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Goldenhar syndrome is a rare congenital defect characterized by incomplete development of the ear, nose, soft palate, lip and mandible on usually one side of the body. Common clinical manifestations include limbal dermoids , preauricular skin tags and strabismus . [ 1 ]
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.
The VACTERL association (also VATER association, and less accurately VACTERL syndrome) refers to a recognized group of birth defects which tend to co-occur (see below). This pattern is a recognized association, as opposed to a syndrome , because there is no known pathogenetic cause to explain the grouped incidence.
[2] [1] Low-set ears can be associated with conditions such as: Down syndrome [3] Turner syndrome; Noonan syndrome [4] Patau syndrome [5] DiGeorge syndrome [6] Cri du chat syndrome; Edwards syndrome; Fragile X syndrome; Okamoto syndrome; It is usually bilateral, but it can be unilateral (one sided) in Goldenhar syndrome. [7]
The recommended treatment is that the skin is peeled off the extra-auricular tissue and protruding cartilage remnants are trimmed. [13] Normal appearance is achieved in majority of cases. The reconstruction successful in true cases of accessory auricle, as it also is in individuals with auricular appendages. [14] [15]
Pierre Robin sequence [a] (/ p j ɛər r ɔː ˈ b æ̃ /; [3] abbreviated PRS) is a congenital defect observed in humans which is characterized by facial abnormalities.The three main features are micrognathia (abnormally small mandible), which causes glossoptosis (downwardly displaced or retracted tongue), which in turn causes breathing problems due to obstruction of the upper airway.
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The treatment of vocal fold paralysis varies depending on its cause and main symptoms. For example, if laryngeal nerve paralysis is caused by a tumor, suitable therapy should be initiated. In the absence of any additional pathology, the first step of clinical management should be observation to determine whether spontaneous nerve recovery will ...