Recurrent dyspnea caused by congenital lobar emphysema in a premature child

To the editor, We report a pediatric patient with congenital lobar emphysema (CLE) who presented with recurrent dyspnea and respiratory infections, and was initially diagnosed with bronchopulmonary dysplasia (BPD) and pneumonia. A 5-year-old girl was admitted to the hospital with a complaint of dyspnea and cough for 5 days. No other symptoms, such as fever, wheezing, chest pain, or vomiting were described during the disease course. Upon physical examination, no cyanosis or finger clubbing was found. The breathing sounds in both lungs were harsh, and fine moist rales were auscultated. Examination of the heart, abdomen and nervous system revealed no abnormalities. Laboratory investigations revealed a total white cell count of 12.9 × 109/L, a neutrophil percentage of 66.3%, and a CRP level of 20.5 mg/L. The results of routine urine and stool tests, sputum culture, electrocardiogram and cardiac ultrasound revealed no abnormalities. Chest CT revealed patchy shadows and scattered consolidation in both lungs, particularly in the right lung. Obvious emphysema was found in the middle lobe of the right lung, and the volumes of the upper and lower lobes of the right lung were reduced (Figure 1). We reviewed the child's previous medical history and chest radiography. She was born prematurely at 33 + 4 weeks of gestational age and recieved oxygen for 10 days. However, she did not have a history of ventilator use. At 7 weeks of age, the patient was admitted to the ICU again because of dyspnea. During this time, the patient underwent two chest X-ray examinations, both of which revealed patchy shadows in the upper and lower fields of the right lung. A chest CT at 84 days of age revealed atelectasis in the upper lobe of the right lung and emphysema in the right middle lobe (Figure 1). She was diagnosed with severe pneumonia and bronchopulmonary dysplasia. As the child aged, decreased activity tolerance was observed. Five episodes of lower respiratory tract infection were reported in the past year, four of which involved wheezing symptoms. The patient's growth and development were similar to those of her peers. A family history of similar conditions was denied. During the hospitalization, a bronchoscopy was performed. Through the bronchoscopy, we found that the mucosae of the right upper lobe and right middle lobe were obviously swollen. The posterior branch of the right upper lobe and the opening of the right middle lobe were narrow. Multiple white and viscous secretions could be observed in both bronchial lumens (Figure 1). On the basis of the medical history, bronchial examination results, and multiple chest imaging examinations after birth, we diagnosed this patient with congenital lobar emphysema and pneumonia. Owing to recurrent respiratory infections in the patient, the surgeon recommended surgical treatment. However, the parents chose to receive conservative treatment and regular follow-up. After receiving anti-infection treatment, the child's symptoms of cough and dyspnea were relieved, and she was discharged from the hospital. CLE is a rare congenital lung malformation, and it is defined as the overinflation of one or more pulmonary lobes. The estimated incidence of CLE is 1/20,000–1/30,000. A total of 14%–20% of the patients with CLE have cardiac anomalies.1 The dysplasia or hypoplasia of the bronchial cartilage is the possible cause of the disease, which results in air trapping during expiration. This leads to emphysematous changes in the involved lobe and compression of the adjacent lobe, ultimately causing respiratory distress.2 Owing to a lack of understanding of the disease, it is often misdiagnosed as pneumothorax, pneumonia, atelectasis, or foreign body aspiration, which leads to unnecessary antibiotic treatment and delays proper therapy and diagnosis.3, 4 Here, we report a pediatric patient with respiratory distress and recurrent respiratory infections. Multiple chest imaging examinations revealed obvious emphysema in the right middle lobe and atelectasis in the upper and lower lobes of the right lung. Bronchoscopy revealed an abnormal bronchial structure. Fortunately, the child did not have malformations of other organs. The patient in our study was a child who was born prematurely. She was diagnosed with BPD shortly after birth because of breathing difficulties. However, the underlying disease was ignored. Actually, severe BPD rarely manifests as obvious emphysema and atelectasis in the early postnatal period. In addition, this infant had no long-term history of mechanical ventilation therapy during early disease stages. On the basis of the current evidence, there are two treatment options for this disease. Symptomatic treatment is recommended if the child's symptoms are mild and there is no history of recurrent respiratory infections. Pneumonectomy is preferred for patients who have clinical deterioration or recurrent severe respiratory tract infections. A lobectomy is often curative, and it allows the surrounding compressed lung to expand.5 A lobectomy was recommended for this patient because of repeated respiratory infections and respiratory distress. However, the child did not choose lobectomy because of concerns about the operation's side effects. In conclusion, we aim to increase the awareness of CLE. When a child presents with recurrent respiratory infections, recurrent dyspnea, local lobar emphysema and adjacent lobar atelectasis, the diagnosis of CLE should be considered, and surgical treatment options should be provided for the child. When this child is born prematurely, it is especially important to distinguish CLE from BPD. Early diagnosis and appropriate treatment of diseases can significantly improve disease outcomes. Lili Wang: Conceptualization; methodology; software; data curation; supervision; formal analysis; validation; investigation; project administration; resources; visualization; writing—review and editing; writing—original draft. Lina Chen: Conceptualization; funding acquisition; supervision; writing—review and editing; validation; resources. Hanmin Liu: Conceptualization; funding acquisition; writing—review and editing; validation; supervision; resources. This study was supported by the following funding: (1) General Program of the National Natural Science Foundation of China (82370001); (2) Science and Technology Major Project of Sichuan Province (2022ZDZX0021); (3) Fundamental Research Funds for the Central Universities (SCU2022D022); (4) Development Fund for Clinical Discipline of West China Second Hospital of Sichuan University (KL118). The authors declare that there are no conflicts of interest. The Institutional Review Board/Ethics Committee affiliated with West China Second University Hospital, Sichuan University (Ethics review number: 2022074), approved this study, which was performed in accordance with the ethical standards of the Declaration of Helsinki. All the datasets generated and analyzed in the present study are included in this published article.