Recurrent pneumonia in a 2-year-old: A rare case of congenital lobar emphysema

Abstract

Introduction and significance

Emphysema is an uncommon but important condition that often appears in the neonatal period. Diagnosis is based on CT, which identifies the affected lung lobe, which is treated with complete surgical resection.

Case presentation

We present a case of a child who had been suffering for about a year from recurrent respiratory infections without arriving at a clear and correct diagnosis. He was evaluated by us and diagnosed correctly despite the difficulty of distinguishing it from pneumothorax. The final treatment was surgical removal.

Clinical discussion

Emphysema is considered one of the important conditions that should be considered as a differential diagnosis if there is clear hyperinflated in the pulmonary lobe. The evaluation is mainly done through CT to reach the correct diagnosis and treatment.

Conclusion

Congenital lobar emphysema is a rare condition that primarily affects children. The majority of children with CLE experience symptoms and necessitate surgery.

Highlights

• •Congenital lobar emphysema (CLE) is a rare lung disorder.
• •Diagnosis is primarily based on a chest CT scan. Echocardiogram may be used to assess for any coexisting heart abnormalities.
• •Surgery is the preferred treatment for symptomatic cases. Close follow-up is essential, even for patients managed conservatively.

1. Introduction

Congenital lobar emphysema (CLE) is a rare lung malformation affecting approximately 1 in 20,000 to 30,000 newborns [1]. It occurs when one or more lung lobes become abnormally due to a partial obstruction within the airways. This obstruction can compress adjacent organs and shift the mediastinum towards the opposite lung. Most CLE cases are diagnosed shortly after birth or in infancy. Adults with CLE are very uncommon, as symptoms typically appear early in life. Common symptoms include tachypnea and a cough [[2], [3]]. A high index of suspicion is essential for diagnosing CLE, especially given its rarity and potential misdiagnosed as pneumothorax. This misdiagnosis can lead to unnecessary procedures, such as chest tube insertion. Chest X-ray is the initial step in diagnosis, typically revealing an enlarged affected lobe and a displacement of the mediastinal structures [4]. In our case, we will present a case of CLE that was managed wonderfully and successfully without pneumothorax misdiagnosis and inappropriate pneumonia treatment, as this case developed an excellent plan for managing suspected cases of CLE.

This case is described in accordance with the criteria of SCARE [5].

2. Presentation of case

2.1. Patient information

We describe the case of a 2-year-old male with a history of recurrent respiratory infections since birth and multiple hospital admissions at an external hospital. The parents reported that the child presented with sudden shortness of breath without fever or cough, prompting a visit to the emergency department of our hospital.

3. Clinical findings

When he arrived at the emergency department, his general condition was good. No central or peripheral cyanosis was observed, and nor were there no signs of use of the accessory pectoral muscles.

4. Diagnostic assessment

Heart rate of 128 beats per minute, blood pressure of 103/62, temperature of 98.2 Fahrenheit, respiratory rate of 43 breaths per minute, and oxygen saturation of 88 % on room air.

A chest X-ray revealed a large air cyst in the upper lobe of the right lung, causing compression of the mediastinum (Fig. 1A-B).

Fig. 1.

A: Chest X-ray (anterior-posterior view) demonstrates hyperinflation of the right upper lobe with a shift of the mediastinal structures to the left.

B: Chest X-ray (lateral view) reveals a large air cyst in the right upper lobe.

C: Chest CT scan (cross-sectional view) shows emphysematous changes with cystic formations involving the right upper lobe. The left lung appears normal, without evidence of inflammatory infiltrates.

Computed tomography (CT) of the chest showed hyperinflation of the upper lobe of the right lung, with cystic cavities within it. The left lung appeared normal (Fig. 1C).

The echocardiogram showed a hyperkinetic heart with mild mitral and tricuspid valve insufficiency.

5. Therapeutic intervention

Based on the diagnostic findings, surgery was performed under general anesthesia. After isolating the right lung, a posterior lateral incision was made on the fifth right intercostal space, accessing the chest cavity. The right upper lobe remained enlarged even after lung isolation, confirming the presence of congenital lobar emphysema (Fig. 2A). To ensure adequate ventilation of the remaining lower and middle lobes, the entire upper lobe was resected (Fig. 2B). A chest tube was placed for drainage, and the chest wall was closed.

Fig. 2.

