Surgical Management Principles for Congenital Upper Limb Malformations

Abstract

It takes a professional lifetime to get a feel for managing congenital upper limb malformations. This is because each deformity is unique – even the two sides in bilateral cases differ. Furthermore, the special investigations to evaluate more deeply than the obvious phenotype are in fact no more than a shadow; for example, radiographs only cast a shadow of calcified tissue (bone), and therefore the full picture cannot be appreciated. Cartilaginous parts and soft tissues that are also always involved cannot be visualized by radiographs. In other words, all tissues must be assessed and dealt with simultaneously.

The overriding principle when planning or suggesting a management protocol is to always test it against the two fundamental rules: First, and most important, will the treatment improve function? Second, will it improve the cosmetic appearance? Special investigations are essential, especially in the more complex deformities. Apart from the gold-standard radiographic image, computed tomography (CT) and three-dimensional (3D) imaging, high-definition sonar (static and video), and nuclear magnetic resonance imaging (MRI) prove to be very informative, particularly regarding the soft tissue aberrations and extent of soft tissue masses. Recently, 3D printing or additive manufacturing has been a major development to create a 3D model of the anatomical variation, which helps to visualize the deformity. This technique is also used to manufacture implants, build missing anatomical parts, and design orthotics and prosthetics.