Algorithm for treatment of apert hand.
The hand in Apert syndrome is one of the most complex examples of congenital deformity of the upper limb. The management is difficult, and mny different approaches have been published. The hands demonstrate many disturbances of soft tissue and bony structures. These include a short thumb with radial clinodactyly, complex syndactyly with a bony fusion involving the index, long and ring fingers, and symphalangism and simple syndactyly of the fourth web space. The soft tissue anomalies involve the intrinsic muscles, the extrinsic tendon insertions, and the neurovascular bundles. Correction of the appearance of the operated hand is readily apparent, but the complexity of the disorders in the bones and soft tissues explains the difficulty of the surgical management. The aim of this study is to propose a better surgical management: on the basis of the experience of our multidisciplinary team (188 procedures in 53 patients) in the light of recent publications and a better comprehension of the syndrome, we attempt to reduce the number of procedures and to select the best possible procedures for each patient. When possible, we perform a 3-step procedure (the first is bilateral, the others are unilateral) between 9 months and 2 years of age. Separation of the fingers improves function even though we must expect an inevitable stiffness in extension of the interphalangeal joints.
Guero
Stéphane J
SJ
Consultant Surgeon, Plastic and Reconstructive Surgery Hôpital Necker-Enfants Malades Paris, France. sguero@ifcm.org
D000168:Acrocephalosyndactylia / Q000145:classification / Q000175:diagnosis / Q000601:surgery*; D000293:Adolescent; D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019637:Orthopedic Procedures / Q000009:adverse effects / Q000379:methods; D012086:Reoperation