A: Intraoperative photograph clearly shows congenital lobar emphysema (CLE) in the right upper lobe.

B: Gross specimen of the resected right upper lobe.

Histopathological examination of the resected tissue demonstrated chronic nonspecific inflammation with moderate infiltrates and emphysematous changes within the lung parenchyma (Fig. 3A-B).

Fig. 3.

A: Hematoxylin and eosin (H&E) stained section shows inflammatory infiltrates and hemosiderin macrophages.

B: H&E stained section demonstrates moderate emphysematous changes.

The child was monitored for approximately five days. The chest tube was then removed, and they were discharged in good overall condition.

6. Discussion

CLE is a rare lung malformation that develops during fetal lung development, typically in the third trimester of pregnancy [6]. The exact cause remains unknown, but several factors are suspected to play a role, including:

• Abnormal cartilage formation: Defects in bronchial cartilage, crucial for airway support, are seen in about 25 % of diagnosed cases. This can lead to airway obstruction.

• Blocked airways: Other factors that can obstruct airways and contribute to CLE development include malformations of the blood vessels supplying the lungs and cytomegalovirus infection [7].

Males are slightly more likely to be affected by CLE than females. The malformation most commonly involves the left upper lobe, involvement of the lower lobes is rare.

Most CLE cases present in newborns or early childhood. Adult cases are uncommon. Symptoms of CLE can vary and may not appear immediately. They can be triggered by recurrent respiratory infections that trap air in the lungs.

Children with CLE may experience: respiratory distress, tachypnea, use of additional chest muscles to assist breathing, decreased breath sounds over the affected lung and high-pitched whistling sound (wheezing) in the affected area.

Chest X-ray is the initial imaging tool used to suspect CLE. It can reveal an overinflated lobe and a mediastinal shift towards the affected side.

In our case, a Chest X-ray is considered a typical and ideal image to describe CLE. It is observed that hyperinflated in the right upper lobe, in addition to the right upper lobe crossing the median line, which is not observed in the case of pneumothorax.

However, chest CT scan is considered the gold standard for diagnosis [8]. Providing a detailed view of surrounding lung tissue, the unaffected lung, and potential abnormalities such as vascular malformations or masses in the chest cavity.

Bronchoscopy has limited use in diagnosing CLE but may be considered if there's a history of foreign body aspiration or to assess airway variations [9].

Echocardiography, an ultrasound of the heart, is crucial due to the association between CLE and heart abnormalities in 14–20 % of cases [6]. It helps identify any coexisting heart problems.

Conservative management may be considered for patients with mild or no symptoms. These patients require close monitoring, as symptoms may develop, and surgery may become necessary in the future [10].

Surgery is the primary treatment for CLE, particularly for cases with severe symptoms. Early intervention is recommended to prevent complications. Most patients who undergo surgery experience symptom resolution and achieve normal growth and development.

7. Conclusion

Differentiating congenital lobar emphysema from pneumothorax is crucial. Our case serves as a textbook example of managing this rare condition effectively. By carefully evaluating clinical findings and radiographs, we avoided premature surgical intervention and ensured an accurate diagnosis and appropriate treatment plan.

Abbreviations

CLE

Congenital Lobar emphysema

CT

Computerized tomographic

Ethical approval

Ethics approval and consent to participate approval is not required for deidentified single case reports or histories based on institutional policies. Single case reports are exempt from ethical approval.

Sources of funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contribution

Kamar Shaker: conceptualization, resources, who wrote, original drafted, edited, visualized, validated, literature reviewed the manuscript.

Khaler Alomar: resources, who wrote, original drafted, corresponding author who submitted the paper for publication.

Mahmoud Aldabea and Hanady Zwaraa: visualization, validation, and review of the manuscript.

Nashwa Alnwaijie: Specialist in pediatric surgery, she performed the surgery and supervised the preparation of the manuscript.

Husam Aldalati: Professor of Pediatric Surgery. Head of the Pediatric Surgery Division at the University Children's Hospital in Damascus. Supervising the surgical operation and following up and coordinating the publication of the manuscript.

All authors read and approved the final manuscript.

Guarantor

Kamar Shaker.

Research registration

N/A.

Consent of patient

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Declaration of competing interest

The authors declare that they have no competing interests.

Data availability

The datasets generated during and/or analyzed during the current study are not publicly available because the Data were obtained from the hospital computer-based in-house system. Data are available from the corresponding author upon reasonable request.