Cleft Hand

Cleft Hand

Cleft hand is a central longitudinal congenital deficiency. This article details the aetiology, features, classification and treatment.

Summary Card


Definition
A congenital central longitudinal deficiency affecting hands and feet.

Clinical Picture
A typical cleft involves soft tissue, phalanges, metacarpals, webspace and syndromes.

Classification
The Manske and Haliki classification assesses the degree of first webspace deficiency.

Management
A multi-disciplinary team to achieve web space reconstruction, syndactyly release, thumb pollicisation, and cleft closure.

Other Types
Symbrachydactyly and phocomelia are other variants of a central longitudinal deficiency.


Definition of Cleft Hand

The Cleft hand is a congenital central longitudinal deficiency characterised by a V-shaped cleft in the centre of the hand due to missing digits.


Clinical Picture of Cleft Hand


Key Point

A typical cleft hand can be remembered by the mnemonic: CLEFTS. It involves soft tissue, phalanges, metacarpals, webspace and syndromes.

The features of a typical cleft hand can be remembered by the mnemonic: CLEFTS (a P'Fella original).

  • Central deficiency of fingers and/or toes which creates a "V-shape"
  • Loss or narrowing of webspace
  • Epiphyses of phalanges are longitudinally bracketed or duplicated.
  • Family history (autosomal dominant)
  • Transverse metacarpals
  • Syndromic, such as EEC (ectrodactyly, ectodermal dysplasia, facial clefts).

These clinical features of a cleft hand are illustrated below.

Features of a cleft hand

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Fun Fact: "Atypical" cleft hand is unilateral, sporadic and associated with Poland Syndrome. It is also referred to as symbrachydactyly. 


Classification of Cleft Hand


Key Point

The severity of a cleft hand is classified by the degree of first webspace deficiency.


The Manske and Haliki classification assesses the degree of first webspace deficiency.

  • Type I: normal
  • Type IIa: mildly narrowed
  • Type IIb: severely narrowed
  • Type III: thumb-index syndactyly
  • Type IV: Index ray suppressed, first webspace merged with cleft
  • Type V: thumb suppressed, absent first webspace.  

This classification of cleft hand is summarised in the table below.

Classification of Cleft Hand


Management of Cleft Hand


Key Point

Cleft hands can be treated through a multi-disciplinary team. Surgery focuses on webspace reconstruction, syndactyly release, thumb pollicisation, and cleft closure.

The management of a cleft hand is through a multi-disciplinary approach.

It is usually performed between the ages of 1-2 but can be earlier if the progressive deformity is caused by transverse bones or syndactyly between digits of unequal length.

The key principles include

The cleft hand can be "closed" through different techniques.

There are two commonly used methods:

  1. Snow-Littler: volar flap from cleft transposed to the first web
  2. Miura-Komada: palmar and dorsal flaps

The Snow-Littler technique can be seen in the video below.


Other Types of Cleft Hand

Symbrachydactyly  

An atypical cleft hand can present with unilateral, short vestigial digits that rarely affect the feet. It is commonly associated with Poland Syndrome.

Blauth and Geleker have classified it based on the following subtypes

  1. Short finger: Thumb + 4 smaller digits; consider phalangeal bone transfer
  2. Oligodactylic: aplastic central hand, consider metacarpal rotation
  3. Monodactylic: Thumb + 4 finger remnants,
  4. Peromelic: No fingers or thumb

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Fun Fact: Unlike cleft hand, a symbrachydactyly generally does not have a family history of a central longitudinal deficiency. 

Phocomelia

Phocomelia is an intersegmental central deficiency associated with thalidomide medication. In this condition, the hand is always present but can exist in various forms, as described below.

  • Type I: hand is attached to the trunk
  • Type II: forearm and hand attached to trunk.
  • Type III: hand is attached to the humerus at the elbow.


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