This article may be too technical for most readers to understand.(August 2016) |
Accessory auricle | |
---|---|
Specialty | Medical genetics |
An accessory auricle is considered a developmental anomaly resulting from the persistence of a structure which variably recapitulates the normal external ear.
The general presentation is of a skin-covered nodule, papule, or nodule of the skin surface, usually immediately anterior to the auricle. However, it may be anywhere within the periauricular tissues. Bilateral presentation can be seen. [1] [2] [3] [4] [5]
A study of a family with 11 affected showed the accessory auricle were inherited in an autosomal dominant manner. [6]
The lesions presents as a nodule or papule, either sessile or pedunculated. They may be soft or have a cartilaginous structure. By histologic examination, it is a recapitulation of normal external auricle. There will be skin, cartilaginous structures, and cartilage (although the later is not seen in all variants of this disorder). [3] [4] Some investigators believe that the tragus is the only hillock which is derived from the first branchial arch. This is clearly suggestive that true cases of Accessory Auricle represent a true duplication of the hillocks that were part of the second branchial arch. [7] The second ear appears as a mirror image folded forward and lying on the posterior cheek.[ citation needed]
These structures are distinctly different from squamous papilloma and benign teratoma.[ citation needed]
The several components or degrees of development range from an ear tag, preauricular appendage, preauricular tag, or accessory tragus, to supernumerary ears or polyotia. [8] It is a relatively common congenital anomaly of the first branchial arch or second branchial arches. Other anomalies may be present concurrently, including cleft palate, cleft lip, or mandibular hypoplasia. There is a known association with Goldenhar syndrome (oculo-auriculo-vertebral syndrome) [9] and with Wildervanck syndrome. [10] [11] [12] There may also be an association with congenital cartilaginous rest of the neck.[ citation needed]
Simple surgical excision is curative. [13] The recommended treatment is that the skin is peeled off the extra-auricular tissue and protruding cartilage remnants are trimmed. [14] 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. [15] [16]
These lesions usually present in neonates, although they may not come to clinical attention until adulthood (for cosmetic reasons). There is no gender predilection. They are present in approximately 3-6 per 1000 live births. [17]
This article may be too technical for most readers to understand.(August 2016) |
Accessory auricle | |
---|---|
Specialty | Medical genetics |
An accessory auricle is considered a developmental anomaly resulting from the persistence of a structure which variably recapitulates the normal external ear.
The general presentation is of a skin-covered nodule, papule, or nodule of the skin surface, usually immediately anterior to the auricle. However, it may be anywhere within the periauricular tissues. Bilateral presentation can be seen. [1] [2] [3] [4] [5]
A study of a family with 11 affected showed the accessory auricle were inherited in an autosomal dominant manner. [6]
The lesions presents as a nodule or papule, either sessile or pedunculated. They may be soft or have a cartilaginous structure. By histologic examination, it is a recapitulation of normal external auricle. There will be skin, cartilaginous structures, and cartilage (although the later is not seen in all variants of this disorder). [3] [4] Some investigators believe that the tragus is the only hillock which is derived from the first branchial arch. This is clearly suggestive that true cases of Accessory Auricle represent a true duplication of the hillocks that were part of the second branchial arch. [7] The second ear appears as a mirror image folded forward and lying on the posterior cheek.[ citation needed]
These structures are distinctly different from squamous papilloma and benign teratoma.[ citation needed]
The several components or degrees of development range from an ear tag, preauricular appendage, preauricular tag, or accessory tragus, to supernumerary ears or polyotia. [8] It is a relatively common congenital anomaly of the first branchial arch or second branchial arches. Other anomalies may be present concurrently, including cleft palate, cleft lip, or mandibular hypoplasia. There is a known association with Goldenhar syndrome (oculo-auriculo-vertebral syndrome) [9] and with Wildervanck syndrome. [10] [11] [12] There may also be an association with congenital cartilaginous rest of the neck.[ citation needed]
Simple surgical excision is curative. [13] The recommended treatment is that the skin is peeled off the extra-auricular tissue and protruding cartilage remnants are trimmed. [14] 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. [15] [16]
These lesions usually present in neonates, although they may not come to clinical attention until adulthood (for cosmetic reasons). There is no gender predilection. They are present in approximately 3-6 per 1000 live births. [17]