Practice Essentials
Microtia is the most common major congenital deformity of the external ear, but it is rare enough that parents usually have never encountered it. It is rarely noticed on antenatal ultrasonography (US). Because of the rarity of microtia, most parents and some healthcare providers may not fully understand how to care for children with this condition.
Consultation regarding a patient born with microtia and atresia ideally should occur soon after birth. The initial examination consists of a detailed head-and-neck examination. If microtia is the only developmental anomaly, the initial workup consists of evaluation of the child's hearing status. Computed tomography (CT) of the ears is not recommended in children younger than 4 years.
Technical advances in the reconstruction of the microtic ear have led to dramatically improved results in the hands of experienced ear reconstructive surgeons. For unilateral microtia and atresia, options include (1) no intervention, (2) a nonsurgical bone-conduction system, (3) a BAHA Softband, (4) ear-canal surgery (ie, atresiaplasty or canalplasty), (5) a bone-anchored auditory processor, and (6) a middle-ear implant system (generally not a primary option). Options for bilateral microtia and atresia are similar, except for the no-intervention option. The most common options are the bone-anchored auditory processor and the ear-canal surgery.
Given the fine details that must be addressed in microtia reconstruction, it is essential for the surgeon to have a complete understanding of the three-dimensional (3D) form of the auricle and an awareness of sound surgical principles of soft-tissue management and transfer. The future of microtia surgery holds much promise.
Anatomy
The reconstructive surgeon must have an intimate knowledge of the anatomy of the external, middle, and inner ear. Important landmarks include the following:
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Helix
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Helical crus
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Antihelix (anthelix)
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Crura of the antihelix
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Tragus
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Antitragus
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Scapha
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Triangular fossa
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Cymba conchae
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Cavum conchae
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Lobule
The primary blood supply to the external ear is via the superficial temporal artery and branches of the posterior auricular artery. The sensory innervation of the external ear is via the anterior and posterior branches of the greater auricular nerve.
The location and reference point of the ear must be well understood. The angle of the long axis of the ear approximates the angle of the nasal dorsum. The superior point of the ear is usually at the eyebrow level. In a child aged 5-7 years, the helical crus is usually approximately 6.5-7.0 cm from the lateral canthus. The most inferior aspect of the lobule on the microtic ear is usually 0.5 cm higher than the normal side.
Pathophysiology
During week 7 of gestation, the first and second arches give rise to six hillocks called the hillocks of His. The first three hillocks are derived from the first arch, and the final three are derived from the second arch. These hillocks then fuse, forming the future ear. The structures of the adult ear corresponding to the hillocks are the tragus (first hillock), helical crus (second hillock), the helix (third hillock), the antihelix (fourth and fifth hillocks), and the antitragus (sixth hillock).
Etiology
The actual cause of microtia is relatively unknown. McKenzie and Craig stated that it may occur as a result of in-utero tissue ischemia secondary to obliteration of the stapedial artery or actual hemorrhage into the local tissues. [1]
Genetic studies have revealed several possible etiologic factors, such as chromosomal aberrations, multifactorial inheritance, and autosomal and recessive traits. The most common syndromes associated with microtia are Goldenhar syndrome and Treacher Collins syndrome.
In addition, several medications, such as thalidomide and isotretinoin, have been implicated as causing severe congenital malformations such as microtia; however, these drugs are no longer used or recommended during pregnancy.
Epidemiology
Microtia has been reported to occur in 1 per 6000-12,000 births. Although the reason is unknown, microtia is seen more commonly in males than in females. In addition, the right side is affected more commonly than the left. Some studies have reported that the prevalence of auricular anomalies at birth increases with maternal age.
Prognosis
The affected ear usually has severe conductive hearing loss (~40-60 dB) secondary to lack of an external auditory canal (atresia) and ossicular fixation. As long as the microtia is unilateral, these children are likely to have normal speech.
Although some children do not want surgery, parents must discuss the benefits of the reconstructions with them. With good results, all children can have very positive physical and psychological outcomes.
Total auricular reconstruction is one of the most challenging procedures performed by the ear reconstruction surgeon. Understanding the psychological issues faced by patients with microtia and providing education to the parents of these patients are fundamental to a successful microtia practice.
Complete knowledge of the 3D anatomy of the ear and of sound surgical principles of soft-tissue management and tissue transfer is necessary. [2]
In children younger than 3-4 years, a microtic ear usually has little psychological impact, and surgery generally is not recommended by surgeons worldwide. After this time, children usually begin to notice that their ears are different. Parents must treat their child with microtia in the same way as they do their other children. Although parents may not realize, children feel the anxiety of their parents. Parents who attempt to cover the ear tend to hinder the confidence of the child. The children with the lowest self-esteem tend to be the ones whose parents transmitted anxiety about the ear throughout their childhood.
