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Outcome of infants operated on for congenital pulmonary malformations
Flaminia Calzolari1, Annabella Braguglia1, Laura Valfrè2
1Department of Medical and Surgical Neonatology, Neonatal Intensive Care Unit, Bambino Gesù Children's Research Hospital IRCCS, Piazza S. Onofrio, 4 00165, Rome, Italy.
Insights
Over half of patients who undergo surgery for congenital pulmonary malformations (CPM) experience long-term health issues. Regular follow-up care is crucial for monitoring these children after surgery for CPM.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Congenital Anomalies
Background:
- Congenital pulmonary malformations (CPM) are anomalies affecting lung development.
- While survival rates after surgical correction are high, long-term morbidity remains poorly understood.
- Understanding sequelae is vital for comprehensive patient care.
Purpose of the Study:
- To report on the long-term sequelae in patients operated on for CPM during infancy.
- To identify factors influencing the outcomes of these patients.
- To establish the necessity of long-term follow-up protocols.
Main Methods:
- Prospective data collection from a dedicated outpatient program for congenital anomaly patients.
- Comparison of CPM patients (n=76) with a control group (inguinal hernia repair, n=68) for auxological, respiratory, and orthopedic outcomes.
- Evaluations at multiple time points: 6, 24, 48 months, and school ages (4, 6, 8, 12 years).
Main Results:
- 78% of CPM patients exhibited clinical or radiological abnormalities at follow-up, compared to 16% in the control group.
- A significantly higher incidence of sequelae was observed in the CPM cohort (OR 16.5, P < 0.0001).
- No significant difference in age at follow-up between CPM and control groups.
Conclusions:
- Over 50% of patients with CPM experience long-term sequelae, irrespective of the specific malformation.
- Long-term follow-up is strongly recommended for all patients operated on for CPM.
- Further research is needed to determine if surgical intervention impacts the natural history in asymptomatic cases.
Introduction:
Patients operated on for congenital pulmonary malformations (CPM) have excellent survival rates, but little is known about long-term morbidity. Our aim is to report the sequelae in patients operated on for CPM in infancy and to define factors that may influence their outcome.
Methods:
All patients operated on for major congenital anomalies are followed in a dedicated outpatient program and evaluated at 6, 24, and 48 months of life (corrected for gestational age) and at school age at 4, 6, 8, and 12 years of life. The data are prospectively collected. Patients operated on for CPM and enrolled in the follow-up clinic between January 2004 and December 2010 are compared with a control group of term infants operated on for inguinal hernia, without other major congenital or acquired abnormalities. The two groups were compared for auxological, respiratory, and orthopedic outcome.
Results:
In the study period, 76 consecutive patients with CPM attended our dedicated follow-up clinic. Eight non-operated patients were excluded from the study. Age at follow-up was 82.0 (56.1-103.7) months in CPM patients and 83.5 (75.2-90.4) months in controls (P = 0.79). Fifty-three patients with CPM (78%) had one or more clinical or radiological abnormality versus six (16%) control patients (OR [95%CI] 16.5 [5.8-47.2]; P < 0.0001).
Conclusions:
Over 50% of patients with CPM present long-term sequelae, regardless type of malformation. Therefore, long-term follow-up of patients operated on for CPM is recommended. Further studies are needed to define if, in asymptomatic patients, surgery may modify the natural history of CPM. Pediatr Pulmonol. 2016;51:1367-1372. © 2016 Wiley Periodicals, Inc.
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