Neonatal Surgery for Congenital Lung Malformations: Indications, Outcomes and Association With Malignancy

Congenital lung malformations (CLM) are a heterogeneous group of anomalies diagnosed with increasing frequency antenatally. Postnatal management is dictated by the clinical status in the early neonatal period. We report our centre's experience in managing symptomatic neonates with CLM.

Single centre retrospective review (January 1995-December 2023) of all infants with CLM that underwent thoracotomy for resectional surgery during the neonatal period. Data are quoted as median (IQR), unless stated otherwise. SPSS version 28 (IBM) and GraphPad Prism were used for analysis of data. A P value of ≤0.05 was regarded as significant.

During the study period 228 patients underwent surgery for CLM. Forty-nine infants (21.5 %) (33 males) required surgery during the first 30 days of life. Surgery in those infants was performed at 5 (1-10) days (emergency surgery, n = 24; expedited, n = 25). The indications included respiratory distress (n = 29; mechanical ventilation n = 16) and size of lesion (n = 20). Antenatal diagnosis was established in 45 (91.8 %) infants and 18 had undergone antenatal intervention with 7 fetuses having more than one procedure. The CLM was left sided in 29 (59 %). Postoperative stay for the surviving infants was 14 (7-21) days with extubation being achieved after 2 (1-4) days. The overall survival was 91.8 % (45/49). Infants that underwent antenatal intervention had a higher rate of postnatal pneumothorax (P = 0.01) and required higher FiO₂ delivery (P = 0.01). Infants that underwent surgery in an emergency setting were found to have a higher need for antenatal intervention (P = 0.01), postnatally required intubation (P < 0.001) with higher FiO₂ delivery (p < 0.001) and developed pneumothorax (P = 0.002). Histopathology findings showed the entire spectrum of CLM, with 6 cases of CPAM Type 1 associated with mucinous adenocarcinoma. Cross sectional radiological imaging was available for 31 patients and in 4 patients with malignancy, a solid component of the lesion was appreciable on CT. Median follow-up period was 6 (2.1-11) years. Sixteen (35 %) children developed a degree of chest wall deformity through none have required surgical intervention.

This is one of the largest series documenting the postnatal management of symptomatic neonates with CLM. One fifth of the cohort undergoing surgery for CLM required neonatal intervention. Histology at surgery was heterogeneous with 12 % of cases being associated with mucinous adenocarcinoma.