Type:
Educational Exhibit
Keywords:
Paediatric, Respiratory system, CT, Plain radiographic studies, Education, Other
DOI:
10.26044/ranzcr2022/R-0198
Imaging findings OR Procedure details
Presented here is a case of paediatric PAP in a 9-year-old male. He initially presented to his general practitioner with an 18-month history of poor weight gain, exertional chest pain and worsening dyspnoea. After a pectus carinatum deformity was noted on examination he was referred to his local paediatric hospital. A chest x-ray revealed widespread pulmonary infiltrates (Figure 1). Subsequent CT chest (Figure 2) showed interstitial and alveolar pulmonary infiltrates throughout the right upper, middle and lower lobes and left upper lobe with ground glass opacities and interlobular septal thickening – i.e. "crazy paving" pattern. The diagnosis of PAP was confirmed with bronchoalveolar lavage producing a thick, milky sample, later demonstrated to be periodic acid-Schiff (PAS) positive. Following this a thorough investigation for primary and secondary causes of PAP noted a pathogenic deletion variant in the CSF2RA gene. Regular bronchoalveolar lavages were pursued for the management of his condition.