Recurrent pneumonia in an otherwise healthy child

Recurrent pneumonia is defined as having at least three episodes of pneumonia in 1 year, or three episodes ever with radiographic clearing of densities.1 Aspiration, immune deficiency, foreign body aspiration, structural airway abnormalities, and congenital pulmonary lesions can all lead to recurrent pneumonia. However, patients with untreated asthma are often misdiagnosed as having recurrent pneumonia.2 Therefore, deciding on further diagnostic workup and management can be challenging. Here, we describe the case of a 9-year-old previously healthy male who was referred to the pediatric pulmonary clinic for recurrent pneumonia. Institutional Review Board review is waived by institutional policy (Mass General Brigham IRB). A 9-year-old healthy male presented to the emergency department with 2 days of cough, right-sided chest pain, and fever to 103°F. He denied abdominal pain, however, did vomit (nonbilious, nonbloody) three times the previous night. He did not recall any choking events. There was no diarrhea, urinary symptoms, or labored breathing. There was no significant family history of respiratory disease and no recent travel or exposures. His heart rate was 132 (normal range 60–100), with a respiratory rate of 20 and an oxygen saturation of 97% while breathing room air. Physical examination revealed decreased breath sounds in the right lung base, but no wheeze, rhonchi, or crackles. A chest radiograph demonstrated a large, rounded opacity in the right middle lobe (RML). He was diagnosed with a RML pneumonia and discharged with amoxicillin and azithromycin along with close follow up with the pediatrician. Symptoms resolved and he was well for 2 months until he again developed a cough. The cough lingered for approximately 2 months, but he remained active and was not limited in daily activities. The development of fever to 101°F and the recurrence of right-sided chest pain led to a repeat chest radiograph which again demonstrated a consolidation in the RML, and a new consolidation in the right lower lobe. He appeared well, had a clear chest upon auscultation, and the oxygen saturation was 99% on room air. He was treated with amoxicillin-clavulanic acid and azithromycin. The recurrence of the RML pneumonia, 4 months apart, prompted a referral to pediatric pulmonology. One month later at the pulmonary clinic he was well-appearing, breathing comfortably with an oxygen saturation of 98% on room air. He was afebrile, endorsed pleuritic right sided chest pain, and examination revealed rhonchi over the RML. There was no wheeze or clubbing on exam. He was growing and developing normally. Spirometry was normal with no signs of obstructive lung disease. A chest radiograph (Figure 1), taken 1 month after the most recent abnormal film, demonstrated a patchy opacity in the medial segment of the RML and peri-bronchial cuffing (Podcast 1). A 9-year-old previously healthy male presents for pediatric pulmonary consultation for recurrent RML pneumonia. He was found to have persistent pleuritic chest pain and radiologic findings in the RML despite antibiotics. What is most likely going on with this patient (leading and differential diagnosis)? What would you do next and why? Listen to Podcast 2 to hear the consortium's decision-making process. Computed tomography (CT) angiography of the chest with intravenous contrast (without expiratory views) was obtained 5 months after the initial presentation of cough and chest pain and demonstrated a small right hilar mass with impingement of the medial segment of the RML with postobstructive mucous plugging (Figure 2). A 9-year-old male with persistent abnormalities in the RML on chest radiography has rhonchi over the same location and a newly discovered mass on chest CT. What is the most likely etiology of the mass found on CT scan (leading and differential diagnosis)? What diagnostic interventions or therapeutic measures would you consider given the findings on the chest CT? Listen to Podcast 3 to hear the consortium's decision-making process. The patient was scheduled for a flexible bronchoscopy with pediatric pulmonology in conjunction with the interventional pulmonary group in anticipation of acquiring a biopsy for tissue diagnosis. Bronchoscopy showed normal airway mucosa with a white to tan mass obstructing the medial segment of the RML (Figure 3). It was not vascular and there was no bleeding. The mass was debulked with forceps and initial intraoperative pathology was consistent with a mucoepidermoid carcinoma. A 9-year-old previously healthy male with recurrent pneumonia in the RML was ultimately found to have an endobronchial tumor consistent with a mucoepidermoid carcinoma. What further workup, if any, would be warranted in this scenario? What is the most appropriate management? Listen to Podcast 4 to hear the consortium's decision-making process. Multidisciplinary discussions between the pulmonologist, the pediatric surgeon, and the hematologist–oncologist led to