We describe a child of hyperimmunoglobulin-E syndrome (HIES) with bilateral pneumatoceles for an elective right thoracotomy for pneumatocele excision. HIES is a rare primary immunodeficiency characterized by the triad of markedly elevated serum levels of immunoglobulin E (>2000 IU/mL) with peripheral eosinophilia, recurrent staphylococcal infections of the skin and lungs, and chronic eczema. A 10-year-old male child was presented with recurrent skin abscesses and lung infections, leading to extensive bilateral pneumatocele formation. Such children present unique anesthetic challenges in terms of immunodeficiency, multiple hospital admissions, difficult intravenous (IV) access, and poor pulmonary compliance affecting intraoperative ventilation. Along with pediatric lung isolation techniques, thorough asepsis, good perioperative analgesia and high index of suspicion for pneumothorax are essential components of successful anesthetic management.
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