ABSTRACT
Limitation of joint motion is characteristic and contractures may develop at the hips, knees, shoulders, elbows and fingers. The claw-hand deformity may be identical to that of the Hurler patient. The hands tend to deviate in an ulnar direction. There is a dorso-lumbar kyphosis and a lumbar gibbus. The abdomen is protuberant and may contain a pronounced diastasis recti and an umbilical hernia. Inguinal hernias are common in males. Hepatomegaly is minimal, and splenomegaly is slight or absent.
