It is a term used to describe a spectrum of deformities. Deformities range from small fingers to deficiency of hand or forearm.
It can occur sporadically or as a part of a group of diseases ( syndromes). Poland syndrome is a commonly associated syndrome.
The hand of a child with this, may be divided into 4 types
1. All fingers present ; but they are short - Such hands are managed with release of fused fingers. Some children benefit with transfer of bone from toe ( Free phalangeal bone transfer).
2. The central fingers ( index finger, middle finger and ring finger ) are short to varying extent. In a selected group of children , vascularised toe transfer from foot is beneficial.
3. Only a thumb present - A hand can be functional if it has a minimum of one finger and thumb. In children with only a thumb , the other finger may be recreated by free vascularised toe transfer from foot.
4. Short upper limb : Functional prosthesis are an excellent option in older children.