Childhood Interstitial Lung Disease Standardised Operating Procedure – Lung Biopsy

This information paper, produced by Lung Foundation Australia in collaboration with chILDRANZ, provides a detailed standardised operating procedure for conducting lung biopsies in children with suspected childhood interstitial lung disease (chILD). Aimed at respiratory physicians, surgeons, radiologists and pathologists, the document outlines best practice for surgical lung biopsy as a diagnostic tool when clinical presentation is unclear or not responding as expected.

The paper highlights the critical importance of a multidisciplinary team (MDT) approach prior to biopsy, ensuring that appropriate lung regions are selected for sampling and that tissue is handled optimally for diagnostic yield. The guidance recommends wedge lung biopsy over less invasive methods like transbronchial biopsy, which offer lower diagnostic accuracy due to limited sample quality. It also outlines potential risks associated with thoracic surgery, particularly in children with respiratory compromise, and the limitations of post-treatment or late-stage biopsies.

The document includes step-by-step protocols for surgical procedures, anatomical pathology processing, and laboratory handling of tissue samples. Key instructions are provided for microbiology, electron microscopy, frozen section, RNA analysis and immunohistochemistry to detect a range of rare conditions including surfactant protein disorders, inborn errors of metabolism, and pulmonary interstitial glycogenosis. A minimum tissue size of 10x10x10mm is recommended for adequate analysis, and specific techniques are described for fixation, staining and specimen labelling.

The pathology report should be integrated with clinical and imaging findings, guided by paediatric pathology expertise. The paper also includes protocols for the handling of explanted lungs and autopsy specimens, and references internationally recognised guidelines to support diagnostic consistency. This evidence-based, co-designed resource supports accurate diagnosis of chILD, enhances clinical decision-making and contributes to improved long-term outcomes for children with diffuse lung diseases. It is an essential reference for health professionals involved in paediatric respiratory diagnostics and tissue management, improving biopsy quality and patient care outcomes across Australia.