996. Reprint requests: Vicki L. Montgomery, MD, Department of Pediattics, University of Louisville, Louisville, KY 40292. Copyright © 1996 by Mosby-Year Book, Inc. 0022-3476/96/$5.00 + 0 9/22/75341

chest. The remainder of the examination findings were normal. Laboratory data on admission included a leukocyte count of 0.6 x 109 cells/L, with 10% polymorphonuclear leukocytes, 40% band forms, and 30% lymphocytes, and a platelet count of 84 x 109 cells/L. A chest roentgenogram revealed a large left pleural effusion with left lower lobe and lingula consolidation. ECLS GABHS PICU

Extracorporeal life support Group A [3-hemolytic streptococcus Pediatric intensive care unit

Needle thoracentesis obtained 300 ml of cloudy fluid, which had a leukocyte count of 3.8 x 1 0 9 cells/L, pH of 7.29, glucose concentration of 1.7 mmol/L (30 mg/dl), and protein concentration of 39 gm/L (3.9 gm/dl). A tube thoracostomy was performed to evacuate the effusion further.

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The patient's respiratory function deteriorated during the next 24 hours, and she required orotracheal intubation and mechanical ventilation. Pepeated examination revealed hepatosplenomegaly; adenopathy; warmth, edema, and erythema of both knees; and a red, sandpaper-like rash over the trunk and extremities. A chest roentgenogram showed progression of the infiltrates. Cultures of blood, throat, and pleural fluid grew group A 13-hemolyticstreptococcus (exotoxin A and B producing), which was susceptible to penicillin. Although repeated blood cultures showed negative results, a repeated throat culture on PICU day 4 grew GABHS. The patient initially received intravenously administered penicillin and then c,ompleted 14 days of intravenous clindamycin therapy, The patient had a pneumomediastinum and pneumothorax on PICU day 3. During PICU days 3 to 4, two doses of surfactant were instilled through the endotracheal tube into the trachea. Although transient improvement occurred, respiratory function continued to deteriorate. On PICU day 5, the arterial oxygen tension/fraction of inspired oxygen ratio decreased to 61, and the pH to 7.13 with an arterial carbon dioxide tension of 13.7 pKa (103 mm Hg). The patient was placed on venoarterial extracorporeal life support to treat worsening air leak syndrome, respiratory failure, and cardiovascular instabifity. Blood pressure, oxygenation, and ventilation immediately improved. On PICU day 7, bilateral pneurnatoceles were visible on a roentgenogr~an of the chest. Decannulation was achieved on ECLS day 20 (PICU day 25) and extubation on PICU day 34. Computed tomography of the chest revealed several pneumatoceles but no evidence of abscess formation. The patient was transferred to a general medicine ward on PICU day 36 and discharged from the hospital on day 42. One month after discharge the patient had an acute onset of shortness of breath and tachypnea. No acute respiratory illness or cough preceded these symptoms. A chest x-ray film revealed a left pneumothorax, presumably caused by rupture of a pneumatocele. The patient underwent resection of the left lingula. Histopathologic study of the resected lung tissue showed marked pleural fibrosis, mild ctu-onie inflammation, and scattered loci of reactive alveolar pneumatocytes.

DISCUSSION GABHS sepsis, though fairly uncommon, may become fulminant, often resulting in death or significant morbidityJ ,2 Severe invasive streptococcal syndrome caused by GABHS may occur in otherwise healthy children. 2 Incidence rates for GABHS sepsis in adults and children range from 0.5 to 2%. 2 Mortality rates range from 7% to 35%, despite early administration of appropriate antibiotic therapy. 2 Strains of GABHS associated with severe invasive streptococcal disease are found to produce streptococcal pyrogenic toxins A and B more often than do strains isolated from patients with uncomplicated pharyngitis.2 Streptococcal toxin A stimulates in vitro production of tumor necrosis factor alpha, which may adversely affect host defenses. 3, 4 Our patient's isolate was confirmed to produce streptococcal pyrogenic exotoxins A and B by the World Health Organiza-

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tion Collaborating Center for Reference and Research on Streptococci at the University of Minnesota. 2 This child had severe respiratory failure, barotrauma, and shock. After less invasive management failed, she was placed on ECLS. This therapy is recognized by many as lifesaving for children with severe respiratory failure.5 Recent pediatric data suggest a 60% to 80% survival rate when ECLS is used for the treatment of severe respiratory failure.5 Pneumatoceles as a complication of pneumonia in infants and children are uncommon. In a review of 356 children hospitalized for pneumonia, Victoria et al.6 reported that pneumatoceles developed in 2.8%. The affected children ranged in age from 4 months to 10 years and had pneumatoceles within 15 days of admission (mean, 6.8 days). Amitai et al.7 reviewed 493 cases of pneumonia in hospitalized children and found pneumatoceles in 2.4%. In our patient, pneumatoceles were first observed on PICU day 7, which is consistent with previous reports. 6-8 The mechanism for pneumatocele formation is thought to involve a combination of parenchymal necrosis and check-valve bronchiolar obstruction, which suggests that alveolar or bronchiolar disease is probably the inciting event. 9 Most pneumatoceles in previously healthy children resolve without complications within 1 year of diagnosis.6, 8, 10 In the reviews by Victoria et al.6 and Amitai et al.,7 no child had recurrent pneumonia, abscess formation, or rupture of a pneumatocele. Although spontaneous rupture of a pneumatocele has: been described in an adult and during the acute illness, 11 spontaneous rupture in a child after recovery from the primary illness has not been reported. Our patient had spontaneous rupture of a pneumatocele, necessitating resection of the left lingula. Before the early 1970s, pneumatoceles were thought of as pathognomonic for Staphylococcus aureus pneumonia. 1° Subsequently, other infectious and noninfectious causes of acute lung injury were associated with the development of pneumatoceles.6, 7,10 Our case is the first report of postpneumonic pneumatoceles caused by Streptococcus pyogenes since 1961 22 As the frequency of invasive GABHS disease with lung abnormalities increases, this Compfication may be more frequently observed. Our report describes a nearly fatal case of severe invasive streptococcal syndrome that was further complicated by the development of pulmonary pneumatoceles and treatment with ECLS. ECLS should be considered for children with severe respiratory failure complicated by barotrauma and shock when less invasive treatment fails. Although most children in whom pulmonary pneumatoceles develop as a result of an acute lung injury will have an uneventful recovery, spontaneous rupture may occur at a time remote from the acute illness. Physicians and parents caring for a child with pneumatoceles should be aware of this rare, potentially life-threatening complication.

