Congenital Aural Atresia Surgery Techniques
•Download as PPTX, PDF•
4 likes•2,829 views
presented in local chapter of ENT conference ... aural atresia ... congenital meatal stenosis
Recommended
More Related Content
What's hot
What's hot (20)
Viewers also liked
Viewers also liked (10)
Similar to Congenital Aural Atresia Surgery Techniques
Similar to Congenital Aural Atresia Surgery Techniques (20)
More from ENT Resident
Recently uploaded
Recently uploaded (20)
Congenital Aural Atresia Surgery Techniques
- 2. CONGENITAL AURAL ATRESIA Dr Zeeshan Ali Resident in Department of ENT Head and Neck Surgery CMH Rawalpindi
- 3. Case 1
- 4. Case detail Displayed with informed consent of patient
- 5. BERA
- 6. CT Scan
- 7. Surgery Displayed with informed consent of patient
- 8. Surgery Displayed with informed consent of patient
- 9. Surgery Displayed with informed consent of patient
- 10. Surgery Displayed with informed consent of patient
- 11. Surgery Displayed with informed consent of patient
- 12. Surgery Displayed with informed consent of patient
- 13. Surgery Displayed with informed consent of patient
- 14. Surgery Displayed with informed consent of patient
- 15. Surgery Displayed with informed consent of patient
- 16. Surgery Displayed with informed consent of patient
- 17. Surgery Displayed with informed consent of patient
- 18. Surgery Displayed with informed consent of patient
- 19. Surgery Displayed with informed consent of patient
- 20. Surgery Displayed with informed consent of patient
- 21. Surgery Displayed with informed consent of patient
- 22. POSTOPERATIVE CARE Displayed with informed consent of patient
- 23. Case 2
- 24. Case detail Displayed with informed consent of patient
- 25. Audiometry
- 26. CT scan
- 27. X ray mastoid
- 28. Surgical steps
- 29. Surgery Displayed with informed consent of patient
- 30. Surgery Displayed with informed consent of patient
- 31. Surgery Displayed with informed consent of patient
- 32. Surgery Displayed with informed consent of patient
- 33. Surgery Displayed with informed consent of patient
- 34. Surgery Displayed with informed consent of patient
- 35. Surgery Displayed with informed consent of patient
- 36. Surgery Displayed with informed consent of patient
- 37. Surgery Displayed with informed consent of patient
- 38. Surgery Displayed with informed consent of patient
- 39. Surgery Displayed with informed consent of patient
- 40. Post operative Displayed with informed consent of patient
- 41. Post operative Displayed with informed consent of patient
- 42. CASE DISCUSSION
- 43. Epidemiology 1 in 10,000 to 15,000 births 10% associated with syndrome Unilateral vs Bilateral 30% are bilateral Atresia : Microtia, 7:1
- 44. Embryology ◦ Treacher Collins syndrome ◦ brachio-otorenal syndrome ◦ Stickler syndrome
- 45. Embryology ◦ Treacher Collins syndrome ◦ brachio-otorenal syndrome ◦ Stickler syndrome ◦ Crouzon syndrome ◦ Noonan syndrome ◦ Foetal alcohol syndrome
- 46. Embryology ◦ Treacher Collins syndrome ◦ brachio-otorenal syndrome ◦ Stickler syndrome ◦ Crouzon syndrome ◦ Noonan syndrome ◦ Foetal alcohol syndrome ◦ Goldenhar’s Syndrome ◦ Hemifacial Microsomia
- 47. Embryology Inner ear, middle ear, and external ear develop independently
- 48. Embryology ‘The better developed the external ear is, the better developed is the middle ear’
- 49. Development of the Auricle
- 51. Embryology
- 52. Embryology First Branchial Arch- (Meckel’s) ◦ Malleus ◦ Incus body Second Arch (Hyoid) (Reicherts) ◦ Long process of incus ◦ Stapes suprastructure Foot plate from the otic capsule and 2nd arch
- 53. Types of aural atresia
- 54. Altman classification Group 1 Minor Aplasia- incomplete recanalization
- 55. Altman classification Group 2 Moderate Aplasia- tympanic bone has developed but has failed to recanalize
- 56. Altman classification Group 3 Severe Aplasia- complete absence of the external canal
- 57. De la Cruz Classification HRCT based Mastoid Pneumatization Inner ear normality Facial nerve Footplate
- 58. De la Cruz Classification Minor Malformations Normal mastoid pneumatization Normal oval window/footplate Good facial nerve–footplate relationship Normal inner ear
- 59. De la Cruz Classification Major Malformations Poor pneumatization Abnormal or absent oval window/footplate Abnormal course of facial nerve Abnormalities of inner ear
- 60. Grading System (Jahrsdoerfer) Variable Points Stapes Present 2 Oval Window Open 1 Facial Nerve 1 Middle Ear Space 1 Mastoid Pneumatization 1 Malleus/Incus Complex 1 Incudostapedial Joint 1 Round Window 1 External Ear Appearance 1 Total Points 10
- 61. Prognostic Rating Scale 10 Excellent 9 Very good 8 Good 7 Fair 6 Marginal ≤5 Poor de Alarcon A, Jahrsdoerfer RA, Kesser BW. Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 2008;29:23–28.
