Frontiers in Reconstructing the Apert Hand: Synostosis Release, Phalangeal Osteotomies, and Distraction Lengthening
Jonah I. Donnenfield*, Laura C. Nuzzi, Amir Taghinia, Brian I. Labow
Department of Plastic and Oral Surgery, Boston Children's Hospital/Harvard Medical School, Cambridge, MA
Apert syndrome is a rare autosomal dominant condition affecting up to 1:65,000 live births and is attributable to a mutation in the fibroblast growth factor 2 gene. Affected individuals feature craniosynostosis, midface hypoplasia, and complex bilateral syndactyly of the hands and feet. The literature on Apert hand treatment largely focuses on syndactyly release, usually in the first 2-3 years of life. The purpose of this investigation is to explore if an extended treatment model for the Apert hand"”using specific, uncommonly performed interventions after syndactyly release"”can benefit patients during periods of skeletal maturation and beyond.
Following division of the Apert hand into five digits, we detail the techniques and benefits of metacarpal synostosis release, corrective phalangeal osteotomies, and thumb distraction lengthening.
In the years following syndactyly release, the Apert hand usually develops fusion between the fourth and fifth metacarpals, resulting in extreme deviation of the fifth digit and functional challenges for the patient. Metacarpal synostosis release involves osteotomy of the fused metacarpal and pin placement to correctively rotate the deviated digit. Also following syndactyly release, other forms of digit deviation (e.g., fingertip deviation) arise between the ages of 2 and 6. These can be functionally and cosmetically ameliorated with surgical straightening and wire placement. The Apert thumb may also be shortened and radially deviated, which presents functional challenges as the thumb is thought to comprise about 60% of hand function. Distraction lengthening has been therapeutically validated in moderate-sized patient cohorts and involves two stages: osteotomy and distractor placement followed by bone grafting and fixation.
We present several validated treatment options for managing the Apert hand following syndactyly release, for which there is little literature documentation. These innovative techniques have been successfully performed by the senior author and provide improved function and appearance for Apert patients.
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