Microtia Repair: The Case for Surgical Reconstruction (original) (raw)
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Congenital malformations of the ear
The Indian Journal of Pediatrics, 1992
Tile external ear develops from I and II branchial arches commencing on 38th day of fetal life. The middle ear is formed from the ends of Ist pharyngeal puch and the surrounding mesenchyme, which also is part of the I and II branchial arches. The congenital defects of the external and middle ear usually occurs in combination, and many times with congenital defects of other systems. The cochlear functions i.e. bone conduction is normal in 50% of these cases, thus rehabilitation of these patients with congenital anomalies of external and middle ear is possible. The coexistence of congenital aural atresia with varying degrees of malformation of inner ear may be more frequent than generally assumed. Moderate and severe forms of congenital aural atresia area encountered in about 1 in 10,000 to 20,000 individualJ Tile more severe forms of congenital auricular malformation are always associated with meatal atresia, whereas meatal atresia may, in a few cases be seen in patients with a normal pinna. Atresia of the meatus may be membranous or osseus, membranous atresia is much less common and is characterised by rudimentary cartilagenous canal sep~ated from the middle ear by a dense structure of con
Modified Surgical Technique for Combined Congenital External and Middle Ear Malformations
International Bulletin of Otorhinolaryngology, 2008
Hypothesis: There is a positive correlation between type and intensity of combined congenital ear malformations and results of surgical reconstruction. Background: Combined congenital malformations of external and middle ear pose a lot of surgical problems and demand adequate skill and planned, fractioned therapy. The goals of these operations are: limited number of operations with minimal complications, well cosmetic result, hearing improvement and easier use of hearing aid, removal of congenital cholesteatoma, preservation of labyrinth and facial nerve. Methods: The aim of this work is to present the experience in treating patients with combined malformations of external and middle ear. In the period between 1992 and 2001 seventeen patients with this type of congenital malformations were operated. Classical dilemma who operates first, plastic surgeon or otosurgeon is solved here by complete otosurgical treatment with reduced number of operations. Results: Totally 36 operation was performed, two reoperations were made in four patients, and also three operations in three patients for the reconstruction of deformity of auricle. In seven patients costal rib cartilage was used for auricular framework. Reoperations were done during the course of auricular reconstruction (14 operations), or because of meatal restenosis (five operations), or cartilage graft necrosis (one case). Atresia was adequately solved in 14 patients, while in three cases connective tissue meatal restenosis was found. The results of reconstruction of auricular malformation were aesthetically good in 13 patients and sufficient in four patients. Conclusion: We found positive correlation between external ear and middle ear deformity, and the results of reconstruction, as well. Two thirds of patients have hearing threshold level under 30 dB, while the threshold in other patients is 30-40 dB. Residual airbone gap amounting about 20 dB was universally present.
Plastic and Reconstructive Surgery, 2005
Congenital auricular anomalies are heterogeneous, with various descriptive and eponymous terms being used. Current classification systems are useful in guiding surgical treatment of severe anomalies. However, they do not generally account for the less severe anomalies, which form the majority of congenital auricular anomalies, nor their contemporary treatment. In this article, the authors review the anatomy and embryology of the external ear and propose a simple classification of congenital auricular anomalies that encompasses all forms of congenital auricular anomalies, facilitates proper diagnosis, and guides treatment. Congenital auricular anomalies should be classified as malformational or deformational anomalies. Malformational auricular anomalies are caused by embryologic maldevelopment that occurs between the fifth and ninth week of gestation resulting in deficient and/or supernumerary auricular components. Deformational auricular anomalies result from in utero or ex utero deformational forces, including those caused by an aberrant insertion of the intrinsic or extrinsic auricular muscles. Malformational auricular anomalies generally require surgical correction during childhood or adolescence. For practical purposes, deformational auricular anomalies have a full complement of chondrocutaneous com-From the
Classification of Congenital Middle and External Ear Malformations: CT Study
The Medical Journal of The Islamic Republic of Iran, 1991
