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Aperts syndrome |
Details
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Aperts syndrome is a rare birth defect that occurs in about one of every 160,000 babies born and is characterized by a tower-shaped skull due to an undeveloped mid-face, craniosynostosis resulting in prominent eyes and recessed cheek bones, webbed toes, fingers, and eyes that are widely protruding and spaced. There may be a lower intellectual capacity in certain patients, but there are some children with this disease who have normal intelligence. Gastrointestinal malformations and cardiac problems may occur in Apert’s individuals. Patients with such an abnormal disease can have characteristics of unusual speech. They frequently have hyponasal resonance due to a small nose, underdeveloped mid-face, and extremely long soft palate. If a cleft palate is present, the patients can also have hypernasal resonance. Speech sound articulation is frequently distorted due to the high arched palate and to the malocclusion. A general developmental delay and impaired hearing will also affect language development as well as cause an effect on speech. The treatment of Aperts disease includes a multiple staged surgery. The release time which is best of the fused sutures is anywhere between ages 3-6 months. This procedure allows a baby’s brain to have enough room to grow. Also, frontal bones along with deformed bones will need to be repositioned to correct the upper facial deformation and bulging eyes. The plastic surgeon along with the neurosurgeon will work together in the operating room to get the best result. The webbing of the fingers is usually treated by separating the digits during the first several years of life so that it is possible to accomplish better function of the hand and better alignment.
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| Category |
Disease Conditions > A
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| Related Searches |
apert syndrome, apert syndrome acrocephalosyndactyly |
| Date Submitted |
19-Sep-2005
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