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Scheie syndrome (MPS‐IS) presented as bilateral trigger thumb

storage disease caused by a deficiency of the enzyme α-Liduronidase.1 Clinical manifestations include mild Hurlerlike dysmorphia, corneal clouding, hepatosplenomegaly, cardiac involvement and joint stiffness, which appear in childhood. Height and intelligence are usually normal and because of its phenotypic mildness in infancy, diagnosis without a family history is usually delayed until adolescence. Here, we report a 3-year-old boy who was diagnosed with Scheie syndrome by histological examination of the tendon sheath obtained from bilateral trigger thumb surgery and subsequent biochemical analysis. Preoperatively, he had no symptoms except for triggering of the thumbs, which was noticed at 3 years of age. To our knowledge, this is the first case in which thumb triggering was the clue for early diagnosis of Scheie syndrome.