- 62. Schuknecht’s classification Type A Type B Type C Type D
- 63. Schuknecht’s classification Type A Type B Type C Type D
- 64. Schuknecht’s classification Type A Type B Type C Type D
- 65. Schuknecht’s classification Type A Type B Type C Type D
- 66. Chiossone’s classification Type I - the fossa in normal position Type II - moderately displaced
- 67. Chiossone’s classification Type I - the fossa in normal position Type II - moderately displaced Type III - fossa overlaps the middle ear
- 68. Chiossone’s classification Type I - the fossa in normal position Type II - moderately displaced Type III - fossa overlaps the middle ear Type IV - lack of mastoid pneumatization
- 70. Management
- 71. Management
- 72. Prerequisite for surgery CT scan Audiometry
- 73. Urgent surgery Congenital cholesteatoma Draining postoperative atretic ear Acute facial palsy
- 74. Timing of surgery 6 year recommended Exostosis formation Less patient cooperation Sufficient costal cartilage Mastoid well pneumatised
- 75. Complications Lateralization of TM 3.4% Meatal Stenosis 3.8% High-tone SNHL 7.5% Facial nerve palsy 1%
- 76. Unilateral atretic ear Decreased incidence of ‘major ‘complications A rewarding operation Offer a clean & dry ear Binaural hearing ‘Minor’ unilateral atresia In early childhood
- 79. Data Jan 2008-Dec 2014 36% 50% 14% Atresia Type I Type II Type III
- 80. 0 1 2 3 4 5 6 7 8 9 10 CMH Regional Intl Data Jan 2008-Dec 2014 Nishizaki K, Masuda Y, Karita K.Surgical management and its post-operative complications in congenital aural atresia.Acta Otolaryngol Suppl.1999;540:42-4. Oliver ER, Hughley BB, Shonka DC, Kesser BW.Revision aural atresia surgery: indications and outcomes. Otol Neurotol. 2011 Feb;32(2):252-8. doi: 10.1097/MAO.0b013e3182015f27 Restenosis Otorrhea Facial nerve paralysis SNHL
Editor's Notes
- Respected teachers and my fellow colleagues AOA. I dr zeeshan resident in ENT head and neck surgery department CMH rwp will be presenting two cases of congenital aural atresia with different surgical scenarios.
- Case no 1 is A 6 year old boy brought to our institute by his consanguineous parents for scheduled ear surgery for bilateral hearing loss since birth. on examination altman grade 3 aural atresia with marx grade 3 microtia were seen bilaterally.
- BERA revealed the boy having 60 and 70 db hearing threshold in right and left ear respectively. free field hearing test reveal 60 and 80 db in right and left ear respectively.the boy had been wearing soft band bone conducting hearing aid since birth and studying in a special education school .speech therapist suggest adequate receptive and expressive language with good attention span.
- Ct san revealed partially canalised right external auditory canal with aerated middle ear and intact ossicles where as left middle ear showed abnormal configuration with no definitive ossicles.
- The boy was graded 9 on Jahrsdoerfer system and planned for surgery of the right ear. Associated risks of total deafness and facial nerve paralysis were explained to the parents.
- lateral face and the skin graft donor site were prepared and draped. A 1/2-inch swath of hair is shaved around the external ear
- An endaural incision is made and Soft tissue was elevated off the mastoid process in a posterior to anterior direction.
- Temporalis facia garft was harvested.
