MANAGEMENT OF CONGENITAL MICROTIA

WITH MEATOPLASTY AND TYMPANOPLASTY

Rachmad suhendra, Delfitri Munir

INTRODUCTION
The first attempt to surgically correct a congenital aural atresia was made by Thomson
in 1843 in England. Althought 2/3 of the cases thick atresia plate was encountered, a third,
documented what was presumably a tympanic membrane, although it appears that stenosis
became a problem postoperatively. In 1883, Kiesselbach attempted an atresia repair on a
child that resulted in facial paralysis. It was not until 1947 with the work of Pattee and
Ombredonne that a significant improvement in hearing could be obtained with surgery. Pattee
reported that fixation of the stapes via the lateral chain to the atresia plate was responsible for
these patients’ deficcits. Ombredonne devised an approach through the mastoid in order to
fenestrate the lateral semicircular canal, and there was reportedly a good hearing results.
Shambaugh, in 1967, recommended surgery in unilateral case only if the cochlear was
reserved and allowing hearing improvement to return the approach that of the normal ear
range of 25 dB. Jahrsdoerfer , in 1978, reported the first large series using the anterior
approach for congenital aural atresia repair, whereby the middle ear cavity was entered
directly via a newly-creaed external canal without performing a mastoidectomy (Fernandez
A.D, 1995).
Atresia of the ear canal with middle ear anomalies can occur in isolation or in
association with microtia or craniofacial dysplasia. The reported incident is 1 in 10,000 to
20,000 births (Lambert P.R, 2006). Microtia deformity has a right-ear predominance (right-
to-left ratio of 3:2) and affecting boys more comparred to girls with a ratio of 2.5:1.
Unilateral cases outnumber bilateral cases by a ratio of 4:1 (Yazdi A.K, a.l, 2007).

CASE REPORT
P 14-year old boy was presented to our department with a chief complaint of Microtia
from both ears since he was born. Microtia was accompanied by atresia of ears. He did
complain of some hearing loss with difficulty in localizing sounds, but was doing fairly well
with excellent school performance.

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Fig.1. Microtia auricular duplex Fig.2. Atresia auricular

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 Fig 3. Temporal bone CT Scan Imaging Fig 4. Pure tone audiogram
On examination, we found anomalous formation of structures of the external ears
(microtia) followed by aural atresia from both ears Examination of nasopharynx and nasal
cavity was normal. He also complained that he felt uncomfortable with his formation on both
ears. Hematological investigation was normal.
Pure tone audiogram showed bilateral conductive hearing loss with severe conductive
hearing loss on the both ears (80 dB). Temporal CT Scan showed aural atresia bilateral on the
right mastoid.
Patient was diagnosed with bilateral microtia with atresia auricular on the both ears
and meatoplasty surgery is planned surgery was done under general anaesthesia. A
postauricular incision was then drilled into mastoid cortex until was located the antrum, next
candidate ear canal anterior was drilled until position of membrane tympani was located. Os
maleus and stapes we found but incus. A hole was drilled on the caput of malleus and stapes
ossicular was modified positioned on the stapes footplate was covered with malleus and the
incus was not present.

Fig.5. post auricular incision Fig. 6. Drill until antrum and we drill canal
anterior candidate.

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Figure 10. Ossicular modificated, positioned on the stapes footplate covered
with malleus, when the incus is not present.

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Fig.12. flap used to closed wall external canal auricular

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 Fig. 13. suture and tampon canal auricular

Figure 14. Pure tone audiometric after surgery.

Next, meatoplasty was done. A flap, based laterally at the concha was raised from the
posterior and superior meatal wall and turned into the mastoid cavity to cover the area of the
facial ridge. The tympanic cavity was obliterated with fascia temporalis profunda and fixated
with dental spongiostan. The postauricular skin wound was closed in two layers.
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 After surgery, Patient was prescribed with antibiotics, steroids and analgetics. Pure
tone audiogram showed bilateral conductive hearing loss with moderate conductive hearing
loss on the left ears (50 dB)

