Anatomy and management of the foot in Apert syndrome: A review of the literature

Background: Apert syndrome (acrocephalosyndactyly type 1) is a complex condition characterised by craniosynostosis and syndactyly of the hand and feet [Holten IW, Smith AW, Bourne AJ, David DJ. The Apert syndrome hand: pathalogic anatomy and clinical manifestations. Plast Reconstruct Surg 1997;99:1681-97]. The effect of Apert syndrome on the feet has received little attention in the literature when compared to upper limb abnormalities. Early treatment and advances in the care of other aspects of Apert syndrome (for example craniofacial and ophthalmologic abnormalities) has meant individuals with Apert syndrome may now have increased life expectancy and quality of life, and hence place increased demands on their feet. Objective: To review the most recent literature pertaining to the feet in Apert syndrome and to present the findings along with suggestions for conservative and surgical management supported by the literature. Method: A search of the CINAHL, OVID and MEDLINE databases in addition to the NHS Education for Scotland e-Library Journal database was performed using "Apert" and "Foot" as keywords. The reference lists from the literature produced from those searches were scrutinised. A hand search of other journals (Journal of the American Podiatric Medical Association, American Journal of Medical Genetics, Clinics in Plastic Surgery) was also performed. Results: The main features of the Apert foot are syndactyly, progressive osseous fusions, rigidity, and difficulty with shoe fitting as a result of an abnormally broad great toe. These alterations in foot mechanics cause increased stress on the soft tissues of the foot causing painful calluses and recurrent nail sepsis. Conclusions: Foot pathology in the Apert foot varies greatly, and a multi disciplinary approach to care is to be commended. Radiography ought to be performed to establish the skeletal nature of the Apert foot even if clinical examination suggests typical Apert feet [Anderson PJ, Smith PJ, Jones BM, Hayward RD. Additional metatarsal bones in Apert's syndrome. Foot 1996;6:37-8]. Conservative measures (footwear/orthotic provision, callous debridement) may be used to alleviate pain around areas of high pressure. The decision to operate will most likely be taken in terms of functional rather than cosmetic improvement, although syndactyly release may be psychologically beneficial in terms of social normalisation.