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Microtia. Diagnosis and Management. About Microtia. Congenital anomaly of the external ear, usually evident at birth A small, abnormally shaped or absent external ear Occurs in 1 every 6,000 to 12,000 births Higher risk with higher maternal age. About Microtia. More common in males

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slide1

Microtia

Diagnosis and Management

slide2

About Microtia

  • Congenital anomaly of the external ear, usually evident at birth
  • A small, abnormally shaped or absent external ear
  • Occurs in 1 every 6,000 to 12,000 births
  • Higher risk with higher maternal age
slide3

About Microtia

  • More common in males
  • More common on the right ear
  • Reasons for the above are unknown
slide4

About Microtia

  • Possible causes:
    • in utero tissue ischemia secondary to obliteration of the stapedial artery
    • Ischemia due to actual hemorrhage into the local tissues
    • Genetic influences
    • Medications such as thalidomide and isotretinoin
    • May present in syndromes such as Goldenhar Syndrome or Treacher Collins Syndrome
slide6

About Microtia

  • Grading:
    • Grade I – malformation of the pinna, with most of the characteristics of the pinna, such as the helix, triangular fossa, and scaphae, are present with relatively good definition
    • Grade II – pinna less developed than in Grade I, the helix may be underdeveloped, with the other structures with less definition
slide7

About Microtia

  • Grading:
    • Grade III - pinna is essentially absent except for a vertical sausage-shaped skin remnant
    • Superior aspect of the remnant consists of underlying unorganized cartilage
    • Inferior aspect of this remnant consists of a relatively well-formed lobule
    • Anotia – total absence of the pinna
classification
Classification

Grade 2

Grade 1

classification1
Classification

Grade 3

Anotia

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About Aural Atresia

  • Absence of the ear canal
  • Patients who have microtiausually have aural atresia
  • Microtia and aural atresia tend to occur together because the outer ear and the middle ear evolve from a common embryologic origin
  • Patients with aural atresia have no hearing on the affected ear
slide11

Embryology of External Ear

  • 7th week of gestation
  • First and Second branchial arches  six Hillocks of His
    • 1 – Tragus (first/mandibular arch)
    • 2 - Crus of Helix (rest are from second/hyoid arch)
    • 3 - Ascending Helix
    • 4 - Upper Helix, Scapha, & Antihelix
    • 5 - Descending Helix, Middle Scapha & Antihelix
    • 6 - Inferior Helix, Antitragus
    • Lobule
slide13

Considerations

  • Inner ear in microtia is almost always functional, and thus hearing loss is basically conductive (the affected ear can still hear by some degree)
  • In unilateral microtia, the unaffected ear is usually normal, so speech development is usually at par with age
  • The MD should reassure the parents of the patient and outline the plan of management for the child
slide14

Management

  • Auricular Reconstruction is usually performed at 6-8 years in children with unilateral microtia
  • Pinna is 85-90% of its adult size by this age
  • At this age the patient is usually large enough that rib size is sufficient to harvest an adequate rib graft
  • Surgery may be done earlier if the child has adequate rib size, and postponed if not
slide15

Management

  • Auricular Reconstruction is usually performed at 6-7 years in children with bilateral microtia
    • The usual goal is to fix at least one ear, and achieve adequate hearing without assistive devices
  • Ear canal and middle ear may be evaluated for reconstruction using CT scans of temporal bones
  • External ear reconstruction is usually done prior to middle ear reconstruction to preserve the skin and blood vessels
  • Bone-anchored Hearing Aids (BAHAs) may be used if the child is not a candidate for surgery
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Management

  • Auricular Reconstruction
    • Stage I: Auricular Reconstruction
    • Stage II: Lobule Transposition
    • Stage III: Postauricular Skin Grafting
    • Stage IV: Tragal Reconstruction and Soft Tissue Debulking