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Pediatric Microtia Surgery Treatment & Management

  • Author: Arturo Bonilla, MD; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
 
Updated: Mar 11, 2016
 

Approach Considerations

Indications for surgery

Unilateral microtia

Auricular reconstruction for unilateral microtia usually begins in children aged 6-7 years, depending on the child's size. Because the pinna reaches about 85-90% of its adult size at this age, surgery earlier than 6-7 years of age is generally not recommended or accepted worldwide. By this age, the child is usually large enough that rib size is sufficient to allow harvesting of an adequate rib graft. If the child is still small, the surgical procedure is postponed until an adequate rib for the framework can be harvested.

For example, if a very small 6-year-old child presents with microtia, postponing auricular reconstruction until 7-8 years of age, when rib growth is sufficient, is reasonable. On the other hand, if a very large 5-year-old child presents with microtia, the surgeon may consider reconstruction sooner because of adequate rib size; however, this is not the norm.

The ideal time to begin the reconstruction is the summer before the first grade. By the time the child starts the first grade, at least two of the three surgical procedures can be completed, allowing the child to have the semblance of an ear. Children tend to be teased during these early school years when the ear is not yet reconstructed.

Bilateral microtia

Surgical reconstruction in children with bilateral microtia is also started around 6-7 years of age, depending on their size. Before the pinna reconstructions, a computed tomography (CT) scan of the temporal bones is obtained to evaluate for ear-canal and middle-ear reconstructive surgery.

The ear reconstructions using the natural cartilage technique are started before the ear-canal surgery (atresiaplasty). If an attempt is made to open the canal before the external ear reconstruction, the elasticity of the "virgin" skin and the circulation are compromised. In addition, the scar tissue resulting from the ear-canal surgery makes it more difficult to attain the ideal result.

Because these children are dependent on bone-conduction hearing aids, the goal of starting earlier is to finish at least one ear. The canal may be opened soon after the ear is reconstructed. Obtaining adequate hearing without hearing aids is the eventual goal. If the child is not a candidate for atresia surgery, the bone-anchored hearing aid (BAHA) is also an option.

Contraindications for surgery

Contraindications to rib surgery for microtia reconstruction include high-risk surgical status and chest-wall deformities. In addition, patients with pulmonary hypoplasia are not candidates for rib-cartilage surgery, because of the potential risk of a pneumothorax (though in the hands of an experienced microtia surgeon, this event is extremely rare).

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Options for Hearing Correction

There are several options for hearing correction in patients with unilateral microtia and atresia, as follows:

  • No intervention - Because the child has a normal-hearing ear, the use of the softband with auditory processor is optional
  • BAHA Softband - This is basically a headband with a vibratory auditory processor.
  • Ear-canal surgery (ie, atresiaplasty or canalplasty) - A CT scan of the temporal bones must be obtained to evaluate the anatomy of the middle ear and inner ear and identify the location of the facial nerve; whether the child is a candidate for ear-canal surgery depends on the favorability of the anatomy
  • Bone-anchored auditory processor - This is an option if the child is not a candidate for ear-canal surgery or if the family prefers this option regardless of candidacy for the ear-canal procedure; the soonest a child with microtia and atresia can undergo this option is 5 years of age because of the thinness of the skull at this age.
  • Middle-ear implant system - This system is not yet approved by the US Food and Drug Administration (FDA) and is generally not a primary option

Similar options are available for hearing correction in patients with bilateral microtia and atresia, except for the no-intervention option, as follows:

  • BAHA Softband
  • Ear-canal surgery
  • Bone-anchored auditory processor
  • Middle-ear implant system

The most common options are the bone-anchored auditory processor and the ear-canal surgery.

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Surgical Options and Natural Cartilage Technique

Surgical options

Surgical options for treatment of microtia and atresia are as follows::

  • Natural cartilage technique (rib technique) - For the past 50 years, this technique has been the "gold standard," and it continues to be the most widely accepted microtia repair technique worldwide; approximately 98-99% of microtia surgeons utilize the natural cartilage technique as their preferred choice
  • Alloplastic implant (porous polyethylene implant) [2, 3] - This technique is used by a few surgeons, though it is not widely accepted, because of the plastic foreign body material and the hardness of the implant; there is a risk of infection, rejection, or fracture over the lifespan of the implant
  • Prosthetic - This technique is usually reserved for failed reconstructions; a few surgeons use it as the initial option, but this is very rare
  • No surgical intervention - Because microtia surgery is elective, the option of watchful waiting by the patient should always be offered

Unilateral microtia and atresia

Tanzer and Brent advocated multistage reconstructions, which are currently favored by most ear reconstruction surgeons worldwide. Some surgeons favor a two-stage procedure. Microtia reconstruction using the natural cartilage technique involves three surgical stages. To optimize the healing process, there is a 2-month interval between stages 1 and 2 and between stages 2 and 3. The three stages in the author's preferred approach are as follows:

  • Stage 1 - Rib-cartilage harvesting with carving and sculpting of the main ear framework, including the tragus, and insertion under a skin pocket on the side affected by microtia
  • Stage 2 - Formation of the lobule (ie, earlobe) and deepening of the conchal base
  • Stage 3 - Separation and elevation of the ear with insertion of an implant wedge and a postauricular full-thickness skin graft

In cases of bilateral microtia, the surgical procedures are combined in order to minimize anesthesia time. 

