 | This article may be too technical for most readers to understand.(August 2016) |
| Accessory auricle | |
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| 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]
The outer ear, external ear, or auris externa is the external part of the ear, which consists of the auricle and the ear canal. It gathers sound energy and focuses it on the eardrum.
Anotia describes a rare congenital deformity that involves the complete absence of the pinna, the outer projected portion of the ear, and narrowing or absence of the ear canal. This contrasts with microtia, in which a small part of the pinna is present. Anotia and microtia may occur unilaterally or bilaterally. This deformity results in conductive hearing loss, deafness.
The auricle or auricula is the visible part of the ear that is outside the head. It is also called the pinna, a term that is used more in zoology.
An infantile hemangioma (IH), sometimes called a strawberry mark due to appearance, is a type of benign vascular tumor or anomaly that affects babies. Other names include capillary hemangioma, strawberry hemangioma, strawberry birthmark and strawberry nevus. and formerly known as a cavernous hemangioma. They appear as a red or blue raised lesion on the skin. Typically, they begin during the first four weeks of life, growing until about five months of life, and then shrinking in size and disappearing over the next few years. Often skin changes remain after they shrink. Complications may include pain, bleeding, ulcer formation, disfigurement, or heart failure. It is the most common tumor of orbit and periorbital areas in childhood. It may occur in the skin, subcutaneous tissues and mucous membranes of oral cavities and lips as well as in extracutaneous locations including the liver and gastrointestinal tract.
Treacher Collins syndrome (TCS) is a genetic disorder characterized by deformities of the ears, eyes, cheekbones, and chin. The degree to which a person is affected, however, may vary from mild to severe. Complications may include breathing problems, problems seeing, cleft palate, and hearing loss. Those affected generally have normal intelligence.
A port-wine stain is a discoloration of the human skin caused by a vascular anomaly. They are so named for their coloration, which is similar in color to port wine, a fortified red wine from Portugal.
Otoplasty denotes the surgical and non-surgical procedures for correcting the deformities and defects of the pinna, and for reconstructing a defective, or deformed, or absent external ear, consequent to congenital conditions and trauma. The otoplastic surgeon corrects the defect or deformity by creating an external ear that is of natural proportions, contour, and appearance, usually achieved by the reshaping, the moving, and the augmenting of the cartilaginous support framework of the pinna. Moreover, the occurrence of congenital ear deformities occasionally overlaps with other medical conditions.
The auricular branch of the vagus nerve is often termed the Alderman's nerve or Arnold's nerve. The latter name is an eponym for Friedrich Arnold. The auricular branch of the vagus nerve supplies sensory innervation to the skin of the ear canal, tragus, and auricle.
Microtia is a congenital deformity where the auricle is underdeveloped. A completely undeveloped pinna is referred to as anotia. Because microtia and anotia have the same origin, it can be referred to as microtia-anotia. Microtia can be unilateral or bilateral. Microtia occurs in 1 out of about 8,000–10,000 births. In unilateral microtia, the right ear is most commonly affected. It may occur as a complication of taking Accutane (isotretinoin) during pregnancy.
The tragus is a small pointed eminence of the external ear, situated in front of the concha, and projecting backward over the meatus. It also is the name of hair growing at the entrance of the ear. Its name comes the Ancient Greek tragos, meaning 'goat', and is descriptive of its general covering on its under surface with a tuft of hair, resembling a goat's beard. The nearby antitragus projects forwards and upwards.
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. It is associated with anomalous development of the first branchial arch and second branchial arch.
The posterior auricular vein is a vein of the head. It begins from a plexus with the occipital vein and the superficial temporal vein, descends behind the auricle, and drains into the external jugular vein.
Hearing loss with craniofacial syndromes is a common occurrence. Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely.
The posterior auricular muscle is a muscle behind the auricle of the outer ear. It arises from the mastoid part of the temporal bone, and inserts into the lower part of the cranial surface of the auricle of the outer ear. It draws the auricle backwards, usually a very slight effect.
A vascular malformation is a blood vessel or lymph vessel abnormality. Vascular malformations are one of the classifications of vascular anomalies, the other grouping is vascular tumors. They may cause aesthetic problems as they have a growth cycle, and can continue to grow throughout life.
Aplasia cutis congenita is a rare disorder characterized by congenital absence of skin. Ilona J. Frieden classified ACC in 1986 into 9 groups on the basis of location of the lesions and associated congenital anomalies. The scalp is the most commonly involved area with lesser involvement of trunk and extremities. Frieden classified ACC with fetus papyraceus as type 5. This type presents as truncal ACC with symmetrical absence of skin in stellate or butterfly pattern with or without involvement of proximal limbs. It is the most common congenital cicatricial alopecia, and is a congenital focal absence of epidermis with or without evidence of other layers of the skin.
Preauricular sinuses and preauricular cysts are two common congenital malformations. Each involves the external ear. The difference between them is that a cyst does not connect with the skin, but a sinus does. Frequency of preauricular sinus differs depending the population: 0.1–0.9% in the US, 0.9% in the UK, and 4–10% in Asia and parts of Africa.
Congenital cartilaginous rest of the neck is a minor and very rare congenital cutaneous condition characterized by branchial arch remnants that are considered to be the cervical variant of accessory tragus. It resembles a rudimentary pinna that in most cases is located in the lower anterior part of the neck.
Wildervanck syndrome or cervico-oculo-acoustic syndrome comprises a triad of:
Supernumerary nipples–uropathies–Becker's nevus syndrome is a skin condition that may be associated with genitourinary tract abnormalities. Supernumerary nipples, also referred to as polythelia or accessory nipples, is a pigmented lesion of the skin that is present at birth. This pigmentation usually occurs along the milk lines, which are the precursors to breast and nipple development. Clinically, this congenital condition is generally considered benign, but some studies have suggested there may be an association with kidney diseases and cancers of the urogenital system.