- The temporal root of the zygomatic arch and the glenoid fossa were identified and the cribriform area of the mastoid process was used as a landmark for drilling
- Drilling was done by staying superior and anteriorly.Care was taken to hug the tegmen and the glenoid fossa
- Dense atretic bone was found and followed medially.
- fused incus-malleus complex was encountered At a depth of approx 1.5 cm
- body of the incus was identified and confirmed by gentle Palpation.
- fused malleus head and incus body were found to be mobile on palpation. diamond bur was Used to thin the atretic plate to eggshell thickness and gently picked away in small pieces.
- facial nerve was seen having a short vertical segment while making a sharp curve anteriorly. Normal mobile stapes suprastructure was confirmed by gentle palpation. .
- peripheral bone was then drilled to gain room for the placement of the fascial graft.
- fascia graft was placed directly on the ossicular Mass so as it was Approximately in center of the new tympanic membrane.
- a “U”-shaped pedicle flap hinged at the tragal remanent was then positioned into the new ear canal and sutured to a cuff of periosteum.
- Following proper placement of the meatus the external ear was stabilized with subcutaneous sutures and ear canal was packed with spongiston wicks and BIPP packs.mastoid dressing was applied.
- The patient was discharged on the 2nd postop day and sutures were removed on 1 postop week. here the patient is seen in the 2nd week showing granulating wound with healthy pedicle skin.silver nitrate was applied to the granulations and EAC was packed with antibiotic impregnated gelfoam. the boy is scheduled to have his 1st audiogram in 3rd week postoperatively.
- Case no 2 is An 18 year-old female patient referred to our institute with painful post-auricular discharging sinus on her left side associated with swelling and redness around it. examination revealed that the patient had aural stenosis dating since birth and grade II microtia with acute mastoiditis that led to sinus formation on the left side,.. the right ear was completely normal.
- Pure tone audiogram showed conductive hearing loss with an air-bone gap of 50 dB on the left side and near normal hearing of right ear
- Ct scan show soft tissue density in the left external auditory meatus with intact ossicles.
- X ray mastoid showed a sclerosed left mastoid with an opacity in the attic region while a well pneumatised right mastoid.
- Patient was prepped for surgery after explaining risks of total deafness and facial nerve paralysis.
- lateral face and the skin graft donor site were prepared and draped. A 1/2-inch swath of hair is shaved around the external ear
- modified pre-auricular incision was made and unhealthy skin around the sinus excised.
- Temporalis fascia graft was harvested and dried on petri dish.
- Cortical mastoidectomy was carried out to reveal an automastoidectomy cavity extending into the attic area.
- Mastoid cavity was Filled with a large cholesteatoma sac
- Whole of the cholesteatoma sac was carefully removed.
- here is the magnified view of the isolated Cholesteama sac.
- the middle ear was entered via an atticoantrostomy approach And atretic plate found which was carefully drilled away. The ossicular mass in the epitympanum was meticulously dissected free of the atretic plate and left intact after ensuring its mobility.
- The horizontal facial nerve was seen medial to the ossicular mass.
- then temporalis fascia graft was used to cover the ossicular mass and line the mastoid cavity.
- Reconstruction of the external auditory canal was done through removal of the bone lateral to the middle ear space with meatoplasty of the cartilaginous portion
- No intra-operative or post-operative complications were seen.
- pack was removed on the 10th post-operative day.granulations were seen in the mastod cavity and repacking was done with BIPP. Pt is on periodic visits for the regular cleaning of the mastoid cavity.
- Congenital aural atresia occurs once in every 10,000 births. Unilateral atresia is seven times more common than bilateral atresia. Aural atresia is associated with a recognizable syndrome in about 10% of cases. In about 5% of nonsyndromic cases, the birth defect is inherited.
- Syndromic conditions associated with congenital aural atresia include: • Treacher Collins; • brachio-otorenal; • Sticklers;
- • Crouzons; • Noonan syndrome; • foetal alcohol syndrome;
- • CHARGE syndrome coloboma, heart choanal atresia, developmental and growth retardation, genito-urinary malformations and ear anomalies (CHARGE)
- The inner ear, middle ear, and external ear develop independently and in such a way that deformity of one does not presuppose deformity of another. Most frequently, abnormalities of the outer and middle ear are encountered in combination with a normal inner ear.
- Some of the literature supports the notion that microtia grade indicate the status of middle ear development.
