Pediatric emergency medicine trisk 698

FIGURE 99.3 Chest radiograph showing pneumothorax If pneumothoraces are recurrent or persistent, the patient should be evaluated for pleurodesis While this may prevent recurrence, such intervention may have implications for lung transplant eligibility and should be discussed with physicians having experience in either CF or lung transplantation Daily CF therapies, such as chest percussion and postural drainage, oscillatory percussive vest therapy, other airway clearance techniques (e.g., positive expiratory pressure [PEP] mask, flutter valve), inhalation of dornase alfa (pulmozyme), and pulmonary function testing, should be suspended temporarily to avoid exacerbating the pneumothorax Inhalational therapy with bronchodilators and/or ICSs may be continued with nebulization, but the usual inhalational maneuvers with MDIs probably should be avoided until the pneumothorax is resolved Timing for reimplementation of therapies is based on resolution of pneumothorax and discussion with the patient’s CF team Hemoptysis Blood streaking of the sputum is common in CF patients The CFF’s Guidelines define hemoptysis as mild (less than 60 cm3 daily), moderate (more than 60 and less than 240 cm3 daily), and severe (more than 240 cm3 daily or more than 100 cm3 per day for more than days) Hemoptysis should be distinguished from epistaxis or hematemesis Mild Hemoptysis Mild hemoptysis requires no specific treatment other than observation Persistent streaking may indicate a pulmonary exacerbation requiring antibiotic treatment Other factors such as chronic use of medications with antiplatelet function activity (e.g., aspirin) or coagulopathy secondary to decreased vitamin K levels should be ruled out and treated accordingly Moderate/Severe Hemoptysis Severe episodes can be life threatening due to asphyxiation from airway obstruction, hemorrhagic shock, and/or chemical pneumonitis Approximately 1% of CF patients experience an episode of major bleeding per year, the majority of patients being 16 years or older The bleeding usually originates from enlarged and tortuous bronchial arteries, two-thirds of which arise from the ventral surface of the aorta The remaining third come from the internal mammary and intercostal arteries Onset is often abrupt Some patients may report localized gurgling or sensation in the specific area of lung involved Physical examination may reveal new, localized pulmonary findings Placing a nasogastric tube or performing endoscopy may become necessary to differentiate GI from pulmonary sources A CXR should be obtained, though the specific area of bleeding is not often visualized IV access must be established and laboratory tests obtained including CBC with differential, coagulation studies, liver function tests, blood gas analysis, and emergency type and cross match Sputum culture should also be obtained Emergency bronchoscopy to localize and treat the site of bleeding should be discussed with the primary CF team In some cases, bronchoscopy may not be helpful either because the patient has stopped bleeding or massive hemorrhage obscures visualization Most cases of severe hemoptysis are self-limited and can be managed using vitamin K, blood products, and antibiotics in an ICU setting Surgery or local vascular therapy with arterial embolization may be necessary for refractory bleeding In that situation, both rigid and flexible bronchoscopy should be available during the procedure in the operating room or ICU Ongoing management after hemodynamic stabilization includes discontinuing medications that could interfere with coagulation (e.g., aspirin, nonsteroidal antiinflammatory drugs [NSAIDs], inhaled drugs such as N-acetylcysteine, dornase alfa, and some aerosolized antibiotics), and correcting coagulation defects with vitamin K, fresh-frozen plasma, or specific factors as indicated Patients may require transfusions as clinically indicated, bearing in mind that those with severe chronic disease may be awaiting lung transplantation Whenever possible, blood products should be prepared in a manner to minimize the risk of posttransplant complications Treatment with IV antibiotics is appropriate considering most major bleeds are associated with pulmonary exacerbations Placing the bleeding lung in the dependent position may help to prevent aspiration into the as yet uninvolved lung IV therapies to halt bleeding, such as pitressin or octreotide, should be discussed with the pulmonologist Local airway treatment may be indicated in acute life-threatening situation, and include endobronchial tamponade, selective double lumen intubation, and iced saline lavage The need for