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Hay-Wells Syndrome

Syndrome of birth defects

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Updated October 12, 2008

Cleft lip may be among the symptoms of Hay-Wells syndrome

Cleft lip may be among the symptoms of Hay-Wells syndrome

Photo (c) A.D.A.M.
Hay-Wells syndrome is a form of ectodermal dysplasia, a group of over 100 inherited disorders that cause hair, teeth, nails, and glands to develop abnormally. Hay-Wells syndrome is also known by its clinical name, ankyloblepharon-ectodermal dysplasia-clefting (AEC) syndrome.

Ectodermal dysplasias affect both males and females of all ethnic backgrounds. It is not known exactly how often Hay-Wells syndrome occurs. It is inherited in an autosomal dominant pattern.

Symptoms

The major symptoms of Hay-Wells syndrome include:
  • sparse, coarse, wiry hair
  • small, sparse eyelashes
  • malformed nails
  • missing teeth
  • cleft palate, sometimes with cleft lip
  • abnormal fibrous tissue connecting the upper and lower eyelids

Diagnosis

Since Hay-Wells syndrome is present from birth, diagnosis is based on the physical appearance of an infant or young child. In some cases, the syndrome is not apparent until the teeth fail to develop normally.

There is no specific diagnostic test for the syndrome.

Treatment

Hay-Wells syndrome is an inherited genetic disorder, so treatment focuses on the symptoms present. Surgery can be done to correct cleft palate and cleft lip. Speech therapy may be necessary because of cleft palate and/or missing teeth. Genetic counseling will be helpful for the individual and family affected by Hay-Wells syndrome.

Sources:

"About Ectodermal Dysplasia." National Foundation for Ectodermal Dysplasia 20 Mar 2008.

"Ankyloblepharon-ectodermal defects-cleft lip palate." Rare Diseases. May 2004 Orphanet 20 Mar 2008.

"Hay-Wells Syndrome." Index of Rare Diseases. National Organization for Rare Disorders. 20 Mar 2008.

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