This paper presents a retrospective review of pediatric patients with Apert’s Syndrome treated at a single institution over a ten-year period, from 1986-1996. The authors included 10 patients, out of a total patient pool of 15, who were treated according to a streamlined protocol. Apert Syndrome, defined as congenital coronal synostosis, midface hypoplasia, and bilateral symmetric complex syndactyly of the hands and feet is an autosomal dominant disease. The authors review the classification schema of Upton (1991) and use it to define their protocol based on type. Type I hands (n=5) presented with complex syndactyly of the index-thru-small fingers and free thumbs. Type II hands (n=1) included simple syndactyly of the thumbs. Type III hands (n=4) had the addition of a complex syndactyly of the thumbs. Outcome was judged by the need for revision surgery, gross grasp and pinch abilities, and patient/parent satisfaction. All patients were treated with release and simple full-thickness skin grafting and local skin flaps. Average age at the first release was 11 months. Eleven thumb osteotomies were performed at an average age of 29 months. Seven of 53 webspaces required revision (13%). No osteotomies/bone grafts required revision. Results showed universal ability of the patients to achieve small object pinch and grasp, as well as thumb opposition. No families expressed dissatisfaction or requested further surgeries. Two of ten families expressed concern about the inability of the children to produce flexion at the interphalangeal joints.
The authors present a rationale for a treatment sequence based on deformity classification. They advocate early initial surgery, in some cases as early as six months of age, to release thumb webs and border digits at an average of 11 months of age. Subsequent surgeries are performed between one and two years of age to sequentially release remaining, non-adjacent, syndactylys and to perform thumb wedge osteotomies and bone grafts when necessary. These later surgeries are performed at intervals of three months. Importantly, the results are five-fingered hands in Types I and II, but four-fingered hands in Type III. This contrasts with more traditional strategies, which aim to produce five separate digits whenever possible. The authors contrast their results with a series by Barot and Caplan (1986) in which the average age at the time of thumb osteotomy was 4.6 years, versus this series’ 29 months. The authors feel that the significant advantage of earlier surgery and completion of reconstruction is reduced cost. However, there is no evidence presented that earlier reconstruction made any positive impact on the patients development, eventual function, or satisfaction. Furthermore, one must consider the complexity of the multi-systemic deficits in these children, the need for associated craniofacial procedures, family concerns and restrictions, and the overall health, development, and maturity of the individual child before prescribing an algorithmic approach to reconstruction of these deformities. Additionally, most families, when given the option, will no doubt prefer a strategy that aims to produce five-fingered hands whenever possible to minimize the already significant cosmetic stigmata of Apert’s Syndrome.
Plastic and Reconstructive Surgery