DISCUSSION
Atresia of the ear canal with middle ear anomalies can occur in alone or in
association with microtia or craniofacial dysplasia. The reported incidence is 1 in 10,000 to
20,000 births. Aural atresia is always associate with other congenital anomalies. Aural atresia
is usually bilateral in one third of the case, and each side can vary in complexity. Patients
with congenital aural atresia are classified into auricular development and external
canal/middle ear development. Deformity of the auricle is divided into three grades. Grade I
microtia represents a minor malformation, with the auricle being smaller than normal but
with all parts disc.ernible. In grade II microtia, the auricle is represented by a curving or
vertical ridge of tissue. In grade III microtia, any resemblance to an auricle is lost, and only a
small rudimentary soft tissue structure is present (Lambert PR, 2006). This patient is
diagnosed with grade III microtia which is represented by represented by anauricle and the
presence of small rudimentary soft tissue structure.
Congenital aural atresia occurs in every 3300 to 10000 births, mostly unilaterally
(70%-90%), and is associated with a recognizable syndrome in 10% of cases (Adunka OF,
Buchman CA, 2011).
Facial nerve abnormalities are most commonly found in atresia patient. The
abnormalities include complete dehiscence of the tympanic segment, inferior displacement of
the tympanic segment, and anterior, lateral displacement of the mastoid segment. The degree
of external ear deformity provides some indication of facial nerve development, with a higher
incidence of facial nerve anomalies occurring in patients with more severe microtia (Lambert
PR, 2006). In this patient, we found a facial nerve exposure and after giving treatment of
steroid paralysis returned to normal.
Most surgeons has recommended operating as the child approaches school age and,
depending on the hearing result an and on the second ear within the next several years.
Although the selection criteria are not as stringent as in unilateral cases, careful patient
screening is essential for routinely satisfactory results.
If elected, surgery can be performed as early as 6 to 7 years of age. By this time,
accurate audiometric tests have been obtained, pneumatization of the temporal bone is well
advanced, and most children are able to cooperate.
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CONCLUSION
Patient was diagnosed with grade III congenital microtia and repair was performed to this
patient. Facial paralysis was found after the operation but a course of steroid therapy has
improved the complication.

DAFTAR PUSTAKA
Alireza Karimi-Yazdi MD, al, in Original article”Comparison of Microtia Reconstruction
Surgery with Autograft versus Homograft, Archives of Iranian Medicine, Vol.10, Number,
Januari 2007.
Aquilar E.F, Surgery of Congenital Facial Anomalies Auricular Reconstruction in Congenital
Anomalies of the Year, In: American Boar of Facial Plastic Surgery, Vol.9, Number 1,
Februari, 2011.

Adunka OF, Buchman C.A; Otology, Neurotology and Lateral Skull Base Surgery. Newyork,
p.107-16.

El-Rifaie K.M, Total Reconstruction of The Auricle In Lobule-Type Microtia. The

Departement of Surgery, Faculty of Medicine, Cairo University. J. Plastic Reconstruction
Surgery, Vol. 27, No.2, July, 2003.p.225-230.

Fernandez A.D.1995; Congenital Aural Atresia; In: The Bobby R. Alford Departement of
Otorhinolaryngology and Communicative Science.

Lambert P. R, Congembert P. R, Congenital Aural Atresia, In: Bailey B.J, Jonsnital Aural
Atresia, In: Bailey B.J, Jonson J.T. Head aon J.T. Head and Neck Surgery Otolaryngology;
Otology. Fourth Edition. Lippincott-Riven Publishes. Philadelphia, 2006, p.2027-40.

Lambert P.R.2006; Congenital Aural Atresia; In: Bailey B.J. Head and Neck Surgery
Otolaryngology; Fourth edition ;Lippincott-Riven Publishes;Philadelphia, p.2027-40).

Parisier SC et al, Ballenger’s Otorhinolaryngplogy17 Head and Neck Surgery. Microtia,

Canal Atresia, and Middle Ear Anomalies. BC Decker Inc. 2009. P.759-67.

See G.B, Abdullah A, Ami M, Saim L. Management Of External Ear Anomalies: Congenital
Microtia And Canal Atresia. Paediatric Otorhinolaryngology (ORL). 2010; Vol. 16;
Supplementary 1.
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Yazdi A. K., Hosseini M. S., Sadeghi M., Sazgar A.A., Safikhani R.; Original Article;
Comparison of Microtia Reconstructive Surgery with Autograft Versus Homograft; Arch
Iranian Med 2007; 10 (1): 43 – 47