From agenesis to hypoplasia: a comprehensive case series of congenital pulmonary under-development
Pulmonary underdevelopment is a rare congenital malformation typically identified in the pediatric population, incorporating a spectrum of pulmonary developmental disorders, including agenesis, aplasia, and hypoplasia of the lung.For diagnostic purposes chest x-rays are used as initial modality while, Contrast-Enhanced Computed Tomography and Computed Tomography angiogram are for confirming the diagnosis and specifying the anomaly extent. Right-sided anomaly is rare, often associated with VACTERL anomalies. We observed five cases of pulmonary underdevelopment, with four patients demonstrating right-sided involvement. A series of five pediatric patients underwent Contrast-Enhanced Computed Tomography (CECT) for evaluation of various forms of congenital pulmonary underdevelopment. Two patients, aged 12, and 21 months, had right-sided Type I (pulmonary agenesis). Type II (aplasia) was observed in 1.5 and 21-month-old child, while Type III (hypoplasia) was identified in a nine-month-old with right-sided involvement. Notably, the youngest of these exhibited features of VACTERL association, including a horseshoe kidney and kyphoscoliotic deformity. Encountering rare presentations of already uncommon disorders highlights the essential role of diagnostic modalities in today’s rapidly evolving medical practice.
Authors
- Marya Hameed
- Madeeha Hassan
Institutions
- Jinnah Sindh Medical University (PK)
- National Institute of Child Health (PK)
Publication Details
- Journal
- Egyptian Journal of Bronchology
- Published
- 2026-02-23
- DOI
- https://doi.org/10.1186/s43168-026-00538-y
- Primary Topic
- Congenital Diaphragmatic Hernia Studies
- Type
- article
- Field-Weighted Citation Impact
- 0.00