Microtia
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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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Microtia

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Microtia

Microtia

Diagnosis and Management


Microtia

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


Microtia

About Microtia

  • More common in males

  • More common on the right ear

  • Reasons for the above are unknown


Microtia

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


Anatomy of the external ear

Anatomy of the External Ear


Microtia

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


Microtia

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


Microtia

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


Microtia

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


Microtia

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


Microtia

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


Microtia

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


Microtia

Management

  • Auricular Reconstruction

    • Stage I: Auricular Reconstruction

    • Stage II: Lobule Transposition

    • Stage III: Postauricular Skin Grafting

    • Stage IV: Tragal Reconstruction and Soft Tissue Debulking


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