Brachydactyly

Description:

Brachydactyly (brachydactylia; brakhi-+ Greek daktylos — a finger; synonym: a korotkopalost) — anomaly of development of hands or legs, shortening of fingers. The brachydactyly can often be combined with a simfalangizm and with various forms of a syndactylia.

Brachydactyly symptoms:

The shortening of fingers caused by an underdevelopment of phalanxes or metacarpal (metatarsal) bones is typical. There is the following classification of brachydactylies. The type A is characterized by shortening of fingers at the expense of average phalanxes. Allocate subgroups of a brachydactyly of type A.

At the A1 type (type of Farabi) all average phalanxes are affected, they rudimentary and sometimes merge with trailer phalanxes. Proximal phalanxes of the first fingers of brushes and feet are shortened, the growth inhibition is sometimes noted.

The A2 type (a brakhimezofalangiya, type Mora — Vrita) is characterized by shortening

average phalanxes of the second fingers of hands and legs at more or less safe other fingers. Because of rhomboid or triangular shape of the affected average phalanxes the second fingers are rejected radially.

At the A3 type (a brakhimezofalangiya of the V finger, a clinodactyly) only average phalanxes of the fifth fingers of brushes with their radial curvature are shortened.

The A4 type (a brakhimezofalangiya II and V fingers, Temtami's type) — II and V fingers of a brush are affected.

The A5 type is characterized by lack of average phalanxes of the II—V fingers with a dysplasia of nails.

At type In average phalanxes are shortened, as well as at type A, but besides there are no trailer phalanxes of fingers of hands and legs, the union II and III fingers is observed.

Type C — average and proximal phalanxes II and III fingers, sometimes with hypersegmentation of a proximal phalanx are shortened, the symphalangism and shortening of metacarpal bones are possible, the low growth and mental retardation are sometimes noted.

At type D shortening of the first fingers of brushes and feet (brakhimegalodaktiliya) is observed. Type E is characterized by shortening of metacarpal and metatarsal bones. The number of the affected fingers varies even within one family. Population frequency — 1,5:100000. Types A, and D meet most often.

Ratio of floors — M1:Zh1.

Brachydactyly reasons:

The brachydactyly is inherited on autosomal dominantly type.

Treatment of the Brachydactyly:

Surgical (division and lengthening of fingers).