Congenital external ear deformities and reconstruction. Dr. Jason Goodwin Dr. David Chang

Transcription

1 Congenital external ear deformities and reconstruction Dr. Jason Goodwin Dr. David Chang

2 Overview Ear development Congenital ear deformities Classification systems Evaluation Reconstruction options

3 Microtia Abnormal development of external ear resulting in deformed auricle Ranges from mild deformity to complete agenesis of ear. 1: births Most often unilateral Right side predominance (twice left) 2.5:1 Male to female ratio Associated with congenital aural atresia Degree of external ear malformation mirror EAC and middle Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61 80

4 Demographics Associated with other anomalies in ~50% Various syndromes of H&N Increased incidence in Japanese, Native American, Hispanic populations Causes Teratogens (Vit A, thalidomide), intrauterine infections (Rubella, CMV etc.), vascular insults to fetus Genetic anomalies Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61 80

5 Ear landmarks

6 Auricle embryology 6 Hillocks of His (mesoderm) 1st pharyngeal arch 1: Tragus 2: Helical crus 3: Helix 2nd pharyngeal arch 4: antihelix 5: antihelix 6: antitragus

7 Ear embryology Week 6: Hillocks begin to form around 1 st branchial pouch Starts low and anteriorly then migrates dorsally and cephalad during months 2-3 In final position at month 5

8 Evaluation Typically present early in life Focus on mandible, eyes, spine rule out other anomalies that may lead to a specific syndrome Cleft lip/palate: 4.3% Facial nerve function Some weakness found in up to 15% 43% of those involve multiple branches Hearing assessment Mostly conductive, but 10-15% will have some sort of SNHL Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) Brent, B.

9 Evaluation Careful counseling to family Protect good ear aggressively Additional studies CT T-bone Renal ultrasound: 4% Cardiovascular: 2.5% Genetics Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) Brent, B.

10 Associated syndromes Up to 50% association with syndrome Often in conjunction with aural atresia Higher likelihood in facioauriculovertebral syndromes Goldenhar Hemifacial microsomia Treacher Collins Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61 80

11 Name the syndrome A. Treacher Collins B. Hemifacial microsomia C. Goldenhar D. Down Hemifacial microsomia: congenital disorder that affects the development of the lower half of the face, most commonly the ears, the mouth and the mandible.

12 Name the syndrome A. Treacher Collins B. Hemifacial microsomia C. Goldenhar D. Down Treacher-Collins: rare autosomal dominant congenital disorder characterized by craniofacial deformities The typical physical features include downward slanting eyes, micrognathia (a small lower jaw), conductive hearing loss, underdeveloped zygoma, drooping part of the lateral lower eyelids, and malformed or absent ears.

13 Name the syndrome A. Treacher Collins B. Hemifacial microsomia C. Goldenhar D. Down Goldenhar: Oculo-auricular-vertebral syndrome. Characterized by incomplete development of the ear, nose, soft palate, lip, and mandible. It is associated with anomalous development of the first branchial arch and second branchial arch. Common clinical manifestations include limbal dermoids, preauricular skin tags, and strabismus.

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15 Microtia classification system Multiple classification systems exist Some incorporate EAC, middle ear, or other syndromic features into scoring system Most common is one that only takes external appearance into account (Weerda) Practically the helix/antihelix, conchal bowl, tragus, and lobule are the structures the reconstructive surgeon wishes to repair and should be considered in staging Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN

16 Microtia classification system Type I: Mild deformity, major structures present to some degree, no tissue needed Type II: major structures present, but tissue deficit to degree additional tissue needed Type III: Few recognizable landmarks, lobule usually present to some degree Type IV: anotia Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN

17 Type I Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN

18 Type II Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN

19 Type III Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN

20 Type IV (Anotia) Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN

21 Reconstructive options No surgery Prosthetic Surgery Autogenous material Synthetic implant

22 History of surgery Scattered reports of repair 2000 yrs ago with local flaps 1575 first published article with metal ear prosthesis First attempts at repairing entire auricle in late 1800s Gillies first to use rib cartilage (1920) Tanzer in mid 60s gained prominence with larger series of rib graft patients Tanzer s pupil Burt Brent expanded and modified the Tanzer technique and is now arguably the world s preeminent expert on microtia reconstruction

23 Surgery Timing of repair Minimum of 6 years old Ear is 85-90% of adult size at this age Need time for sufficient rib cartilage to grow Ribs Not 85% adult size at this age Rib cartilage retains growth potential of source Psychologic issues start around this age Any surgical correction of the external ear needs to consider the effect of surgery on possible EAC or middle ear reconstruction

24 Brent Reconstruction Technique Modification of Tanzer technique Four stage technique 1: Rib harvest of construction of framework 2: Lobule interposition 3: Elevation of ear 4: Tragus construction Minimum of two months between first and second and then three months between subsequent stages Brent, B.

25 From Brent, B.: Total auricular construction with sculpted costal cartilage. In B. Brent (Ed.), The Artistry of Reconstructive Surgery, St. Louis, 1987, The C.V. Mosby Co., pp Pre-op planning

26 First Brent Stage Harvest of rib cartilage Taken from contralateral side Fused section of ribs 6 and 7 Brent, B.

27 First stage Framework construction Brent, B.

28 First stage Incision made along area of planned ear Unusable cartilage removed Undermine widely Close with drains Brent, B.

29 Second Brent stage Lobule transposition Brent, B.

30 Third Stage Elevating the ear Incise along the posterior ear and raise it Cover post-auricular areas with FTSG, typically taken from abdomen Brent, B.

31 Fourth stage Tragus reconstruction Composite graft taken from opposite conchal bowl Brent, B.

32 Nagata Reconstruction Multiple variations of Brent technique, Nagata being most popular Two-stage technique Stage I: fabrication of auricular framework from ipsilateral costal cartilage, tragus reconstruction, and lobule transposition Stage II: framework elevation

33 Nagata first stage

34 Nagata first stage

35 Alloplastic material In past implants have been made from: wire mesh, steel wire, acrylic, silastic, teflon All of these had unacceptably high extrusion rates Medpor started in early 1990s Typical Medpor surgery is a two stage surgery

36 Medpor reconstruction No rib harvest Earlier age of repair Excellent contour and shape Shorter learning curve? Does not grow with patient Implant extrusion Need for TP flap

37 Prosthetic ear Osseo-integrated anchoring device Indication: Failed autogenous reconstruction Severe soft- tissue/skeletal hypoplasia Low or unfavorable hairline Acquired total or subtotal auricular defect, usually in adults cartilage may be unfavorable Prosthesis changes every 2 to 3 years $ Meticulous hygiene at skin/implant interface Preclude future autogenous reconstruction

38 Prosthetic ear

39 Prosthesis Advantages Avoids chest surgery May look very realistic May be of value for an older patient who does not want surgery or is at risk for a surgical procedure Disadvantage Does not grow with patient Still requires surgery (unless adhesive-type is used) Screws are drilled on side of head and a bar is placed to hold the prosthetic ear Color mismatch if skin tans The patient will never feel like the ear is part of his/her body, although this may not be the case in a poorly reconstructed ear or an ear affected by trauma

40 Complications Pneumothorax from rib harvest Infection Hematoma Skin loss Implant extrusion (Medpor)

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