Apert syndrome is a relatively uncommon condition that is instantly recognizable on the basis of the pan-
syndactylies involving both the hands and feet. For more than 10 years, the treatment of
Apert syndrome hand and foot anomalies was approached in a comprehensive manner, with attempts to maximize the final results and minimize the total number of operations. Numerous conventions were abandoned in the development of this approach, with the introduction of some new methodologies, including (1) release of all 10 fingers, and toes, in only two operations, (2) elimination of routine digital
amputations, (3) abandonment of the zigzag incision in favor of straight-line release, (4) substitution of equal-length anterior and posterior flaps for the long dorsal web space lining flap, (5) leaving of small areas of exposed bone without vascularized tissue coverage, and (6) performance of midphalangeal
osteotomies, among older children, to improve hand function. Fifty-seven children with
Apert syndrome have been treated at the author's center since 1990, and 43 underwent surgical treatment of their hands and feet by a single surgeon. Treatment can be separated into two phases, early (
syndactyly releases) and late (functional
osteotomies). Seventeen of those 43 patients were treated at the author's center from birth (type I, 11 patients; type II, two patients; type III, four patients), and 10 fingers and 10 toes were achieved for all patients in two operations. No digital
amputations were performed for any of the 43 patients. However, four of 26 patients (15 percent) not treated at the author's center from birth had undergone at least one digital
amputation before coming to the center. Twenty-two of those 26 patients required a two-stage
syndactyly release to accomplish the separation of all 10 fingers and toes. Aside from the patients who had previously undergone
amputations, all other patients successfully achieved 10 fingers and toes, except for one patient (38 of 39 patients, 97 percent). The average
operative time for the first-stage
syndactyly release of the hands and feet was 4 hours 11 minutes (range, 185 to 300 minutes), and that for the second stage was 3 hours 49 minutes (range, 160 to 300 minutes). Twenty-eight hands were treated with functional
osteotomies, which involved permanent angulation of the fused phalanges at the proximal interphalangeal level, to create a "position of function" and provide pulp-to-pulp pinch. In addition, targeted
osteotomies of the feet were performed for many of these patients, to decrease
pain with walking. There were no major complications. Minor complications included one reexploration because of
bleeding, early in the series. There were 13 incomplete secondary
syndactylies that required a subsequent operative release (10 spaces treated at the author's center and three treated elsewhere), of a total of 342 spaces (3 percent author incidence). There were no
scar contractures or functional limitations that required release. This distinctive protocol seemed to result in improved functional outcomes, with a reduction in the total number of operative procedures.