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DEPARTMENT OF OTOLARYNGOLOGY/ HEAD AND NECK SURGERY FACIAL PLASTIC AND RECONSTRUCTIVE SURGERY

Microtia: Information for Parents and Patients

For more information, please contact: Facial Plastic and Reconstructive Surgery & Pediatric Otolaryngology at UNC Carolina Crossing 2226 Nelson Highway, Suite #101 Chapel Hill, NC 27517

Phone: 919.490.3280

MED.UNC.EDU/ENT

DEPARTMENT OF OTOLARYNGOLOGY/ HEAD AND NECK SURGERY FACIAL PLASTIC AND RECONSTRUCTIVE SURGERY


Microtia

is when the external ear, or auricle, fails to develop normally. The cause is unknown and likely unpreventable. In some patients, it can be part of a larger syndrome such as craniofacial microsomia. There is a broad spectrum of microtia and affected ears can range from nearly normal in appearance to severe deformity. There are three commonly accepted “grades” of ear malformations, depicted in Figures 1-3. Anotia is a severe form of microtia where the ear is absent (Figure 4). Microtia is the most common type of congenital malformation of the ear and usually causes no health risks. However it can still present a major concern for children and their parents. Microtia is only treated when the child or parent feels that it may affect the child’s quality of life. However, because the outer ear

and ear canal are derived from the same embryonic structures, microtia frequently occurs with aural atresia (malformation of the ear canal and middle ear structures).

?

What is

Microtia

Aural atresia results in decreased hearing in the affected ear. Atresia and microtia are largely unilateral conditions, however both ears may be affected in some children and hearing aids may be necessary at an early age. Any hearing issues experienced by your child will be addressed on an individual basis by your physicians and hearing specialists (audiologists).

Managing microtia

requires a team approach. At UNC, this includes facial plastic surgery, pediatric otolaryngology, otology, audiology and genetics. Although unilateral microtia does not usually have a significant effect on hearing, an age appropriate hearing assessment will be conducted because poor hearing at an early age can lead to deficits in speech development. If hearing loss is present, regular visits with audiologists and speech pathologists may be necessary. Children with microtia will often have a CT scan of the hearing organs to determine if there is involvement of the hearing apparatus. Other testing, scans or ultrasounds may also be recommended by your physician.

?

Where do we go

from here

How will it affect

?

my child’s hearing

Parents are concerned

about hearing loss in a child with microtia, as well as the effects of hearing loss on their child’s speech and language development. It is important to stress that children with unilateral (one sided) ear deformity usually have normal hearing in the other ear and may not require a hearing aid. Children with unilateral hearing loss may notice difficulty localizing sounds (determining which direction sounds originate), as they lack stereo sound perception. However, if both ears are affected, bone conduction hearing aids or other hearing devices may be recommended. Hearing tests are conducted soon after birth and are repeated on a regular basis during follow-up visits. Because children with unilateral atresia have only one functional ear for hearing, it is very important to take noise precautions in order to preserve hearing. Ear infections should be treated aggressively and care by a pediatric otolaryngologist may be necessary. Recurrent ear infections may require more frequent visits.


Microtia

is when the external ear, or auricle, fails to develop normally. The cause is unknown and likely unpreventable. In some patients, it can be part of a larger syndrome such as craniofacial microsomia. There is a broad spectrum of microtia and affected ears can range from nearly normal in appearance to severe deformity. There are three commonly accepted “grades” of ear malformations, depicted in Figures 1-3. Anotia is a severe form of microtia where the ear is absent (Figure 4). Microtia is the most common type of congenital malformation of the ear and usually causes no health risks. However it can still present a major concern for children and their parents. Microtia is only treated when the child or parent feels that it may affect the child’s quality of life. However, because the outer ear

and ear canal are derived from the same embryonic structures, microtia frequently occurs with aural atresia (malformation of the ear canal and middle ear structures).

?

What is

Microtia

Aural atresia results in decreased hearing in the affected ear. Atresia and microtia are largely unilateral conditions, however both ears may be affected in some children and hearing aids may be necessary at an early age. Any hearing issues experienced by your child will be addressed on an individual basis by your physicians and hearing specialists (audiologists).

Managing microtia

requires a team approach. At UNC, this includes facial plastic surgery, pediatric otolaryngology, otology, audiology and genetics. Although unilateral microtia does not usually have a significant effect on hearing, an age appropriate hearing assessment will be conducted because poor hearing at an early age can lead to deficits in speech development. If hearing loss is present, regular visits with audiologists and speech pathologists may be necessary. Children with microtia will often have a CT scan of the hearing organs to determine if there is involvement of the hearing apparatus. Other testing, scans or ultrasounds may also be recommended by your physician.

