Commentary on the Case Report on Pulmonary Hemorrhage in a 3-Year-Old Child

Etiology and Differential Diagnosis for Pulmonary Hemorrhage

An understanding of causes of hemoptysis in pediatric patients can help guide the initial evaluation. Although hemoptysis is the cardinal symptom of pulmonary hemorrhage, hemoptysis can be a result of hemorrhage from non-pulmonary sources, such as the upper airway (e.g., epistaxis) or gastrointestinal tract (e.g., ulcers). The etiologies for pulmonary hemorrhage are diverse, including infections such as pneumonia, bronchitis, abscesses, and tuberculosis; trauma such as a foreign body or lung contusion; vasculitis such as Goodpasture's syndrome or lupus; vascular malformations; increased pulmonary pressures frequently associated with congenital heart disease; coagulopathies; and more rare causes such as tumors or pulmonary capillaritis. In a retrospective study of 228 children seen at a U.S. tertiary care center, cystic fibrosis and congenital heart disease were found to account for 65% and 16% of cases of presumed pulmonary hemorrhage respectively, ² but the most common etiologies may vary by location. For example, in a South Korean study of 40 children, respiratory infections and congenital heart disease were found to account for 25% and 15% of cases respectively. ³ The differential for hemoptysis for the presented patient included gastrointestinal sources of bleeding given her prior history of abdominal surgery, infection given her chest radiograph, pulmonary hypertension given her interstitial lung disease, and her underlying interstitial lung disease itself.

Approach to the Problem, Including Diagnostic Studies

Optimal management of a patient with pulmonary hemorrhage is directed by a thorough history and physical examination. The clinician must assess when the bleeding began, how long it has lasted, and if it is continuous or intermittent. Knowledge of the amount of bleeding may also guide evaluation and treatment. Associated symptoms may also assist the evaluation; fever and cough may point toward an infectious etiology. Because foreign body aspiration is a common cause of hemoptysis in children, a thorough respiratory history can be helpful, including history of choking or coughing spell or persistent wheeze without etiology.^4,5 Growth history and generalized symptoms such as fatigue and decreased exercise tolerance may provide clues to the chronicity of the hemorrhage. The past medical history and underlying medical conditions (e.g., cystic fibrosis, tracheostomy) may clarify an etiology. Finally, travel and exposure histories may increase suspicion for bleeding due to a specific source or infection. Additionally, specific physical examination findings can aid in the evaluation of pulmonary hemorrhage. Careful examination of the head and neck may reveal a nasopharyngeal source. Patients may be tachycardic, short of breath, or hypoxemic, pointing toward a more acute, possibly massive, hemorrhage. Pallor, cyanosis, or digital clubbing may indicate a more chronic process.

Laboratory and radiologic studies may aid in determining the cause of pulmonary hemorrhage and guide management. Basic studies, including complete blood count and possibly coagulation studies, may diagnose infection, anemia (due to chronic blood loss), and coagulopathies. Sputum studies in patients with suspected infection may be diagnostic and guide antibiotic therapy. Electrolytes, renal and hepatic function panels, and urinalysis may help diagnose more rare causes of pulmonary hemorrhage. ⁶ Chest radiograph should be performed in all patients with suspected pulmonary hemorrhage, as this may reveal underlying infection, foreign body (with areas of air trapping), and even interstitial processes. Furthermore, chest imaging may help localize the source of the bleeding. High resolution chest computed tomography (HRCT) with contrast should be considered in cases where chest radiographs are not helpful or when vascular lesions or interstitial processes are strong possible etiologies. ⁷ Diffuse patchy findings on imaging combined with positive serologic laboratory studies should prompt consideration of pulmonary capillaritis, which, although rare, can be life threatening. ⁸ Finally, if the pulmonary evaluation is negative (including bronchoscopy), then cardiac evaluation should be considered. This evaluation may include an echocardiogram to evaluate for heart defects and pulmonary hypertension, and a ventilation/perfusion scan to evaluate for possible pulmonary embolism.

