{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T12:25:05Z","timestamp":1787574305284,"version":"build-2736575974"},"reference-count":9,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T00:00:00Z","timestamp":1787529600000},"content-version":"vor","delay-in-days":235,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001782","name":"University of Melbourne","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001782","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Pediatrics"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Kaposiform lymphangiomatosis (KLA) is a rare and aggressive lymphatic anomaly characterised by chylous effusions, respiratory failure and poor prognosis. Diagnosis is often challenging and delayed due to the mosaic distribution of causative somatic mutations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>\n                      A previously well four\u2010year\u2010old girl presented with pleural and pericardial effusions and methicillin\u2010sensitive\n                      <jats:italic>Staphylococcus aureus<\/jats:italic>\n                      bacteraemia. She was treated with intravenous antibiotics, and chest and pericardial drains were inserted. Despite these measures, she developed high\u2010volume, refractory chylous effusions. Management included dietary modification, as well as trials of octreotide and intravenous methylprednisolone, neither of which reduced chyle output. Magnetic resonance lymphangiography demonstrated diffuse abdominal and thoracic lymphatic abnormalities. She was subsequently treated with sirolimus, which was associated with reduced pleural and pericardial chylous drainage but ongoing clinical deterioration. Cell\u2010free DNA analysis of pleural fluid identified a somatic NRAS variant (NM_002524.5 (NRAS):c.182A\u2009&gt;\u2009G (p.Gln61Arg)). In the absence of histopathology, a presumed diagnosis of NRAS Q61R\u2010driven KLA was made based on clinical, radiologic and molecular findings. She was started on trametinib, a MEK inhibitor. Over the subsequent 7\u2009weeks, she was weaned from respiratory support and parenteral nutrition, transitioned to a fat\u2010containing diet and experienced resolution of clinically significant effusions, allowing discharge home.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights the diagnostic challenges of complex lymphatic anomalies, demonstrates the utility of cell\u2010free DNA analysis from effusions in identifying pathogenic somatic variants and underscores the therapeutic potential of targeted MEK inhibition in NRAS Q61R\u2010driven KLA.<\/jats:p>\n                  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