Educational aimsTo recognize that exogenous lipoid pneumonia (ELP) in children resulting from cultural and medical practices is a rare albeit preventable interstitial lung disease. To review the clinical-radiological patterns of paediatric ELP which vary widely making this diagnosis indistinguishable from other causes of persistent pneumonia. To describe the association of non-tuberculous mycobacteria (NTM) infections and paediatric ELP resulting in significant comorbidity. To rank studies on ELP in children in the existing literature based on diagnostic certainty and propose standardized case definitions and consistent reporting of treatment outcomes and efficacy.
a b s t r a c tObjectives: To describe the clinical-radiological-pathological characteristics and treatment outcomes of children with suspected exogenous lipoid pneumonia (ELP). Design: Systematic review. We searched electronic databases and reference lists published between 1967 and 2018, restricted to non-accidental cases. Results: Forty-four studies including 489 participants aged 1 day to 17 years from 13 countries were included. Cultural, medical, and behavioural rationale for oil-use was described. The clinicalradiological presentation varied widely. Diagnostic certainty was deemed highest if ELP was confirmed on bronchoalveolar lavage/frozen section lung biopsy with documented extracellular lipid on cytological staining and/or fat analysis. Non-tuberculous mycobacteria infection was identified in six studies: Mycobacterium fortuitum/chelonei, Mycobacterium smegmatis and Mycobacterium abscessus. Treatment comprised supportive therapy, corticosteroids, stopping oil, therapeutic lung-lavage and surgical resection. Outcomes were reported inconsistently. Conclusion: Paediatric ELP resulting from cultural and medical practices continues to be described globally. Preventive interventions, standardized reporting, and treatment efficacy studies for cases not averted, are lacking.Protocol registration: PROSPERO CRD42017068313.