Is It Better to Operate Congenital Lung Malformations when Patients are Still Asymptomatic?

J Indian Assoc Pediatr Surg. 2021 May-Jun;26(3):170-176. doi: 10.4103/jiaps.JIAPS_70_20. Epub 2021 May 17.

Abstract

Context: Congenital lung malformation (CLM) is a rare developmental anomaly of the lower respiratory tract. The purposes are to define if the presence of respiratory symptoms, in CLM may affect surgical outcomes and to define optimal timing for surgery in asymptomatic patients.

Settings and design: Retrospective review of patients with CLM from 2004 to 2018. Asymptomatic and symptomatic patients were compared. Moreover, asymptomatic patients were stratified according to age at surgery (< or >6 months).

Subjects and methods: Demographic data, prenatal diagnosis, symptoms, CLM's characteristics, operative and postoperative data were collected. Patients were divided into two groups based on the presence or none of respiratory symptoms.

Statistical analysis: Data were compared using the Fisher's exact test for qualitative values and Mann-Whitney test for quantitative values P < 0.05 was statistically significant.

Results: One hundred and eighty-six patients were treated. Asymptomatic were 137 (74%), while symptomatic were 49 (26%). The most common presenting symptoms were respiratory distress (n = 30, 61%) followed by pneumonia (n = 18, 38%). Prenatal diagnosis of CLM was performed in 98% of asymptomatic patients compared to 30% of symptomatic (P = 0.001). Surgical excision was performed in all cases, and in 50% by thoracoscopy, without difference between the two groups. In 97% of all cases, a lung sparing surgery was performed without difference between the groups. Symptoms are significantly associated with older age, location in the upper lobe, and lobar emphysema. Length of stay in intensive care, postoperative complications, and reintervention rate were higher in the symptomatic group. Eighty-one asymptomatic patients underwent surgery <6 months of life; they had a lower rate of surgical complications (2%) compared with those >6 months (7%).

Conclusions: The present study describes a comprehensive picture of CLM. In addition, we emphasize the role of early postnatal management and thoracoscopic surgery, also before 6 months of life, to prevent the onset of symptoms that are associated with worse outcomes.

Keywords: Congenital lung malformation; congenital pulmonary airway malformation; lung-sparing surgery; thoracoscopy.