The three variants of Maroteaux-Lamy syndrome
(mucopolysaccharidosis type VI) are severe, intermediate, and mild. The
severe form of this condition is similar to the severe form of Hurler
syndrome, except for the preservation of intelligence in these patients.
Other clinical features include macrocephaly, coarse facial features, and short
stature. Corneal opacities are often present. Stubby fingers, joint
restrictions, clawhands, lumbar lordosis, and hip pain occur after age 3 or
4 years.