Stage 1: rib-cartilage harvesting and framework insertion

The first stage consists of two separate components, as follows:

  • Harvesting the costal cartilage
  • Inserting the carved sculpted cartilage into a skin pocket on the microtia side

A small, slightly oblique incision is made over the sixth, seventh, and eighth ribs (see the image below). When the child is thin, the incision is usually very small (~1.0-1.5 in., or 2.5-3.5 cm). The incision is made on the side opposite the microtia because this provides the convexity required for the normal ear. In other words, if the patient has microtia on the right side, the costal cartilage is removed from the left chest area.

Arrow points to small incision. Average length is 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.

The synchondrosis of the sixth and seventh ribs and the cartilaginous portion of the eighth rib are harvested according to a previously measured template (see the images below).

Very small (1.0-1.5 in.) incision is made on skin, 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.
Incision should be as small as possible without je 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.

A small piece of synchondrosis is used to form the tragus. The cartilaginous part of the eighth rib is used to form the rim or helix. The three pieces of rib are carved, sculpted, and sutured together to yield a cartilaginous framework in the shape of an ear (see the image below).

Image shows carved cartilaginous framework from sm Image shows carved cartilaginous framework from small portions of ribs 6, 7, and 8.

The first stage concludes with placement of the newly formed ear under the skin pocket (see the video below).

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.

Because an epidural injection is placed while the child is under general anesthesia, it is very common to for the child to wake up without any pain or discomfort. Patients are sent home with pain medication as needed and antibiotics. An ear dressing without pressure is applied during surgery and left intact for about 3 days. At that time, the wound is checked and the ear is rebandaged.

The drain stays in place for about 5-7 days to provide negative suction so that the skin can coapt to the newly inserted framework. The child is seen 1 week after surgery to remove the drain in the office. The sutures are removed on postoperative day 7. In the image below, obtained at the 1-week postoperative visit, it is apparent that the ear has a very natural shape and that the skin color is normal.

Patient shown underwent first stage of microtia su 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.

The child usually resumes normal activity within 1 week, though heavy contact sports are restricted for approximately 4-6 weeks. The hair may be washed 1 week after surgery. The second stage of the reconstruction may be carried out approximately 2 months later.

Stage 2: formation of lobule and deepening of conchal bowl

The second stage consists of formation of the earlobe (transposition of the lobule). An incision is made both in front of and behind the skin vestige (see the image below). The skin vestige is rotated into place via a Z-plasty and spliced to the cartilaginous framework that was sculpted during the first stage. The conchal bowl is deepened as well, and the tragus is refined.

Second stage consists of forming earlobe (otherwis 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.

The image below shows the new ear 2 weeks after the second stage. It may be seen that the earlobe is in its natural location, the tragus is well formed, and the conchal bowl is naturally deepened.

Patient shown underwent second stage of microtia s 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).

Patients are sent home the same day, and pain medication is rarely needed. An ear dressing is placed for 3 days. Sutures are removed on postoperative day 7. Patients may resume normal activities after 1 week if the ear is protected with a protective cup. The hair may be washed soon after surgery. The third and final stage of the reconstruction may be carried out approximately 2 months later.

Stage 3: separation and elevation of ear

The final stage of the repair consists of elevating the newly formed ear from the side of the head. A full-thickness skin graft is harvested from the groin area so that the incision site can be hidden (see the image below).

Full-thickness skin graft is harvested from groin Full-thickness skin graft is harvested from groin area in order to hide incision site.

An incision is made behind the newly constructed ear, and the undersurface is undermined. A small, custom-designed alloplastic wedge is placed behind the ear and covered with a conchal-based flap. The full-thickness skin graft is then placed and sutured with 6-0 polypropylene sutures. Once the graft is secured, a sterile sponge is placed to prevent a seroma or hematoma. An ear dressing without pressure is applied. (See the video and the image below.)

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.
Once microtic ear is separated and elevated, ears 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.

The surgical procedure lasts approximately 2 hours, and the patient goes home the same day with pain medication as needed and antibiotics. The sponge behind the ear is removed in 1 week, and the sutures are removed 1 week later. Contact sports are avoided for about 4 weeks. The hair may be washed a few weeks after surgery.

