Hand Function in Apert Syndrome

Abstract

Background: 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. Methods: 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. Results: 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. Conclusions: 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.

Functionality Assessment of Patients With Cleft Hands

“…The bMHQ focuses on functionality, sensibility, pain, and hand appearance. In Brazil, children do not begin learning to read until the age of 7, and for this reason, Group 1 was limited to children who were 7 years old or younger 20,21 . Information regarding outcomes for this age group is based on parent-reported outcomes.…”

Management of Paronychia in Patients With Apert Syndrome

“…1). 1,10,15,16 The normal anatomic nail plate is bordered by the eponychium, hyponychium, and perionychium. Paronychia is one of the most common hand infections and involves the proximal and lateral nail folds.…”

Discussion of “Does the Mutation Type Affect the Response to Cranial Vault Expansion in Children With Apert Syndrome?”

“…This is particularly true in relation to the treatment of patients with Apert syndrome hands, as we can achieve 5-digit hands in 2 operations for all patients with Upton type I and II hands, and 5-digit hands in 3 operations in 72.7% of patients with Upton type III hands. 12,13 Considering that the Pro253Arg mutation is associated with more severe syndactyly, 14 the type of genetic mutation involved determines not only the number of separate surgical procedures but also potential hand function and long-term outcomes.…”