Congenital lobar emphysema is a rare cause of respiratory distress during infancy which is cured by surgery. Normally male babies are affected more often than females. Congenital lobar emphysema should be suspected in any infant presenting with breathlesness. It may present immadiately at birth, in the neonatal period or in infancy [
2]. It is usally unilateral, affecting the left upper lobe, followed by the right middle lobe and right upper lobe, althoug bilateral involvement has been reported [
3]. However, in our series the left upper lobe was most common affected (3/4). The exact cause of CLE is to determine, and no apparent cause is found in 50% of cases [
4]. The current theory suggests inadequate cartilagenous support of the bronchus, has been by. It has been found an increase in the number of alveoli in the affected lobe.
Congenital lobar emphysema produces symptoms in infancy. Often, a history of tachypnea, retraction of the chest wall, and wheezing since birth exists [1,2]. In our cases, the onset of the symptoms was typically in early infancy. Physical examination reveals a shift of the trachea and mediastinum to the contrlateral hemithorax. Breath sounds are decreased on the affected side, with associated hyperresonance. The diagnosis is usually confirmed on a plain chest radiograph, often with mediastinal displacement and herniation of the affected lobe may compress the contrlateral lung [2], and in these cases lung isotope scanning is indicated to differentiate CLE from hypolasia of the contrlateral lung [5]. On a plain chest x-ray, careful inspection of vascular markings reduces the risk of misdiagnosis of this lesion as a tension pneumothorax. One of the our patients initially interpreted as pneumothorax, and diagnostic aspiration was performed at another institution. A chest drain was inserted with no further air drainage and no clinical improvement. Attempts at needle aspiration must be avoided, for this almost results in a tension pneumothorax that can be fatal. The radiological differential diagnosis includes pneumothorax, pneumatocele, atelectasis, or hypoplasia of the lung with hyperinflation of the contrlateral lung, diaphragmatic hernia, and congenital cystic adenomatoid malformation [8]. In an infant with mild to moderate respiratory distress, CT can establish the diagnosis of CLE by showing the hyperlucent expanded lobe and stretched, attenuated vessels and CT can also exclude extrinsic causes of lobar emphysema, such as vascular anomalies or mediastinal mass. Chest CT was performed in three patients and confirmed the radiographic finding. Bronchoscopy can worsen patients condition, but may be useful in excluding non-opaque foreign as the cause of the hyperinflation in older children [5]. Bronchoscopy was performed in one patient aged ten months, who presented with acute respiratory symptoms suggestive of foreign body inhalation. The ventilation / perfusion radionucleide scan is useful in evaluating the function of the affected and compressed lobes. However, it is difficult to perform in a small infant.
Despite reports of successful conservative management [6] the accepted treatment is surgical lobectomy of the affected lobe. Operative mortality rate is 3% to 7% whereas with conservative therapies it is 50 to 75% [7]. The conservative treatment may be considered in selected infants and older children with mild symptoms. In neonates and infants with severe respiratory distress, emergency thoracotomy may be life saving. In the present study emergency lobectomy was performed in one patient. At operation, the chest is opened as soon as possible after induction of anesthesia. Positive-pressure ventilation causes further overinflation of the involved lobe and increases the risk of mediastinal shift and cardiac arrest. Before resection, the mediastinum must be carefully examined for lesions that could have obstructed the bronchus.
We feel that surgical excision of the affected lobe could be managed as soon as possible in all infants younger than 2 months, and in infants older than 2 months who present with severe respiratory symptoms. Conservative management can be performed in older children who present with mild symptoms with a close follow-up of the patient.
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