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Hemifacial microsomia

Hemifacial microsomia is a congenital disorder that effects the development of the lower half of the face, most commonly the ears, the mouth and the mandible. It can occur on one side of the face or both. If severe it can lead to difficulties in breathing, obstructing the trachea and requiring a tracheotomy. It is the second most common facial birth defect after clefts, with an incidence in the range of 1 in 3500 to 4500 . more...

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Other names

The condition is also known by various other names:

  • Lateral facial dysplasia
  • First and second brachial arch syndrome
  • Oral-mandibular-auricular syndrome
  • Otomandibular dysostosis
  • Craniofacial microsomia

Cause

The condition develops during the fetal stage of pregnancy when some form of vascular problem leads to clotting and a poor supply of blood to the face. This is usually caused by a physical trauma, though there is some evidence of it being hereditary . This restricts the developmental ability of that area of the face.

Severity

The severity of hemifacial microsomia depends on the extent of the physical injury (the area with a short supply of blood) and the gestational age of the fetus – the earlier the injury, the greater the chance of wide-scale problems.

In some cases, the central nervous system is affected, causing difficulties moving the face symmetrically.

Treatment

Depending upon the treatment required, it is sometimes most appropriate to wait until later in life for a surgical remedy – the childhood growth of the face may highlight or increase the symptoms. When surgery is required, particularly when there is a severe disfiguration of the jaw, it is common to use a rib graft to help correct the shape.

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New ears will stop playground taunts
From Evening Standard (London), 10/2/98 by Jo Revill

LONDON doctors have developed model plastic ears which could spare the blushes of children born with ones that are undersized or malformed.

Children as young as eight are set to benefit from the computer techniques which create and position perfect ear copies for young skulls.

The technique is being used to help scores of children who are disfigured by a condition known as hemifacial microsomia - a deformity which affects between one in 3,500 and one in 10,000 children. Scientists working together at King's College and University College London have been able to make exact plastic copies of young children's ears by using magnetic resonance imaging and laser scans with a group of 24 patients. This information is downloaded into a computer which devises a perfect copy of a normal ear, and also sets out exactly where the copy should be fitted to the side of the head. "This condition is very psychologically disturbing for both parents and the children," said Roger Watson, a professor of prosthetic dentistry at King's College who has been working on developing the procedure. "Often the skull or the jaw is also small, so they may have had to have other operations. This enables them to have an artificial ear without further plastic surgery." In the past, children were often fitted with artificial plastic ears which were fashioned from hand-carved wax models. The copies depended on the modelling skills of individual surgeons. The artificial ears, developed with grants from the charity Action Research, are attached to titanium skull implants which cannot be seen once the ears are in place.

Copyright 1998
Provided by ProQuest Information and Learning Company. All rights Reserved.

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