Topic
Microtia
About: Microtia is a research topic. Over the lifetime, 1132 publications have been published within this topic receiving 16152 citations. The topic is also known as: congenital Microtia.
Papers published on a yearly basis
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Papers
TL;DR: In this article, the authors present a sound approach to auricular construction using methods that have evolved through two decades of my personal experience with 600 cases and discuss pertinent information I have gleaned from a questionnaire sent to my operated patients.
Abstract: The purpose of this paper is twofold: to present a sound approach to auricular construction using methods that have evolved through two decades of my personal experience with 600 cases and to discuss pertinent information I have gleaned from a questionnaire sent to my operated patients. This series comprises 546 completed ears in 500 microtia patients (46 bilateral) and 75 completed ears in acquired deformities. Follow-up ranges from 1 to 17 years. Major complications such as infection, hematoma, or skin loss with cartilage exposure occurred in only 1.6 percent of cases and were limited to the perioperative period of 12 days. None have occurred in the last 9 years (481 frameworks). This paper describes the evolution and rationale for my current management of total ear repair and covers preoperative planning, how to fabricate the rib cartilage framework, how to modify the framework for specific deformities, and how to cover the framework, i.e., how to assess and use local skin and vestiges or when to supplement the coverage by recruiting fascial flaps or using tissue expansion. When considering fascial flaps, patient selection was found to be particularly important because of long-term effects on the donor scalp; expansion was found to be most useful intraoperatively. This paper also covers how to manage the hairline, how to stage the surgery, when to combine procedures, and how to manage bilateral microtia in a team approach. The survey revealed that autogenous cartilage frameworks grow, are durable, retain their detail over the years, and withstand trauma well. More than 40 severe traumas occurred in surgically constructed ears, and all healed without incident. Ears constructed in young patients generally grew to keep pace with the opposite normal side; 41.6 percent actually overgrew by several millimeters. Emotional and psychological benefits were universal, and patient satisfaction was high. Among patients who were classified as "severely affected" by the original deformity, 100 percent were pleased with the result. When considered "moderately disturbed" by the microtic defect and operated on by age 14, 95.5 percent of patients were satisfied with the surgical repair; 83.3 percent of adolescents between the ages of 15 and 20 who did not consider themselves "severely affected" by the deformity were pleased with the outcome, and the rest were "undecided."
400 citations
TL;DR: Current knowledge of the epidemiology and genetics of microtia is reviewed, including potential candidate genes supported by evidence from human syndromes and animal models, and the possible etiopathogenesis in light of the hypotheses formulated to date: Neural crest cells disturbance, vascular disruption, and altitude are discussed.
Abstract: Microtia is a congenital anomaly of the ear that ranges in severity from mild structural abnormalities to complete absence of the ear, and can occur as an isolated birth defect or as part of a spectrum of anomalies or a syndrome. Microtia is often associated with hearing loss and patients typically require treatment for hearing impairment and surgical ear reconstruction. The reported prevalence varies among regions, from 0.83 to 17.4 per 10,000 births and the prevalence is considered to be higher in Hispanics, Asians, Native Americans, and Andeans. The etiology of microtia and the cause of this wide variability in prevalence are poorly understood. Strong evidence supports the role of environmental and genetic causes for microtia. Although some studies have identified candidate genetic variants for microtia, no causal genetic mutation has been confirmed. The application of novel strategies in developmental biology and genetics has facilitated elucidation of mechanisms controlling craniofacial development. In this paper we review current knowledge of the epidemiology and genetics of microtia, including potential candidate genes supported by evidence from human syndromes and animal models. We also discuss the possible etiopathogenesis in light of the hypotheses formulated to date: neural crest cells disturbance, vascular disruption and altitude.
400 citations
TL;DR: The modification stages involved in total reconstruction of the auricle for small concha-type microtia are described and discussed in this paper.
Abstract: Among the microtic disorders, those with the remnant ear, ear lobule, concha, acoustic meatus, tragus, and incisura intertragica are classified as concha-type microtia. From these characteristic factors, concha-type microtia has been thought to be a milder form of microtic disorder in comparison with lobule-type microtia. The constructed modified three-dimensional costal cartilage framework is grafted to the superior portion of the auricle and fixed to the inferior remnant cartilage of the auricle (conchal portion). This may appear to be a simple procedure in comparison with the other microtic disorders, but it is extremely difficult to fix the modified three-dimensional framework to the remnant auricular cartilage so that a smooth configuration is attained at the seam. In this paper, the modification stages for total reconstruction of the auricle for concha-type microtia are discussed.
374 citations
TL;DR: The phenotypic characteristics of a single craniofacial clinic population of 294 individuals affected with oculoauriculovertebral dysplasia (OAV) and variants are described, which is the largest population so described in the literature.
Abstract: Here we describe the phenotypic characteristics of a single craniofacial clinic population of 294 individuals affected with oculoauriculovertebral dysplasia (OAV) and variants. To our knowledge, this is the largest population so described in the literature. The study population was divided into five subgroups based on the presence of combinations of minimal diagnostic criteria: microtia, mandibular hypoplasia, anomalies of the cervical spine and/or epibulbar or lipodermoids. The following data were recorded: sex (M:F 191:103); race (78% Caucasian); the presence of unilateral or bilateral microtia (193 unilateral, 98 bilateral); the presence of symmetric microtia in bilateral cases (34/98); the presence of mandibular hypoplasia ipsilateral or contralateral to the microtic ear or most severely microtic ear in bilateral cases (135/137 were ipsilateral in unilateral cases, 55/62 were ipsilateral in bilateral cases); the number of individuals with no other congenital anomaly in addition to the minimal diagnostic criteria (154/294), with only one other congenital anomaly (51/294), and with two or more other congenital anomalies (89/294); and the type of other congenital anomalies. Finally, we compared our results with other studies. Findings from our study include: mandibular asymmetry should be expected in patients with unilateral or bilateral microtia; bilateral involvement is frequent in patients with microtia; other malformations are seen frequently in all subgroups; anomalies of the cervical spine are more likely to be associated with other anomalies; and other malformations are seen in all systems and should be searched for to provide optimal management.
348 citations
TL;DR: The results represent a significant breakthrough in clinical translation of tissue engineered human ear-shaped cartilage given the established in vitro engineering technique and suitable surgical procedure.
275 citations
Related Topics (5)
Performance Metrics
| Year | Papers |
|---|---|
| 2025 | 47 |
| 2024 | 58 |
| 2023 | 93 |
| 2022 | 129 |
| 2021 | 83 |
| 2020 | 68 |