Development of the Upper Limb-Amelia-Congenital Absence of the Radius-Syndactyly-Lobster Hand-Brachydactyly-Floating Thumb-Polydactyly-Local Gigantism

Development of the Upper Limb

The limb buds appear during the sixth week of development as the result of a localized proliferation of somatopleuric mesenchyme. This causes the overlying ectoderm to bulge from the trunk as two pairs of flattened paddles. The arm buds develop before the leg buds and lie at the level of the lower six cervical and upper two thoracic segments. The flattened limb buds have a cephalic preaxial border and a caudal postaxial border. As the limb buds elongate, the anterior rami of the spinal nerves situated opposite the bases of the limb buds start to grow into the limbs.

The mesenchyme situated along the preaxial border becomes associated and innervated with the lower five cervical nerves, whereas the mesenchyme of the postaxial border becomes associated with the 8th cervical and 1st thoracic nerves.

Later, the mesenchymal masses divide into anterior and posterior groups, and the nerve trunks entering the base of each limb also divide into anterior and posterior divisions. The mesenchyme within the limbs differentiates into individual muscles that migrate within each limb. As a consequence of these two factors, the anterior rami of the spinal nerves become arranged in complicated plexuses that are found near the base of each limb so that the brachial plexus is formed.

Amelia

Absence of one or more limbs (amelia) or partial absence (ectromelia) may occur. A defective limb may possess a rudimentary hand at the extremity of the limb or a well-developed hand may spring from the shoulder with absence of the intermediate portion of the limb (phocomelia) .

Congenital Absence of the Radius

Occasionally, the radius is congenitally absent and the growth of the ulna pushes the hand laterally.

Syndactyly

In syndactyly, there is webbing of the fingers. It is usually bilateral and often familial. Plastic repair of the fingers is carried out at the age of 5 years.

Lobster Hand

Lobster hand is a form of syndactyly that is associated with a central cleft dividing the hand into two parts. It is a heredofamilial disorder, for which plastic surgery is indicated where possible.

Brachydactyly

In brachydactyly, there is an absence of one or more phalanges in several fingers. Provided that the thumb is functioning normally, surgery is not indicated .

Floating Thumb

A floating thumb results if the metacarpal bone of the thumb is absent but the phalanges are present. Plastic surgery is indicated where possible to improve the functional capabilities of the hand.

Polydactyly

In polydactyly, one or more extra digits develop. It tends to run in families. The additional digits are removed surgically.

Local Gigantism

Macrodactyly affects one or more digits; these may be of adult size at birth, but the size usually diminishes with age. Surgical removal may be necessary.