and timing of embolization and access to surgery must be determined in a timely manner If a surgeon and interventional radiologist are not readily available, referral to another center should be considered Viral Respiratory Tract Infection Simple viral respiratory infections are often inciting events for pulmonary exacerbations CF patients will be more likely to suffer increased and/or prolonged symptoms due to impaired mucociliary clearance and decreased respiratory reserve Whereas CXRs are routinely not indicated for most non-CF patients with what appears to be simple URIs, patients with CF with new respiratory symptoms should have CXR obtained and compared with prior studies If there is suspicion for CF exacerbation, antibiotics should be prescribed as discussed above Wheezing Patients with CF may wheeze secondary to common diagnoses such as acute viral processes, asthma, and foreign bodies In addition, ABPA must be considered in wheezing patients with CF ABPA occurs in 1% to 15% of patients with CF It is an exaggerated type I hypersensitivity reaction to the ubiquitous organism Aspergillus fumigates Clinically, patients present with chronic wheeze that is difficult to control, decline in pulmonary function, chronic cough, and transient infiltrates on CXR Symptoms typically respond well to oral steroids Any CF patient with recurrent wheezing and cough, changes on CXR and declining lung function not responsive to antibiotic therapy and airway clearance should be evaluated for ABPA Diagnostic criteria include elevated total serum IgE level, positive skin reactivity to Aspergillus, and positive specific serum antibodies to Aspergillus Treatment consists of a prolonged course of oral steroids (prednisone or prednisolone), usually starting at mg/kg/day, with subsequent taper and close follow-up IgE levels should be followed at regular intervals both as indication of response to therapy and as a warning of reexacerbation There are no current studies to suggest a clear benefit of antifungal therapy along with steroids, although some physicians use oral itraconazole therapy as it may help shorten the course of oral steroids Pulmonary Embolism There is no current literature to suggest there is an increased incidence of pulmonary embolism (PE) in children with CF However, it should be considered in the differential diagnosis if there is acute onset of chest pain, shortness of breath, and tachypnea Because of chronic changes seen on CXR and CT with chronic lung disease, interpretation of imaging may be challenging to unequivocally confirm or refute PE The risks of anticoagulation or thrombolytic therapy for patients with more than mild pulmonary disease are not trivial, considering the propensity of CF patients to have hemoptysis Pleuritis Pleuritic chest pain can occur in CF patients during acute or subacute bacterial exacerbations or acute viral infections The pain usually improves with oral analgesia and antibiotic treatment if bacterial exacerbation is suspected Gastroesophageal Reflux Disease While many patients with CF take acid suppression medications (e.g., H2 blocker or protein pump inhibitor [PPI]) for enhancement of exogenous pancreatic enzyme function, the incidence of GERD in children with CF is as high as 55% in some studies Acute exacerbations of GERD can cause symptoms of gastritis and esophagitis including significant chest pain in the epigastrium and retrosternal regions Medications, such as NSAIDs, recent dietary changes, stress, and ethanol may exacerbate GERD An empiric trial of increased acid control may be warranted, but all patients with recurrent symptoms of GERD, including regurgitation and chest pain, should be followed closely after ED discharge In refractory cases, referral to a gastroenterologist for a formal evaluation is appropriate to determine need for upper GI series, pH/impedance probe study, and/or endoscopy Other Causes of Chest Pain Chest pain is a common complaint in patients with CF and can stem from a variety of underlying processes ( Table 99.5 ) Chest pain of cardiac origin is rare in the pediatric CF population While cardiac pain is more common outside the pediatric patient age group, the rare pediatric CF patient with severe pulmonary disease, nonpulmonary pain, and ... Chest pain of cardiac origin is rare in the pediatric CF population While cardiac pain is more common outside the pediatric patient age group, the rare pediatric CF patient with severe pulmonary... coagulation studies, liver function tests, blood gas analysis, and emergency type and cross match Sputum culture should also be obtained Emergency bronchoscopy to localize and treat the site of bleeding