Perioperative multidisciplinary rescue of a patient with Erdheim - Chester disease and multi-system involvement: a case report

Erdheim - Chester disease (ECD) is a rare, slowly progressive non-Langerhans cell histiocytosis that affects bones and multiple viscera. In 2016 the World Health Organization classified ECD as a distinct entity within the histiocytic neoplasm family. Fewer than 1500 cases have been documented in the international literature and the ECD Global Alliance registry. We report a 49-years-old man with biopsy-proven Erdheim - Chester disease (ECD) who developed acute suppurative appendicitis. After systematic evaluation he underwent laparoscopic appendectomy under general anesthesia and, because of multi-organ ECD involvement, was transferred to the intensive care unit (ICU). A multidisciplinary regimen of lung-protective ventilation, continuous renal-replacement therapy, broad-spectrum antibiotics, granulocyte colony-stimulating factor and glucocorticoids was instituted, leading to stable respiratory and renal function; he was discharged to the hematology ward on post-operative day 10. ECD patients are prone to peri-operative deterioration due to pulmonary infiltration, refractory hypoxemia, acute kidney injury and immune paralysis; successful outcome depends on coordinated multidisciplinary care, precise hemodynamic and volume management, early continuous renal replacement therapy (CRRT) and immunomodulation.