Right-sided pulmonary aplasia: Longitudinal lung function studies in two cases and comparison to results from term healthy neonates

Abstract

Agenesis of the right lung was diagnosed prenatally in two neonates born at 36 and 37 weeks, respectively. Computed tomographic scans and magnetic resonance imaging indicated that both cases had a Type 2 pulmonary agenesis, which was confirmed later by bronchoscopy. Both patients were clinically stable during the neonatal period. Serial pulmonary function tests revealed a decrease in specific respiratory system compliance (sCrs) in both neonates and a marked discrepancy between functional residual capacity measured by the nitrogen washout technique (FRCN2) and by plethysmography (FRCpleth) on follow‐up. Early decrease of respiratory system compliance (Crs) and increase of respiratory system resistance (Rrs) in one infant preceded the onset of tracheal stenosis, which remained asymptomatic until the age of 8 weeks, when the infant developed acute respiratory failure requiring intubation and mechanical ventilation with high airway pressures. Aortopexy, implantation of a tissue expander into the right hemithorax, and laser ablation of fibrotic tissue at the site of tracheal stenosis were performed to achieve successful extubation. The second infant remained asymptomatic. Values for lung mechanics and volumes for both infants with pulmonary aplasia were as follows: Crs, 3.43 and 10.60 mL · kPa−1 · kg−1; sCrs, 0.23 and 1.28 kpa−1; Rrs, 11.1 and 7.4 kpa · s · L−1; FRCN2, 14.9 and 10.2 mL · kg−1; FRCpleth, 28.2 and 25.8 mL · kg−; FRCN2: FRCpleth ratio, 0.56 and 0.54 for patients 1 and 2, respectively. These values differed considerably from results of a control group of nine term healthy neonates (Crs, 10.0 ± 1.8 mL · kPa−1 · kg−1; sCrs, 0.43 ± 0.08 kpa−1; Rrs, 5.10 ± 0.55 kpa · s · L−1; FRCN2, 24.0 ± 2.5 mL · kg−1; FRCpleth, 31.1 ± 6.0 mL · kg−1; FRCN2:FRCpleth ratio, 0.78 ± 0.10). In conclusion, serial assessment of lung mechanics and pulmonary gas volumes detects airway obstruction early in neonates with unilateral lung agenesis. Bronchoscopy is recommended. Along with conventional surgical procedures, an expandable implant may improve management or prevent respiratory failure in selected cases. Pediatr Pulmonol. 1998; 26:138–144. © 1998 Wiley‐Liss, Inc.

Our patients with developmental anomalies of lung

“…[12] Sol akciğer agenezisinde hayatta kalım daha fazladır. Agenezilerin çoğu yeni doğan döneminde respiratuvar distress gibi belirtilerle tanınabileceği gibi, belirti vermeyen olgular geç yaşlarda insidental olarak tespit edilirler.…”

Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature

“…Cases of isolated lobar defects with or without tracheobronchial abnormalities, has only been rarely reported. [8][9][10][11][12][13] With unilateral lobar pulmonary hypoplasia, gross pathology of the affected lobes shows a decrease in size and weight and, histologically, a decrease in the number of alveoli. The alveoli are often immature but otherwise demonstrate no distinct abnormalities.…”

“…Chest CT or MRI may be needed to better delineate the defect. Nowotny et al 10 recommended pulmonary function tests for infants with pulmonary hypoplasia. If PFTs reveal airflow obstruction and elevated resistance, then flexible bronchoscopy is recommended.…”

Agenesia pulmonar: reporte de doscasos

“…5 La frecuencia aproximada de la agenesia pulmonar es de 1 por cada 15 000 nacidos vivos. [6][7][8] El predominio del sexo varía según los distintos autores. La agenesia pulmonar bilateral es incompatible con la vida.…”

“…Schneider y Schwalbe la clasifican en otros tres grupos en el llamado complejo agenesiahipoplasia: [6][7][8]10 1. Agenesia pulmonar: ausencia total de pulmón, bronquio y arteria pulmonar.…”