Patients whose ears have not been reconstructed as children may have difficulty dealing with the peer pressure of adolescence. During this time, fitting in with their peers is of considerable importance. Teenagers are particularly conscious of their looks and are very aware of their microtic ear. Although they tend to be more eager to undergo surgical reconstruction than younger children, their expectations tend to be more unrealistic.
Accordingly, it is imperative to counsel patients and parents as early as possible. With early counseling, expectations become much more realistic, and the patients are able to deal with certain social issues much more easily.
Patient Education
Microtia is rarely noticed antenatally; when discovered, it is very traumatic for parents. Another major stressor is the feeling of guilt that parents may experience. It is very important to reassure parents that they were not the cause of their child's condition. Such reassurance can be provided only through excellent communication between the parents, the child and the microtia surgeon.
For more information, patients and their parents may visit the Microtia-Congenital Ear Deformity Institute Web site.
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Microtia, grade I. Pinna is malformed and smaller than normal. Most characteristics of pinna (eg, helix, triangular fossa, and scaphae) are present with relatively good definition. Patient is also lacking ear canal (atresia).
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Microtia, grade II. Pinna is smaller and less developed than in grade I. Helix may not be fully developed. Triangular fossa, scaphae, and antihelix (anthelix) have much less definition.
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Microtia, grade III. Pinna is essentially absent, except for vertical sausage-shaped skin remnant. Superior aspect of this sausage-shaped skin remnant consists of underlying unorganized cartilage, and inferior aspect consists of relatively well-formed lobule.
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Anotia (total absence of pinna).
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Three-dimensional sonogram of fetus with microtia.
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Arrow in CT scan indicates lack of ear canal (atresia) on left side. Note normal ear canal on right side.
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Arrow points to small incision. Average length is approximately 1.0-1.5 in. (2.5-3.5 cm), depending on experience of microtia surgeon and weight of child.
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Microtia rib cartilage used for patient with microtia. Very small (1.0-1.5 in.) incision is made on skin, and synchondrosis of ribs 6 and 7 is excised, as well as small cartilaginous segment of rib 8. Ribs 6-7 synchondrosis forms main framework, and tragus of ear and rib 8 forms rim or helix of ear.
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Incision should be as small as possible without jeapordizing visualization of cartilage. In this example, incision was only 0.75 in. (2 cm), which was sufficient to harvest cartilage for this child with right-side microtia and atresia.
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Incision should be as small as possible while still permitting excellent visualization of tissues. In this example, incision on right chest area was only 1 in. (2.5 cm), which was sufficient to harvest necessary cartilage to build ear of patient born with left-side microtia.
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Image shows carved cartilaginous framework (including tragus) from small portions of ribs 6, 7, and 8.
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Final portion of first stage of microtia surgery. Once cartilage is sculpted into shape of ear, it is inserted into skin pocket, and once suction is placed, new ear is evident. Note how color of new ear with cartilage technique is normal. There is no skin that has to be removed, and thus, sensation of ear is maintained as well. Earlobe will be mobilized into its future site in next stage of procedure. Video courtesy of Arturo Bonilla, MD.
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Patient shown underwent first stage of microtia surgery 1 week previously. Because cartilage framework is simply placed under skin pocket, note natural color of ear. No skin is removed with rib cartilage technique.
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Second stage consists of forming earlobe (otherwise known as transposition of lobule). Incision is made both in front of and behind skin vestige as shown. Skin vestige is then rotated into place via Z-plasty and spliced to cartilaginous framework that was sculpted during first stage.
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Patient shown underwent second stage of microtia surgery 2 weeks previously. Earlobe has been positioned into its permanent location, and conchal bowl has been deepened (as evidenced by shadow-effect of conchal bowl).
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Full-thickness skin graft is harvested from groin area in order to hide incision site.
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Third (separation and elevation) stage of microtia reconstruction. Swelling is normal and will decrease after several weeks, revealing fine detail of ear. Video courtesy of Arturo Bonilla, MD.
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Once microtic ear is separated and elevated, ears are symmetrical. Patient had classic grade III microtia and atresia. Photograph on right shows excellent separation from third stage.
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Patient with most common type of microtia (ie, grade III microtia and atresia) before surgical reconstruction.
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Patient with grade III microtia and atresia after surgical reconstruction with natural cartilage technique. Note how skin color remains normal. Sensation of skin also remains normal.
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Video shows how alloplastic polyethylene implant can fracture before it is used for microtia surgery. Lifelong risk of infection, rejection, or fracture is why most surgeons do not use this technique to treat microtia.
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Result after rib cartilage framework is placed under skin pocket. Note natural skin color of ear. Cartilage technique does NOT require skin graft placed over ear that will give it two-toned color effect.
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Printing of ear cartilage cells using 3D bioprinting is promising technique in microtia reconstruction.
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Custom-designed porous high-density polyethylene wedge was designed to enhance projection of reconstructed microtia ears with use of rib cartilage technique. This avoids need to harvest extra ribs to provide projection. Wedge is well-hidden behind ear, affording natural look to reconstructed cartilage ear. Image courtesy of Poriferous, LLC.