Abstract

Pulmonary underdevelopment is a rare congenital disease which manifests as persistent hemithorax opacification at chest radiography. We present three patients with different types of pulmonary underdevelopment, their imaging features and associated anomalies. Case 1 is a premature neonate with persistent respiratory distress. Further imaging confirmed right pulmonary hypoplasia, associated with a patent foramen ovale, patent ductus arteriosus and vertebral anomalies. Case 2 is a 6-year-old child with corrected anorectal malformation, and recurrent pneumonia. Further imaging confirmed left pulmonary aplasia, associated with an aberrant right subclavian artery and vertebral anomaly. Case 3 is a full term neonate who developed excessive drooling of saliva and respiratory distress. Further imaging confirmed right pulmonary agenesis, associated with an atrial septal defect, patent ductus arteriosus and tracheo-oesophageal fistula. Pulmonary underdevelopment is classified into three types: hypoplasia, aplasia and agenesis. The majority of them have associated anomalies. This condition should be considered a differential diagnosis in paediatric patients with an opaque hemithorax on chest radiography.

Highlights

  • Pulmonary underdevelopment is a spectrum of rare malformations, consisting of agenesis, aplasia and hypoplasia.[1]

  • The incidence of pulmonary agenesis and aplasia is between 0.0034% and 0.0097%,2 whilst the true prevalence of pulmonary hypoplasia is unknown.[3]

  • Case 1 would be classified as hypoplasia, case 2 as aplasia, and case 3 as agenesis

Read more

Summary

Introduction

Pulmonary underdevelopment is a spectrum of rare malformations, consisting of agenesis, aplasia and hypoplasia.[1]. CTA thorax (Figure 2) revealed reduced left hemithoracic volume, a hyperexpanded right lung and total mediastinal shift to the left. CXR revealed complete opacity of the right hemithorax, ipsilateral mediastinal shift, contralateral lung hyperinflation and looping of the feeding tube at mid upper thoracic level. CTA thorax (Figure 3) revealed total absence of the right lung, right main bronchus and right pulmonary vessels in keeping with pulmonary agenesis. She displayed failure to thrive despite having a good appetite

Discussion
Findings
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call