Abstract Introduction Congenital lobar emphysema (CLE) is a rare pulmonary malformation characterized by overexpansion of a lung lobe without alveolar destruction, leading to respiratory distress in infancy. Its estimated prevalence is 1 in 20,000-30,000, with a male predominance (3:1). The left upper lobe is most frequently affected. It is believed that CLE results from obstruction of the developing airway that leads to “ball-valve” mechanism of air trapping. We present a case of a child with congenital malformations which were initially masked by an acute respiratory infection. Case Presentation 10-month-old Male ex 28 weeker, with Trisomy 21 presented with respiratory distress, initially attributed to RSV bronchiolitis. Initial chest x-ray demonstrated left sided air trapping, with concern of mucus plugging. His clinical status worsened and transferred to the intensive care units and pulmonology was consulted. A chest CT was ordered, which demonstrated obstructive emphysema of the left upper lobe without evidence of extrinsic compression, consistent with CLE. Additionally, a left-sided congenital diaphragmatic hernia (Bochdalek type) was identified. Due to acute illness, a decision was made to postpone any surgical procedure until the clinical picture improved. He improved gradually and was discharged, with elective surgical procedure scheduled at a later date. After the patient fully recovered, they underwent left upper lobectomy and primary diaphragmatic hernia repair via thoracotomy. Postoperative recovery was complicated by a bronchopleural fistula, requiring chest tube insertion, which was removed prior to discharge. Discussion CLE is a rare but potentially life-threatening cause of respiratory distress in infants which may mimic other respiratory conditions, delaying diagnosis. Its etiology is unknown, although some believe it might be secondary to absent bronchial cartilage, dysplasia or hypoplasia. CT imaging is the Gold standard in diagnosis and its essential in assessing the status of the adjacent lobes. Although trisomy 21 is associated in 10-20% to Congenital diaphragmatic hernia, the coexistence with CLE is rare.Surgical lobectomy remains the definitive treatment for symptomatic CLE. Infants with mild to moderate symptoms might be managed conservatively. Prognosis after surgical management is excellent with few reported complicationsConclusionThis case highlights the importance of considering congenital pulmonary malformations in children with hyperinflation, even in the setting of acute pulmonary diseases. High suspicion is essential in timely diagnosis and discussion of treatment and alternatives. This abstract is funded by: None
Alvergue et al. (Fri,) studied this question.
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