?

Where do we go

from here

How will it affect

?

my child’s hearing

Parents are concerned

about hearing loss in a child with microtia, as well as the effects of hearing loss on their child’s speech and language development. It is important to stress that children with unilateral (one sided) ear deformity usually have normal hearing in the other ear and may not require a hearing aid. Children with unilateral hearing loss may notice difficulty localizing sounds (determining which direction sounds originate), as they lack stereo sound perception. However, if both ears are affected, bone conduction hearing aids or other hearing devices may be recommended. Hearing tests are conducted soon after birth and are repeated on a regular basis during follow-up visits. Because children with unilateral atresia have only one functional ear for hearing, it is very important to take noise precautions in order to preserve hearing. Ear infections should be treated aggressively and care by a pediatric otolaryngologist may be necessary. Recurrent ear infections may require more frequent visits.


Microtia

Repair

Open communication

between the family and our team of physicians is our first goal. Typically, observation is recommended until 7 to 9 years of age, when the child is better able to participate in the decisionmaking process and post-operative care. Additionally, this allows the child’s body to grow to better tolerate surgery, and allows the child’s opposite ear to mature in order to be used as a model or template for surgical reconstruction of the small ear. The child should be asked about the effects of microtia on his or her interactions with friends and classmates, and an informed decision to pursue treatment should be made with the child, family, and healthcare team. It is important to stress that observation is an acceptable option for microtia and atresia. However, most families choose to pursue surgical treatment of microtia. Several options exist for children and are outlined:

1. Observation

provides the advantage of avoiding surgery. The child and family must be comfortable with the appearance of the ear and be able to handle possible psychosocial effects of microtia. It is important to realize that surgery can be pursued later in childhood or early adulthood.

2. Prosthetic Management

Several types of prosthetics are available which simulate the normal ear. Prosthetic ears can be made to look very realistic; however they require daily maintenance of the attachment site and removal of the prosthesis before

sleep at night. It is possible for the ear to be accidently dislodged, which may create embarrassing situations for the child, or lead to misplacement prosthetic ear. Pursuing prosthetic management of microtia permanently excludes the possibility of surgical reconstruction of the ear using native tissue or other implants. Prosthetic management involves at least two surgeries; first the microtic ear must be removed, then a procedure is performed to create an attachment site for the prosthesis.

3. Reconstruction

Ear reconstruction is the most popular method of microtia repair as it allows for the use of the patient’s own tissue in reconstruction and requires less maintenance than a prosthesis. However aesthetic outcomes vary, and extra care must be

taken after surgery to ensure survival of the reconstructed ear. The most common method of reconstruction involves a sequence of surgical procedures called stages. This method involves using the patient’s own rib cartilage to reconstruct the ear. The reconstruction is typically divided into four stages that occur roughly every 3 months in order to optimize the healing process. The stages are described below and are accompanied by post-operative pictures. Of note, another reconstructive option offered at some institutions is a synthetic MEDPOR implant; however this method more frequently results in skin breakdown and implant breakage. UNC does not offer MEDPOR implants. Surgical reconstruction is performed in four stages:


Microtia

Repair

Open communication

between the family and our team of physicians is our first goal. Typically, observation is recommended until 7 to 9 years of age, when the child is better able to participate in the decisionmaking process and post-operative care. Additionally, this allows the child’s body to grow to better tolerate surgery, and allows the child’s opposite ear to mature in order to be used as a model or template for surgical reconstruction of the small ear. The child should be asked about the effects of microtia on his or her interactions with friends and classmates, and an informed decision to pursue treatment should be made with the child, family, and healthcare team. It is important to stress that observation is an acceptable option for microtia and atresia. However, most families choose to pursue surgical treatment of microtia. Several options exist for children and are outlined:

1. Observation

provides the advantage of avoiding surgery. The child and family must be comfortable with the appearance of the ear and be able to handle possible psychosocial effects of microtia. It is important to realize that surgery can be pursued later in childhood or early adulthood.

2. Prosthetic Management

Several types of prosthetics are available which simulate the normal ear. Prosthetic ears can be made to look very realistic; however they require daily maintenance of the attachment site and removal of the prosthesis before

sleep at night. It is possible for the ear to be accidently dislodged, which may create embarrassing situations for the child, or lead to misplacement prosthetic ear. Pursuing prosthetic management of microtia permanently excludes the possibility of surgical reconstruction of the ear using native tissue or other implants. Prosthetic management involves at least two surgeries; first the microtic ear must be removed, then a procedure is performed to create an attachment site for the prosthesis.