The images below are "before" and "after" pictures of a patient with grade III microtia and atresia treated by means of the natural cartilage technique.

Patient with most common type of microtia (ie, gra Patient with most common type of microtia (ie, grade III microtia and atresia) before surgical reconstruction.
Patient with grade III microtia and atresia after 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.

Bilateral microtia or atresia

Children born with bilateral microtia or atresia are treated differently from those with unilateral microtia or atresia. Computed tomography (CT) of the temporal bones is performed before the onset of the first microtia surgery. The CT scan will reveal the anatomy of the outer, middle, and inner ear.

To minimize surgical intervention, combination stages are performed in patients with bilateral microtia. In other words, after the first stage of surgery on one side, the contralateral first stage and the ipsilateral second stage may begin within 4-6 weeks. After an interval of 2 months, the next combination stage, consisting of the contralateral second stage and the ipsilateral third stage, is performed. After another 2 months, the contralateral third stage is performed.

After 3-4 months, the ear canal may be drilled to improve hearing status (if the anatomy is favorable). Although beginning the middle-ear surgery early for hearing improvement is advantageous, the surgical risks must be considered. The most common risk associated with middle-ear surgery is restenosis of the external auditory canal. The most detrimental risk is actual damage to the facial nerve, though such damage is extremely rare. As a result, the CT scan must be examined carefully so that the location of the facial nerve can be tracked.

Ongoing technical developments

Technical options for microtia reconstruction are being developed and studied. Cao et al described a tissue-engineered autologous cartilage to use in place of harvested rib cartilage.[4] In this approach, autologous chondrocytes are harvested and seeded into a mesh that is shaped in the form of the normal ear. This new framework is then inserted under the microtic vestige as a first-stage procedure. If successful in the long term, this form of cartilage harvesting may supplant present-day rib harvesting and may completely replace the plastic porous polyethylene alloplastic implant. Other investigators have also reported promising results with similar approaches.[5, 6]

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Complications

Natural cartilage technique

If done correctly, this option gives the best and longest-lasting results with minimal complications. Potential complications include an unpleasant aesthetic result, infection, and bleeding. A pneumothorax during rib excision is a very rare complication.

Alloplastic porous polyethylene implant

Because the polyethylene is a foreign material, the risk of infection, rejection, and fracture (see the video below) exists for life. Loss of hair, loss of sensation, or an unsightly scar on the scalp can also occur as a complication of the harvesting of the temporoparietal fascia flap. Another common complaint is that the plastic material used to reconstruct the ear is very hard and that it hurts to sleep on the side of the alloplastic implant.

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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Consultations

There are several specialists that should be involved in the management of a child with microtia and atresia, including the following:

  • Neonatologist - This specialist is in charge of alerting pediatrician and other specialists to the microtia
  • Primary care physician (pediatrician or family physician) - This individual is usually the primary doctor who refers to all of the multiple specialties
  • Geneticist - This individual performs a detailed family history, rules out other syndromes, and assesses the chances of having another child with microtia
  • Microtia/ear reconstruction surgeon - This specialist must be involved early on so as to educate the parents about the treatment of microtia
  • Otolaryngologist/neurotologist - This specialist monitors for ear infections and hearing loss and is also involved with ear canal surgery recommendations
  • Audiologist - This professional performs hearing tests
  • Speech/language therapist - This professional is involved if there is a concern about speech delay
  • Psychologist (rarely necessary) - The participation of this specialist may be required if there are psychological issues with the child or the family
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Contributor Information and Disclosures
Author

Arturo Bonilla, MD Founder and Director, Microtia - Congenital Ear Deformity Institute

Arturo Bonilla, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alan D Murray, MD Pediatric Otolaryngologist, ENT for Children; Full-Time Staff, Medical City Dallas Children's Hospital; Consulting Staff, Department of Otolaryngology, Children's Medical Center at Dallas, Cook Children's Medical Center; Full-Time Staff, Texas Pediatric Surgery Center, Cook Children's Pediatric Surgery Center Plano

Alan D Murray, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, American Academy of Pediatrics, American College of Surgeons, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Ravindhra G Elluru, MD, PhD Professor, Wright State University, Boonshoft School of Medicine; Pediatric Otolaryngologist, Department of Otolaryngology, Dayton Children's Hospital Medical Center

Ravindhra G Elluru, MD, PhD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, Association for Research in Otolaryngology, Society for Ear, Nose and Throat Advances in Children, Triological Society, American Society for Cell Biology

Disclosure: Nothing to disclose.

Acknowledgements

Orval Brown, MD Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas

Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

References
  1. Craig MJ. Mandibulo-facial dysostosis. Arch Dis Child. 1955. 30:391-397.

