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Microtia. Diagnosis and Management. About Microtia. Congenital anomaly of the external ear, usually evident at birth Occurs in 1 every 6,000 to 12,000 births Higher risk with higher maternal age. About Microtia. Possible causes:
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Microtia Diagnosis and Management
About Microtia • Congenital anomaly of the external ear, usually evident at birth • Occurs in 1 every 6,000 to 12,000 births • Higher risk with higher maternal age
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
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
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
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
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
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