Early in the evaluation, it is essential for the clinician to determine if bleeding is from the respiratory system or the gastrointestinal tract. Patients with hemoptysis (bleeding from the respiratory tract) often do not have nausea and vomiting, may have a history of lung disease, and after the acute episode, the sputum remains blood tinged. Patients with gastrointestinal bleeding often have a history of nausea and vomiting, underlying gastric or liver disease, and their sputum clears after the acute episode. Evaluation of the sputum contents can also be helpful. ⁹ In hemoptysis, the sputum is frothy, liquid, or clotted and bright red to pink in color with an alkaline pH and white blood cells on laboratory analysis. Patients with hematemesis produce material with a coffee-ground appearance that is brown to black in color and mixed with food particles with acidic pH. In this case, the patient was both coughing and retching, and had a history of gastric surgery (Nissen and GT), thus direct visualization was indicated to confirm the suspected pulmonary hemorrhage. Furthermore, flexible and rigid bronchoscopy can be helpful in direct treatment of the bleeding and to determine the specific location of the bleeding should more aggressive management be necessary. ¹⁰ Finally, in cases of intermittent bleeding or when more rare causes of pulmonary hemorrhage are suspected (e.g., idiopathic pulmonary hemosiderosis [IPH]), laboratory evaluation of bronchoalveolar lavage fluid is instrumental in making the diagnosis. The presence of hemosiderin-laden macrophages in the bronchoalveolar lavage fluid may be detected 3–14 days following an episode of hemoptysis in patients with IPH, a diagnosis of exclusion. ¹¹

In the presented case, endoscopy of upper gastrointestinal tract did not reveal any sources of bleeding, and subsequent events during reversal of anesthesia proved the source to be pulmonary. As pediatric interstitial lung disease can be associated with pulmonary hypertension, an echocardiogram was obtained. Her pulmonary pressures were estimated to be 50% of systemic pressures, but this elevation in pressure may be a function of invasive positive pressure ventilation as well as hypercarbia from one-lung ventilation. A subsequent echocardiogram 2 months later demonstrated normal pulmonary pressures. Hemoptysis or pulmonary hemorrhage has not been reported with the patient's interstitial lung disease, neuroendocrine cell hyperplasia of infancy (NEHI). Thus we suspect the patient's NEHI and her hemoptysis were unrelated, and the most likely etiology for the pulmonary hemorrhage was pneumonia.

Treatment Options and Limitations

In most cases of minor pulmonary hemorrhage, clinical stabilization, expectant observation, and treatment of the underlying cause leads to successful resolution. In the vast majority of pediatric hemoptysis cases in the developed world, infection is the underlying cause and bleeding is self-limited with treatment and recovery from the inciting infection.^2,4,12 Early use of immunosuppression may be warranted when pulmonary capillaritis or vasculitis is present and infection is excluded. ⁸ When pulmonary hemorrhage becomes massive and potentially life threatening, as in this case, more aggressive management is warranted. To prevent asphyxiation, selectively intubating the non-bleeding lung and placing the patient in a side-lying position with the affected lung decubitus can be lifesaving. ¹³ Occlusion of the bleeding lung may aid in protecting the healthy lung and may be therapeutic to stop the bleeding. This can be performed by using a balloon catheter or increased bronchoscopic pressure to tamponade the bleeding from the airway. ⁹ If the bleeding can be directly visualized and well controlled in the setting of flexible bronchoscopy, application of topical vasoconstrictors (e.g., oxymetazoline, epinephrine, iced 0.9% saline) may decrease blood loss. ¹⁴ If bleeding is ongoing or cannot be localized, arteriography and selective bronchial artery embolization may be necessary to identify the source and stop the bleeding. Embolization in children usually takes place with specifically sized polyvinyl alcohol particles or gelatin sponge, rather than larger metal coils, which limits the occlusion of other nearby vessels and unintended complications.^15–17 A more serious complication of this procedure is unintended embolization of the spinal arteries and subsequent neurologic ischemia and injury. Embolization can also be used as a temporizing measure to stabilize a patient in whom definitive resection of the bleeding lesion is necessary. Surgical intervention (ranging from segmentectomy to pneumonectomy) is sometimes required for ongoing severe bleeding, but must be considered carefully due to the resulting loss of potentially recoverable lung tissue.^16,18 In this case, Interventional Radiology and Surgery were consulted, but both services felt she would be a poor candidate for any urgent procedures based on her small size and poorly localized lesion. Expectant observation was recommended and resulted in resolution of the acute hemorrhage and the patient's subsequent extubation the following day.

Acute hemoptysis can be frightening for both children and their parents. In this discussion, we have provided an approach to the pediatric patient with pulmonary hemorrhage. In summary, clinicians must make certain breathing continues, bleeding stops, and the cause of the bleeding is diagnosed and treated when possible. Communication between all providers involved in the patient's care is paramount to ensuring a safe and optimal outcome, as occurred with this case.