  2. Constantine KK, Gilmore J, Lee K, Leach J Jr. Comparison of microtia reconstruction outcomes using rib cartilage vs porous polyethylene implant. JAMA Facial Plast Surg. 2014 Jul-Aug. 16 (4):240-4. [Medline].

  3. Cabin JA, Bassiri-Tehrani M, Sclafani AP, Romo T 3rd. Microtia reconstruction: autologous rib and alloplast techniques. Facial Plast Surg Clin North Am. 2014 Nov. 22 (4):623-38. [Medline].

  4. Cao Y, Vacanti JP, Paige KT, et al. Transplantation of chondrocytes utilizing a polymer-cell construct to produce tissue-engineered cartilage in the shape of a human ear. Plast Reconstr Surg. 1997 Aug. 100(2):297-302; discussion 303-4. [Medline].

  5. Reiffel AJ, Kafka C, Hernandez KA, Popa S, Perez JL, Zhou S, et al. High-fidelity tissue engineering of patient-specific auricles for reconstruction of pediatric microtia and other auricular deformities. PLoS One. 2013. 8 (2):e56506. [Medline].

  6. Zhang L, He A, Yin Z, Yu Z, Luo X, Liu W, et al. Regeneration of human-ear-shaped cartilage by co-culturing human microtia chondrocytes with BMSCs. Biomaterials. 2014 Jun. 35 (18):4878-87. [Medline].

  7. Aguilar EF. Auricular reconstruction of congenital microtia (grade III). Laryngoscope. 1996 Dec. 106(12 Pt 2 Suppl 82):1-26. [Medline].

  8. Avelar JM, Psillakis JM. Microtia: total reconstruction of the auricle in one single operation. Br J Plast Surg. 1981 Apr. 34(2):224-7. [Medline].

  9. Brent B. Technical advances in ear reconstruction with autogenous rib cartilage grafts: personal experience with 1200 cases. Plast Reconstr Surg. 1999 Aug. 104(2):319-34; discussion 335-8. [Medline].

  10. Cilingir M, Malkoc C, Duman A, et al. Microtia and pectoralis muscle agenesis. Plast Reconstr Surg. 2004 Jun. 113(7):2222-4. [Medline].

  11. Harris J, Kallen B, Robert E. The epidemiology of anotia and microtia. J Med Genet. 1996 Oct. 33(10):809-13. [Medline].

  12. Lynberg MC, Khoury MJ, Lammer EJ, et al. Sensitivity, specificity, and positive predictive value of multiple malformations in isotretinoin embryopathy surveillance. Teratology. 1990 Nov. 42(5):513-9. [Medline].

  13. More V, Ahuja SR, Kulkarni HV, Kulkarni MV. Bilateral anotia with congenital hypothyroidism. Indian J Pediatr. 2004 Apr. 71(4):369-70. [Medline].

  14. Nagata S. Total auricular reconstruction with a three-dimensional costal cartilage framework. Ann Chir Plast Esthet. 1995 Aug. 40(4):371-99; discussion 400-3. [Medline].

  15. Shih JC, Shyu MK, Lee CN, et al. Antenatal depiction of the fetal ear with three-dimensional ultrasonography. Obstet Gynecol. 1998 Apr. 91(4):500-5. [Medline].

  16. Smithells RW, Newman CG. Recognition of thalidomide defects. J Med Genet. 1992 Oct. 29(10):716-23. [Medline].

  17. Song Y, Song Y. An improved one-stage total ear reconstruction procedure. Plast Reconstr Surg. 1983 May. 71(5):615-23. [Medline].

  18. Tanzer RC. Correction of microtia with autotenous costal cartilage. In: Tanzer RC, Edgerton MT, eds. Symposium on Reconstruction of the Auricle. St. Louis:. Mosby. 1974:46-57.

  19. Yanai A, Fukuda O, Yamada A. Problems encountered in contouring a reconstructed ear of autogenous cartilage. Plast Reconstr Surg. 1985 Feb. 75(2):185-91. [Medline].

 
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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).
Microtia, grade II. Pinna is smaller and less developed than in grade I. Helix may not be fully developed. Triangular fossa, scaphae, and antihelix have much less definition.
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.
Anotia (total absence of pinna).
Three-dimensional sonogram of fetus with microtia.
Arrow in CT scan indicates lack of ear canal (atresia) on left side. Note normal ear canal on right side.
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.
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.
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.
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.
Image shows carved cartilaginous framework from small portions of ribs 6, 7, and 8.
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.
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.
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.
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).
Full-thickness skin graft is harvested from groin area in order to hide incision site.
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.
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.
Patient with most common type of microtia (ie, grade III microtia and atresia) before surgical reconstruction.
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.
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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