713 - Congenital Pulmonary Airway Malformation: Prevalence, Demographics, and Associated Clinical Outcomes in the U.S.

Publication Number: 713.4515

Somaya Abuelazm, Cleveland Clinic Children's, Cleveland, OH, United States; Vanishree Nandakumar, Cleveland Clinic Children's Hospital, Cleveland, OH, United States; Stacey Ramey, Cleveland Clinic Children's, Cleveland, OH, United States; Nicole Buzalka, Cleveland Clinic Children's, cleveland, OH, United States; Mohamed A. Mohamed, Cleveland Clinic Children's, Cleveland, OH, United States; Hany Aly, Cleveland Children’s Hospital, Cleveland, OH, United States

Background: Congenital Pulmonary Airway Malformation (CPAM), formerly known as Congenital Cystic Adenomatoid Malformation (CCAM), is uncommon developmental anomaly of the lung occurring in utero with an unknown etiology. It is characterized by the formation of single large or multiple small cysts within the lung parenchyma, leading to suppression of alveolar growth. The neonatal presentation is variable with some cases manifesting as severe respiratory distress at birth

Objective: To describe the prevalence of CPAM, epidemiologic, demographic, associated clinical characteristics, and potential adverse outcomes of infants with CPAM

Design/Methods: The National Inpatient Sample (NIS) datasets produced by the Healthcare Cost and Utilization Project (HCUP) for the years 2016–2020 were utilized for this study. We identified all infants included in the dataset. Extremely low birth weight infants and those with congenital anomalies that could independently impact outcomes, such as central nervous system anomalies, congenital heart disease, congenital diaphragmatic hernia, abdominal wall defects, multiple congenital anomalies, and chromosomal disorders were excluded. Clinical diagnoses were identified using the International Classification of Diseases, Tenth Revision (ICD-10) codes, with CPAM identified using code Q33.0. Chi-square tests were employed to compare frequencies, and logistic regression models were used to calculate adjusted odds ratios (ORs), controlling for confounding factors.

Results: The sample included 18,324,345 infants. Among these, 1,871 infants (0.01% or 0.1 per 1,000 births) had a CPAM diagnosis in their admission records. Table 1 shows the demographic, clinical, and adverse outcomes associated with CPAM in newborn infants. In general, CPAM was less in females and less common in Asian/Pacific Islander populations. Infants with CPAM were more likely to be small for gestational age and to be associated with maternal diabetes mellitus. CPAM was highly associated with significant pulmonary complications; respiratory distress, prolonged mechanical ventilation exceeding 96 hours, pneumothorax, pulmonary hemorrhage, pulmonary hypertension, and anemia. CPAM was not associated with increased rates of sepsis, necrotizing enterocolitis, or acute kidney injury. Mortality among infants with CPAM was significantly higher than in the general population.

Conclusion(s): CPAM is a rare serious condition associated with several respiratory complications and a high mortality rate. Proper antenatal counseling is mandated to ensure the appropriate place of delivery and to arrange for specialized neonatal and surgical care.

Table (1)
Demographic and clinical characteristics, and adverse outcomes associated with congenital pulmonary airway malformation in hospitalized newborns in the U.S. (2016-2020)