Accessory auricle

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Accessory auricle
Accessory auricle.jpg
Classification and external resources
Specialty medical genetics
ICD-10 Q17.0
ICD-9-CM 744.1
OMIM 610420
MeSH Dimpal

An accessory auricle is considered a developmental anomaly resulting from the persistence of a structure which variably recapitulates the normal external ear.

Signs and symptoms[edit]

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]

Genetics[edit]

A study of a family with 11 affected individuals showed the phenotype was inherited in an autosomal dominant manner.[6]

Diagnosis[edit]

An intermediate power of a microscopic view of an accessory auricle.

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]

Differential diagnosis[edit]

These structures are distinctly different from squamous papilloma and benign teratoma.

Classification[edit]

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.

Management[edit]

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]

Epidemiology[edit]

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]

See also[edit]

References[edit]

  1. ^ Cosman, B. C. (1993). "Bilateral accessory tragus". Cutis; cutaneous medicine for the practitioner. 51 (3): 199–200. PMID 8444054. 
  2. ^ "Preauricular sinus and accessory auricle. (Photoclinic)." Consultant Feb. 2002: 256+. Health Reference Center Academic. Web. 2 Nov. 2011.
  3. ^ a b Jansen, T.; Romiti, R.; Altmeyer, P. (2000). "Accessory tragus: Report of two cases and review of the literature". Pediatric dermatology. 17 (5): 391–394. PMID 11085670. doi:10.1046/j.1525-1470.2000.017005391.x. 
  4. ^ a b Brownstein, M. H.; Wanger, N.; Helwig, E. B. (1971). "Accessory tragi". Archives of dermatology. 104 (6): 625–631. PMID 5131708. 
  5. ^ Hodges FR; Sahouria JJ; Wood AJ (2006). "Accessory tragus: A report of 2 cases". J Dent Child (Chic). 73 (1): 42–4. PMID 16734313. 
  6. ^ Yang, Y; et al. (2006). "A locus for autosomal dominant accessory auricular anomaly maps to 14q11.2–q12". Human genetics. 120 (1): 144–147. PMID 16775710. doi:10.1007/s00439-006-0206-1. 
  7. ^ Stevenson, Roger E.; Hall, Judith G. Human Malformations and Related Anomalies (2nd ed.). Oxford University Press, USA, 2005. pp. 339, 340. ISBN 019974808X. 
  8. ^ Lam, J.; Dohil, M. (2007). "Multiple Accessory Tragi and Hemifacial Microsomia". Pediatric Dermatology. 24 (6): 657–658. PMID 18035991. doi:10.1111/j.1525-1470.2007.00560.x. 
  9. ^ Konaş, E.; Canter, H. I.; Mavili, M. E. (2006). "Goldenhar complex with atypical associated anomalies: Is the spectrum still widening?". The Journal of craniofacial surgery. 17 (4): 669–672. PMID 16877912. doi:10.1097/00001665-200607000-00011. 
  10. ^ Tadini, G.; Cambiaghi, S.; Scarabelli, G.; Brusasco, A.; Vigo, P. (1993). "Familial occurrence of isolated accessory tragi". Pediatric dermatology. 10 (1): 26–28. PMID 8493161. doi:10.1111/j.1525-1470.1993.tb00006.x. 
  11. ^ Gao, J. Z.; Chen, Y. M.; Gao, Y. P. (1990). "A survey of accessory auricle anomaly. Pedigree analysis of seven cases". Archives of otolaryngology--head & neck surgery. 116 (10): 1194–1196. PMID 2206506. 
  12. ^ Resnick, K. I.; Soltani, K.; Bernstein, J. E.; Fathizadeh, A. (1981). "Accessory tragi and associated syndromes involving the first branchial arch". The Journal of dermatologic surgery and oncology. 7 (1): 39–41. PMID 7204730. doi:10.1111/j.1524-4725.1981.tb00591.x. 
  13. ^ Pan, B.; Qie, S.; Zhao, Y.; Tang, X.; Lin, L.; Yang, Q.; Zhuang, H.; Jiang, H. (2010). "Surgical management of polyotia". Journal of Plastic, Reconstructive & Aesthetic Surgery. 63 (8): 1283–1288. PMID 19617017. doi:10.1016/j.bjps.2009.06.037. 
  14. ^ Scott-Brown's Otorhinolaryngology (7th ed.). Hodder Arnold. p. 969. ISBN 978 0 340 808 931. 
  15. ^ Ku, PK; Tong, MC; Yue, V (1998). "Polyotia- a rare external ear anomaly". Int J Pediatr Otorhinolaryngol (46:117). 
  16. ^ Bendor-Samuel, RL; Tung, TC; Chen, YR (1995). "Polyotia". Ann Plast Surg (34:650). 
  17. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 894. ISBN 1-4160-2999-0. 

Further reading[edit]

Lester D. R. Thompson; Bruce M Wenig (2011). Diagnostic Pathology: Head and Neck: Published by Amirsys. Hagerstown, MD: Lippincott Williams & Wilkins. pp. 7:2–3. ISBN 1-931884-61-7.