- Growth of mesenchymal tissue from the first and second branchial arches forms six hillocks around the primitive meatus that fuse to form the auricle .
- Microtia is a result of first and second branchial arch Anomalies and is classified by Marx into 4 grades.
- During the second month The first branchial pouch grows outward to form the middle ear cleft while a solid core of epithelium migrates inward from the rudimentary pinna toward the first branchial pouch.
- Meckel’s cartilage form the malleus and incus body. Reichert’s cartilage forms the long process of incus and the stapes superstructure while The footplate has a dual origin from the second arch and the otic capsule.
- Congenital aural atresia can range in severity from a thin membranous canal atresia to complete lack of tympanic bone, depending on the time of arrest of intrauterine development
- Of historical significance is a classification by Altmann. In this system, atresias are categorized into three groups, In Group 1 Some part of the EAC is hypoplastic.
- In Group 2 The EAC is completely absent, the tympanic cavity is small, and its content is deformed, and the “atresia plate” is partially or completely osseous
- In Group 3 The EAC is absent, and the tympanic cavity is markedly hypoplastic or missing.
- The De la Cruz classification includes surgical feasibility guidelines using HRCT, taking into consideration mastoid pneumatization, inner ear normality, facial nerve and footplate relationship.
- The malformations are divided into minor and major malformations.
- Jahrsdoerfer and colleagues developed a widely used point grading system to guide surgeons in preoperative assessment of the best candidates for hearing improvement. Point allocation is based primarily on the findings on HRCT.
- Jahrsdoerfer proposed that the best results are achieved with a score of 8 or better.
- Schuknecht’s system is based on a combination of clinical and surgical observations. Type A atresia is limited to the fibrocartilaginous.
- type B there is narrowing of the fibrocartilaginous and bony EAC.
- Type C is a totally atretic ear canal, but a well-pneumatized tympanic cavity.
- Type D is a hypopneumatic atresia which is common in dysplasias such as Treacher Collins syndrome.
- Chiossone’s classification is based primarily on the location of the glenoid fossa. Patients with types I and II are ideal surgical candidates.
- Type III cases have a tendency toward graft lateralization.
- Patients with type IV are not surgical candidates
- Where one congenital abnormality is found, others must be sought. After physical examination,evaluation of auditory function should be performed using auditory brainstem response audiometry within the first few days of life. initial evaluation of an older individual is with Audiometry and high-resolution CT scan
- In bilateral cases, a bone-conduction hearing aid should be applied as soon as possible, ideally in the third or fourth week of life. A child with aural atresia and associated cephalic abnormalities surgical correction has poor results so BAHA is used in such cases.
- Prompt and careful counseling of the parents of a child is necessary to alleviate concerns. The child should be enrolled in special education at an early age to maximize speech and language acquisition, in preparation for “mainstreaming” at preschool age.
- There are two requirements for planning surgery in congenital aural atresia: radiographic three-dimensional evaluation of the temporal bone and audiometric evidence of cochlear function
- Other conditions mandating prompt surgical intervention are congenital cholesteatoma, a draining postoperative atretic ear, and acute facial palsy. The CT scan should always be reviewed for cholesteatoma,which necessitates surgery at any age
- In bilateral or unilateral atresia, auricular reconstruction and atresiaplasty are recommended at 6 years of age. Before this age, there may be a tendency to form exostosis-like bony growth and there is less patient cooperation. the costal cartilage has developed sufficiently to allow for reconstruction and the mastoid has become pneumatized.
- Complications of surgery include Lateralization of the tympanic membrane ,stenosis of the meatus ,high-tone SNHL and facial nerve palsy.
- Elective surgery in unilateral atresia is a controversial topic .now a day it is rewarding for the surgeon and the patient ….offering benefits of a clean, dry ear with binaural Hearing. it is done in patient with “minor” unilateral atresia it may be offered in childhood with the parents’ consent.
- A total of 21 patients of both sexes with congenital aural atresia were operated from January 2008 to Dec 2014 with a male to female ratio of 2.5:1
- 26 patients had unilateral atresia while 16 patients had bilateral aural atresia with a ratio of 1.6:1.
- Type I meatal atresia was present in 36 % patients, while 50% patients had type II meatal atresia and 14% patients had type III meatal atresia.
- Our data was compared with regional and international study which show comparable results. our results showed low rate of restenosis in comparison to the regional and international study.