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We thank Edward I. Kaplan, MD, Patrick M. Schlievert, Phi), and the World Health Organization Collaborating Center for Reference and Research on Streptococci for assistance in determination of toxin production.

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5.

6. REFERENCES

1. Stevens DL, Tanner MH, Winship J, et al. Severe group A streptococcal infections associated with a toxic shock-like syndrome and scarlet fever toxin A. N Engl J Med 1989;321:1-7. 2. Belani K, Schlievert PM, Kaplan EL, Ferrieri P. Association of exotoxin-producing group A streptococci and severe disease in children. Pediatr Infect Dis J 1991;10:351-4. 3. Fast DJ, Schlievert PM, Nelson RD. Toxic shock syndromeassociated staphylococcal and streptococcal pyrogenic toxins are potent inducers of tumor necrosis factor production. Infect Immun 1989;57:291-4. 4. Fast DJ, Schlievert PM, Nelson RD. Nonpurulent response to toxic shock syndrome toxin 1-producing Staphylococcus au-

7. 8. 9. 10.

11. 12.

reus: relationship to toxin-stimulated production of tumor necrosis factor. J Immunol 1988;140:949-53. Moler FS, Custer JR, Bartlett RH, et al. Extracorporeal life support for severe pedialfic respiratory failure: an updated experience 1991-1993. J Pediatr 1994;124:875-80. Victoria MS, Steiner MD, Rao MD. Persistent postpneumonic pneumatoceles in children. Chest 1981;79:359-61. Amital I, Mogle P, Godfrey S, Aviad I. Pneumatocele in infants and children. Clin Pediatr 1983;22:420-2. Dines DE. Diagnostic significance of pneumatocele of the lung. JAMA 1968;204:1169-72. Qnigley MJ, Fraser RS. Pulmonary pneumatocele: pathology and pathogenesis. AJR Am J Roentgenol 1988;150:1275-7. Asmar BI, Thirumoo~ai MC, Dajani AS. Pneumococcal pneumonia with pneumatocele formation. Am J Dis Child 1978;132:1091-3. McGarry T, Rohman M, Huang CT. Pneumatocele formation in adult pneumonia. Chest 1987;92:717-20. Kevy VK, Lowe BA. Streptococcal pneumonia and empyema in childhood. N Engl J Med 1961;264:738-43.

Methotrexate treatment of Wegener granulomatosis in children Beth S. Gottlieb, MD, Laurie C, Miller, MD, a n d Norman T, Ilowite, MD From the Divisions of Pediatric Rheumatology, Departments of Pediatrics, Schneider Children's Hospital, Long Island Jewish Medical Center, Long Island Campus of the Albert Einstein College of Medicine, New Hyde Park, New York, and the Floating Hospital for Children at New England Medical Center, Tufts University School of Medicine, Boston, Massachusetts

Wegener granulomatosis traditionally has been treated with glucocorticoids and cyclophosphamide. Both the disease and its treatments are associated with significant morbidity and mortality rates. There has been an effort to find effective but less toxic alternative treatments. We describe three children with Wegener granulomatosis who responded well to treatment with glucocorticoids and methotrexate, similar to a regimen used in adults. (J Pediatr 1996;129:604-7) Wegener granulomatosis is a rare form of necrotizing vasculitis that affects mainly the small arteries and veins. The classic triad of W G is upper and lower respiratory tract and kidney involvement. W G is especially uncommon in the pediatric population.2, 2 The largest series of pediatric W G described 23 children with the disease. 3 This clinical syndrome Submitted for publication Jan. 11, 1996; accepted June 6, 1996. Reprint requests: Norman T. Ilowite, MD, Chief, Division of Pediattic Rheumatology, Schneider Children's Hospital, Long Island Jewish Medical Center, Long Island Campus of the Albert Einstein College of Medicine, New Hyde Park, NY 11042. Copyright © 1996 by Mosby-Year Book, Inc: 0022-3476/96/$5.00 + 0 9/22/75546

is sometimes limited to the upper respiratory tract, including nasal and sinus involvement, in which case it is referred to as localized WG. 4 In other cases it may be widespread, afANCA C-ANCA P-ANCA TMP-SMX WG

Anti-neutrophilic cytoplasmic antibodies Cytoplasmic anti-neutrophilic cytoplasmic antibodies Perinuclear anti-neutrophilic cytoplasmic antibodies Trimethoprim-sulfamethoxazole Wegener granulomatosis

fecting the skin, central nervous system, heart, lungs (manifesting as pulmonary infiltrates, nodules, pleuritis, or he-