Hand Function in Apert Syndrome

The Michigan Hand Questionnaire is widely used to assess hand outcomes in congenital hand deformities. The purpose of the present study is to compare Apert syndrome hand outcomes according to Upton hand type and age stratification with age-matched unaffected controls.

The Brief Michigan Hand Questionnaire was administered to 39 Apert patients after completion of the digit separation surgical regimen, and 140 age-matched unaffected controls. Patients were divided into 3 groups according to age. In group 1 (from 4 months to 7 years of age), responses were provided by parents of Apert patients, and in group 2 (from 8 to 17 years of age), patients responded with assistance from their parents, and in group 3 (18 years of age or older), responses were provided by the patients themselves. Groups were substratified according to Upton hand type (type I, II, and III). Comparisons were made among groups, subgroups, and controls.

Comparisons of hand types for intragroups 1, 2, and 3, did not demonstrate any statistically significant differences (P > 0.05) between hand outcomes according to Upton hand type, regardless of patient age. Comparisons between Apert patients and their age-matched controls demonstrated statistically significant differences (P < 0.05), as the control group had higher outcome scores.

Similar hand outcomes scores were achieved by all Apert patients regardless of hand type. Following completion of the digit separation regimen, Apert patients presented hand outcome scores that were lower than those of the patients in the normative control group.