the recommendation of a RML lobectomy. Given the low-grade nature of the tumor, and low likelihood of spread, the team felt comfortable not pursuing further diagnostic imaging. He underwent a successful RML lobectomy and spent two nights on the pediatric floor before being discharged home. Final pathology revealed a 1.2 cm low-grade mucoepidermoid carcinoma (Figure 4) with no evidence of spread to local lymph nodes. A previously healthy 9-year-old boy presented to pulmonary clinic with recurrent pneumonia in the RML. Ultimately, a chest CT and biopsy led to the diagnosis of a mucoepidermoid carcinoma which was successfully resected. Community acquired pneumonia is common in childhood and is often diagnosed clinically. This patient was diagnosed with recurrent pneumonia based on two episodes of pneumonia over a 4-month period. Recurrent pneumonia by definition depicts radiographic clearing of the disease. Normally, the chest radiograph lags behind clinical symptoms and can take 4–6 weeks to fully clear. The initial pulmonary visit occurred 1 month after the most recent episode of radiologically diagnosed pneumonia. This was long enough for clinical symptoms to have cleared, but still in the window when radiographic abnormalities may persist. Therefore, we had to determine if the clinical picture was consistent with a resolving lower respiratory tract infection or if there was an underlying etiology leading to recurrent symptoms. Untreated asthma is often misdiagnosed as recurrent pneumonia.2 Airway inflammation and airway wall edema leads to retention of secretions and atelectasis which may present similarly to pneumonia. Furthermore, the RML is the most commonly affected area due to the RML bronchus being narrow and having poor collateral ventilation decreasing the likelihood of reinflation after the development of atelectasis.2 This patient had adventitious breath sounds and described chest pain over the RML 4 weeks after completing therapy for pneumonia. He was afebrile, breathing comfortably, had a normal oxygen saturation level, and pulmonary function testing did not reveal obstruction. Therefore, it was less likely the current chest radiograph findings represented a new infection, reversible airway obstruction, or simply a lag of radiographic clearing of a previous illness. Furthermore, this patient did not have an atopic history or a clinical picture depicting cough, wheeze or dyspnea with viral illnesses. The absence of a choking event or localized wheeze on exam made a foreign body aspiration less likely but did not completely rule that out as a possible diagnosis. A chest CT was ordered as we needed to expand the differential diagnosis and better characterize the persistent abnormality seen in the RML. Once the chest CT revealed the endobronchial mass, it was crucial to obtain tissue which would guide treatment options. Primary lung tumors can arise from the lung parenchyma, the airway, or the chest wall and do not present with typical findings associated with pediatric malignancies such as fever, weight loss or fatigue.3 Therefore, a high index of suspicion is necessary for a timely diagnosis which may decrease the morbidity associated with surgical resection. Airway tumors are rare in the pediatric population and often present as cough, wheeze, or recurrent pneumonia. This patient presented with recurrent respiratory symptoms and persistent findings of a consolidation in the RML. Benign airway tumors, such as hemangiomas, papillomas, inflammatory pseudotumors, or granular cell tumors most frequently occur in the larynx or proximal trachea. Whereas malignant airway tumors including carcinoid, mucoepidermoid carcinoma, and adenoid cystic carcinoma tend to occur more distally. Bronchial tumors account for approximately 5%4 of all primary pulmonary neoplasms in children, but are the largest group (40%) of primary malignant lesions.5, 6 Upon gross examination, this endobronchial lesion did not appear vascular in nature given the absence of any bleeding surrounding the tumor. Furthermore, carcinoid tumors most often present with recurrent wheeze, cough and potentially hemoptysis.7 Therefore, mucoepidermoid carcinoma, which often presents as recurrent pneumonia7 was high on the differential once we visualized the lesion endoscopically. Mucoepidermoid carcinomas arise from bronchial mucous glands8 and the exact incidence is unknown. They are classified as either low-grade or high-grade lesions. Low-grade lesions tend to occur in younger patients, often less than 30 years of age, whereas high-grade lesions are more prevalent in older populations.9 Complete resection of low-grade lesions carries an excellent prognosis,10 especially in the absence of local spread which was absent in this case. This case highlights the challenges that pediatricians face when managing patients with recurrent pneumonia. Many cases do not require further diagnostic evaluation. However, recurrent symptoms and persistent abnormalities on chest radiography without a clear etiology do warrant further investigation. Airway tumors are exceedingly rare in the pediatric population, but prognosis is favorable if diagnosed at a relatively early stage of illness. All authors contributed to the development, writing, and editing of this manuscript. The authors declare no conflict of interest. Data sharing is not applicable to this article as no new data were created or analyzed in this study. