Anesthetic Management for a Patient with Rosai-Dorfman Disease, Cowden Syndrome, and Lhermitte-Duclos Disease: An Extremely Rare Disease Combination

Rosai-Dorfman disease (RDD) is a rare condition that causes massive lymphadenopathy, most commonly in the cervical area. Cowden syndrome (CS) causes hamartomas in the skin and mucosa and predisposes individuals to various malignancies. Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma, is often associated with CS. A 41-year-old female with all three conditions presented with abnormal uterine bleeding and endometrial intraepithelial neoplasia (EIN). Precautions should be considered when evaluating patients with RDD and CS preoperatively and during airway management owing to the potential for multisystem involvement, anatomical distortion, and difficult airways. The likelihood of having all three conditions is extremely rare.