Congenital Hand Deformities
- 1 Introduction
- 2 Etiology
- 3 Epidemiology
- 4 Classification
- 5 Treatment for congenital hand deformities
- 6 Surgical Management of Some Common Congenital Hand Deformities
- 7 Complications
- 8 Physiotherapy
- 9 References
Congenital hand deformities are deformities of the hand that develop in a child before birth. Congenital hand deformities are also known as- congenital hand differences, congenital hand anomalies, congenital hand conditions and congenital hand problems.
Congenital hand deformities usually occur between the fourth and eighth week after the embryo is formed. Failure of the transformation from arm bud cells to the upper limb can lead to an abnormal appearing/functioning upper extremity which is presented at birth. Some causes are linked to genetics while others are affected by the environment, and the rest have remained unknown.
Of the 1% to 2% of newborns that are born with congenital defects, 10% of these are born with upper extremity malformations. Congenital hand deformities affect approximately 1 in 500 persons and present unique diagnostic and classification challenges. In a study, congenital hand deformities were seen 1.5 times in male patients compared with female patients.
1.Malformations: Malformations are abnormalities of formation and/or differentiation of the tissues. Examples are- brachydactyly, symbrachydactyly, syndactyly, polydactyly, cleft hand/ectrodactyly, apert hand etc.
3.Dysplasias: Dysplasias are abnormalities that result from a lack of normal organization of cells into the tissue. Examples- hemihypertrophy, macrodactyly, osteochondromatosis, fibrous dysplasia etc.
Treatment for congenital hand deformities
Specific treatment for congenital hand deformities will be determined based on:
- Age, overall health, and medical history
- Extent of the condition
- Cause of the condition
- Patient's tolerance for specific medications, procedures, or therapies
- Expectations for the course of the condition
- Parents' opinion or preference
Treatment may include:
- Limb manipulation and stretching
- Splinting of the affected limbs
- Tendon transfers
- External appliances
- Physical therapy
- Correction of contractures
- Skin grafts
Surgical Management of Some Common Congenital Hand Deformities
An extra digit or polydactyly is one of the more common congenital hand conditions encountered in clinical practice. The commonest form of polydactyly is that of thumb duplication. In thumb polydactyly, reconstruction is usually offered. Besides the obvious cosmetic improvement, surgery also places the retained digit in a more functional position, and the reconstruction aims to enhance the stability of an often unstable metacarpophalangeal joint. While there is no real upper limit to surgery, most surgeons would prefer to do reconstruction sometime between six months and two years of age.
Syndactyly refers to an abnormal linkage between adjacent digits. Surgery to separate the digits aims to produce a hand with as many stable and functionally independent fingers as possible. Separation is done earlier if the ring and little fingers or the index finger and thumb are involved, as the differential longitudinal growth rates between the digits will lead to bone and joint deformity if left untreated.
Constriction Ring Syndrome
This condition is also known as the amniotic band syndrome or Streeter's disease. It is marked by constrictions or bands around digits or limbs. Release of the constrictions is not urgently required unless there is evidence of vascular compromise distally, such as an oedematous or ischaemic digit. Other techniques of reconstruction, such as toe transfers or lengthening procedures, may be required if the amputations are significant.
Paediatric Trigger Thumb
This condition presents as a flexion deformity of the interphalangeal joint of the thumb. It is more commonly unilateral. Unlike adult trigger thumbs, it does not usually present as triggering per se, but with a flexion deformity. One important differential diagnosis is that of a clasped thumb. In more than 60% of cases, the condition will resolve spontaneously, but this requires about 48 months of waiting. As an alternative, surgical release is highly effective and has limited morbidity.
Many of the congenital hand deformities are relatively minor and do not affect function. However, the appearances of the deformities have significant psychological impact on both the parents and child.
- A considerable amount of therapy is needed, the keystones being stretching and splinting. It is essential to have parental understanding and input.
- Community therapy services are very valuable but close communication with the hospital services is needed to ensure shared objectives and continuity of care. In some conditions therapy needs to start in the neonatal period, for instance radial clubhand, flexed proximal interphalangeal joints lacking extension and the “wind blown” conditions where the thumb or fingers lack extension.
- Treatment plans geared specifically for children, achieving increased independence with activities of daily living,
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