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Introduction <p>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.</p> Case outline <p>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.</p> Conclusion <p>Encountering rare presentations of already uncommon disorders highlights the essential role of diagnostic modalities in today’s rapidly evolving medical practice.</p>

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From agenesis to hypoplasia: a comprehensive case series of congenital pulmonary under-development

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  • 出版模式：

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  • 发表日期：

    2026年2月23日

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  • Marya Hameed,
  • Madeeha Hassan

摘要

Introduction

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.

Case outline

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.

Conclusion

Encountering rare presentations of already uncommon disorders highlights the essential role of diagnostic modalities in today’s rapidly evolving medical practice.