The authors used high-resolution computed tomography (HRCT) for studying 52 congenitally malformed ears in 45 children between five and 10 years of age. In six children the malformations were bilateral. The malforma tions clinically manifested as microtia, atresia of the external auditory canal and conductive deafness. Analyzing anatomical details and pathological changes on HRCT axial sections the authors established three groups of malformations. In the first group, auditory assicles were malformed in almost all cases, in the second group besides deformed auditory ossicles in majority of cases, the mastoid was apneumatised, and in the third group there were malformed auditory ossicles and the mastoids were apneumatised and in more than half of cases cavum tympani was malformed or filled with mesenchyme. These features have great importance in surgical reconstruction of congenital middle ear malformations. MJIRI, Vo1.5, No.3 & 4,105-109,199
Unclassified congenital deformities of the external ear
Indian Journal of Plastic Surgery
ABSTRACTCongenital ear deformities are a common entity. They are found in isolation or as a part of syndrome in patients. They may involve the external, middle or inner ear or in any of these combinations. Three patients of different ages presented with deformities including mirror image duplication of the superior auricle, unclassified deformities of ear lobule (wavy lobule) and deformity of superior auricle with unclassified variety of lateral ear pit. This article highlights that there are further cases of ear deformities that are noticed in the general population who come for cosmetic correction, and hence, there is a need for further modifying the classification of ear deformities.
American Journal of Roentgenology, 1985
The external auditory canal, middle ear, and bulk of the ossicular chain develop from the first branchial groove, first and second branchial arches, and first pharyngeal pouch. Embryologic development of these structures is complex and only rarely are two anomalies identical. Development of the inner ear structures occurs independently of external ear structures, and concomitant involvement is unusual. This study includes 11 cases of unilateral external auditory canal atresia and two cases of bilateral atresia. Eight cases (four bilateral) of isolated congenital ossicular anomalies are also included. Emphasis is placed on findings of surgical import. All patients were studied with computed tomography only, because it was believed that the bony and soft-tissue detail achieved is superior to that with conventional multidirectional tomography.
Classification and diagnosis of ear malformations
GMS current topics in otorhinolaryngology, head and neck surgery, 2007
In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90%) and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations) or acquired in nature. Malformations can affect the outer ear (pinna and external auditory canal, EAC), middle ear and inner ear, not infrequently in combination. Formal classification is advisable in order to be able to predict the prognosis and compare treatment schedules. Various classifications have been proposed: pinna and EAC malformations according to Weerda [1], middle ear malformations according to Kösling [2], and inner ear malformations according to Jackler [3], [4], to Marangos [5] and to Sennaroglu [6]. Additionally, we describe Altmann's classification of ...
From labyrinthine aplasia to otocyst deformity
Neuroradiology, 2010
Introduction Inner ear malformations (IEMs) are rare and it is unusual to encounter the rarest of them, namely labyrinthine aplasia (LA) and otocyst deformity. They do, however, provide useful pointers as to the early embryonic development of the ear. LA is characterised as a complete absence of inner ear structures. While some common findings do emerge, a clear definition of the otocyst deformity does not exist. It is often confused with the common cavity first described by Edward Cock. Our purpose was to radiologically characterise LA and otocyst deformity. Methods Retrospective analysis of CT and MRI data from four patients with LA or otocyst deformity. Middle and inner ear findings were categorised by two neuroradiologists. Results The bony carotid canal was found to be absent in all patients. Posterior located cystic structures were found in association with LA and otocyst deformity. In the most severe cases, only soft tissue was present at the medial border of the middle ear cavity. The individuals with otocyst deformity also had hypoplasia of the petrous apex bone. Conclusions These cases demonstrate gradual changes in the two most severe IEMs. Clarification of terms was necessary and, based on these findings, we propose defining otocyst deformity as a cystic structure in place of the inner ear, with the cochlea, IAC and carotid canal absent. This condition needs to be differentiated from the common cavity described by Edward Cook. A clear definition of inner ear malformations is essential if outcomes following cochlear implantation are to be compared.