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. (Radiologist): The initial chest radiograph is a frontal view of the chest which demonstrates an ill-defined opacity abutting the right heart border as well as the medial portion of the right hemi-diaphragm which seems to be localized to the right middle lobe. The remainder of the lungs are clear, there is no pleural effusion or pneumothorax. This is consistent with right middle lobe pneumonia. After 4 months there is either a recurrence or persistence of the opacity in the right middle lobe, and now appears more dense with a parapneumonic effusion. The most recent chest radiograph demonstrates resolution of the opacity, however, there is an unusual convex shape of the right heart border which signifies a lesion in the right middle lobe. Given the history of pneumonia in this area, I would suggest a computed tomography (CT) scan of the chest. (Pulmonologist #1): I would be concerned for an endobronchial lesion given the persistent findings in the right middle lobe. This does not seem consistent with a congenital lesion such as a congenital pulmonary airway malformation or a bronchopulmonary sequestration. The lack of clubbing and systemic findings makes a chronic pulmonary issue less likely. (Pulmonologist #2): If possible, I find it helpful to review prior imaging to determine if there might be a congenital lesion, but this patient did not have any previous imaging. Despite the lack of an obvious choking episode, a foreign body aspiration would be on the differential. (Pulmonologist #3): Persistent findings in the right middle lobe always make me think about underlying asthma, so I may have obtained postbronchodilator spirometry and possibly treated for asthma with the plan to repeat the chest radiograph in 4–6 weeks. However, the initial fever does point more toward an infectious process at the onset. There is no history of recurrent infections, chronic diarrhea, or poor growth to suggest an immune deficiency. (Pulmonologist #1): I think asthma and atelectasis are less likely given the localization of findings specifically to the right middle lobe. Therefore, I think we need to obtain a chest CT. (Radiologist): There is a round nonenhancing mass impinging on the medial segment of the right middle lobe with postobstructive atelectasis. The shape and configuration of the mass suggest it is endobronchial. Carcinoid tumors, which can be found endobronchially are often enhancing lesions, therefore, this would not be typical for a carcinoid tumor. This could be consistent with a foreign body aspiration with surrounding granulation tissue and mucous plugging. Atelectasis in the absence of infection typically enhances on chest CT. The lesion I see here is not-enhancing suggesting the postobstructive lesion is more consistent with mucous plugging than atelectasis. (Pulmonologist #1): At this time a foreign body or a carcinoid tumor were high on the differential. Although less likely, endobronchial tuberculosis (TB) could present in similar fashion. (Radiologist): If this were TB, the mass could represent a necrotic lymph node, however, I would have expected to see rim enhancement and pneumonitis which are not present. (Pulmonologist #2): This lesion is focal and localized to the right middle lobe. I might go back and take a detailed family history to assess for neuroendocrine tumors, for example. At the same time, I would consult my surgical and hematology oncology colleagues to discuss the best way to obtain a tissue sample. (Pulmonologist #3): This could be consistent with a bronchogenic cyst, or a granuloma in the setting of a histoplasmosis infection if there was travel to endemic areas. Ultimately, this will require a bronchoscopy and biopsy to determine the etiology of this lesion. (Pulmonologist #3): This is a malignant tumor, therefore, I would discuss next steps with hematology oncology. In the short term, I would want to see the patient in clinic after the biopsy to determine if there is evidence of retained lower airway secretions. (Pulmonologist #2): I would also want to follow up on the bronchoalveolar lavage (BAL) and would treat with antibiotics if the culture grew any pathogens. I would also consider airway clearance modalities if there was concern for retained lower airway secretions. (Pulmonologist #1): I was not sure what the likelihood of spread could be at this stage. If this type of tumor tends to spread, then he would need more diagnostic testing to determine the extent of tumor burden. However, if it tends to remain local, then surgical excision could be curative.