3. Reconstruction

Ear reconstruction is the most popular method of microtia repair as it allows for the use of the patient’s own tissue in reconstruction and requires less maintenance than a prosthesis. However aesthetic outcomes vary, and extra care must be

taken after surgery to ensure survival of the reconstructed ear. The most common method of reconstruction involves a sequence of surgical procedures called stages. This method involves using the patient’s own rib cartilage to reconstruct the ear. The reconstruction is typically divided into four stages that occur roughly every 3 months in order to optimize the healing process. The stages are described below and are accompanied by post-operative pictures. Of note, another reconstructive option offered at some institutions is a synthetic MEDPOR implant; however this method more frequently results in skin breakdown and implant breakage. UNC does not offer MEDPOR implants. Surgical reconstruction is performed in four stages:


Stage I An incision is made in the chest and rib cartilage grafts are removed. The rib is then sculpted by the surgeon into the shape of the new ear, using the patient’s other ear as a template. The framework is then placed beneath the patient’s skin. The unused pieces of cartilage are placed back into the chest or underneath the scalp for later use. You and your child may notice a small bump or area of raised skin above this storage site. This is called “banked cartilage.” Stage II The ear lobe is rotated into a more natural position and is attached to the framework of the new ear. Stage III Before this stage, the reconstructed ear rests flat against the head. Incisions

are made along the back of the ear in order to lift it away from the head and create a more normal outward projection of the ear. Banked rib cartilage is retrieved and placed under the back of the ear for support. A skin graft is taken and placed over the back of the ear, trying to create the natural valley behind the ear. Stage IV The new ear is further defined by creation of the tragus, or the “bump” just in front of the opening of the ear canal. The conchal bowl is also addressed, trying to make it deeper. Tissue is taken from the opposite ear to create these structures and a skin graft may be necessary. Additional stages or “touch up” procedures may be required based on individual cases.

Grade I Microtia

Grade II Microtia

Grade III Microtia & Final Result

Grade III Microtia

Grade II Microtia & Final Result

Expectations Following the staged ear reconstruction, the patient has an ear that is the general shape and size of the normal ear. However, it is not possible to create an ear with all the unique characteristics of the normal ear. The photos included in this educational booklet should help give you some idea of what the new ear will look like. As reconstructive surgeons, our goal is to recreate as normal appearing ear as possible. Our results are never perfect, but there is usually a significant improvement.


Stage I An incision is made in the chest and rib cartilage grafts are removed. The rib is then sculpted by the surgeon into the shape of the new ear, using the patient’s other ear as a template. The framework is then placed beneath the patient’s skin. The unused pieces of cartilage are placed back into the chest or underneath the scalp for later use. You and your child may notice a small bump or area of raised skin above this storage site. This is called “banked cartilage.” Stage II The ear lobe is rotated into a more natural position and is attached to the framework of the new ear. Stage III Before this stage, the reconstructed ear rests flat against the head. Incisions

are made along the back of the ear in order to lift it away from the head and create a more normal outward projection of the ear. Banked rib cartilage is retrieved and placed under the back of the ear for support. A skin graft is taken and placed over the back of the ear, trying to create the natural valley behind the ear. Stage IV The new ear is further defined by creation of the tragus, or the “bump” just in front of the opening of the ear canal. The conchal bowl is also addressed, trying to make it deeper. Tissue is taken from the opposite ear to create these structures and a skin graft may be necessary. Additional stages or “touch up” procedures may be required based on individual cases.

Grade I Microtia

Grade II Microtia

Grade III Microtia & Final Result

Grade III Microtia

Grade II Microtia & Final Result

Expectations Following the staged ear reconstruction, the patient has an ear that is the general shape and size of the normal ear. However, it is not possible to create an ear with all the unique characteristics of the normal ear. The photos included in this educational booklet should help give you some idea of what the new ear will look like. As reconstructive surgeons, our goal is to recreate as normal appearing ear as possible. Our results are never perfect, but there is usually a significant improvement.


DEPARTMENT OF OTOLARYNGOLOGY/ HEAD AND NECK SURGERY FACIAL PLASTIC AND RECONSTRUCTIVE SURGERY

Microtia: Information for Parents and Patients

For more information, please contact: Facial Plastic and Reconstructive Surgery & Pediatric Otolaryngology at UNC Carolina Crossing 2226 Nelson Highway, Suite #101 Chapel Hill, NC 27517

Phone: 919.490.3280

MED.UNC.EDU/ENT

DEPARTMENT OF OTOLARYNGOLOGY/ HEAD AND NECK SURGERY FACIAL PLASTIC AND RECONSTRUCTIVE SURGERY


Microtia Booklet