Microtia é uma deformidade congênita, na qual o pavilhão auricular é subdesenvolvido. Uma orelha completamente subdesenvolvida é referida como anotia. Devido ao fato de microtia e anotia terem a mesma origem, a condição pode ser chamada genericamente de microtia-anotia. A microtia pode ser unilateral ou bilateral. A microtia ocorre em cerca de 1 a cada 8.000 a 10.
Introdução
O que você precisa saber de cara
Anotia é a ausência congênita total de uma ou ambas as orelhas externas. Geralmente associada a outras anomalias craniofaciais, pode afetar a audição e a estética.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Anotia
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4 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
Total Ear Reconstruction with Costal Cartilage in Challenging Cases: Silicone-Induced Vascularized Capsule Technique.
Total ear reconstruction using an autogenous costal cartilage framework within a mastoid skin pocket becomes challenging when suboptimal mastoid skin prevents complete framework embedding. Twelve patients with suboptimal mastoid skin underwent a silicone-induced vascularized capsule technique. Eight patients had congenital dystopic lobule-remnant microtia or anotia, accompanied by a severely low hairline, hemifacial microsomia, or both. Three patients had lobule-remnant congenital microtia requiring revision, and 1 patient had posttraumatic microtia. In 7 cases, hair-bearing mastoid skin was treated preoperatively with laser depilation. During the first stage, silicone blocks were placed in the recessed area of the cartilage framework-either the concha (11 patients) or scapha (1 patient)-to promote formation of a vascularized capsule between the silicone and bare cartilage, enhancing skin-to-framework contact. The second stage involved framework elevation to achieve ear projection. In the third stage, silicone blocks were removed, and skin grafts were applied. Two patients were excluded because of incomplete final-stage surgery. Nine patients developed well-vascularized capsules, enabling full-thickness skin grafting, without tissue loss. One had partial capsule loss from silicone exposure through a scar, but the remaining capsule supported successful grafting. During the 10-month to 4-year follow-up, grafted skin remained stable, with well-preserved contours. The capsule provided additional coverage of 227 mm 2 to 841 mm 2 (mean, 553 mm 2 ). All patients reported high satisfaction. This technique offers a reliable option for total ear reconstruction in patients with suboptimal mastoid skin, allowing tension-free coverage and stable long-term outcomes.
A Modified Method for Ear Projection in Auricular Reconstruction: Split-Thickness Skin Graft Combined with Retroauricular Fascia Flap.
The 2-stage Nagata technique is one of the most prevalent methods for auricular reconstruction. Nevertheless, in the second stage, the use of a temporoparietal fascial flap usually requires an additional incision, and the procurement of donor skin from the groin or chest area can result in extra scarring, which may reduce patients' satisfaction with the surgical outcomes. The authors propose a refined method using a retroauricular fascia flap combined with a split-thickness skin graft for postauricular coverage, which can avoid additional scarring in the temporoparietal and donor skin area. From April of 2019 to January of 2024, auricular reconstructions on 337 ears across 324 patients were performed using this novel technique. The classification of microtia, duration of surgery, and postoperative complications were recorded. The authors finally evaluated the scar condition and patient satisfaction with surgery outcomes in the 1- to 2-year postoperative period. In the authors' study, 154 patients were diagnosed with lobule-type microtia, 53 patients were diagnosed with small concha-type microtia, 62 patients were diagnosed with concha-type microtia, and 55 patients were diagnosed patients with anotia. The average duration of the second-stage surgery was 2.8 hours. Patients who underwent this modified technique exhibited no noticeable long-term postoperative scarring and no instances of flap necrosis, framework deformation, cartilage exposure, infection, or mismatched skin color following the procedure. Furthermore, 94% of the patients reported satisfaction with the surgical outcomes. The use of continuous split-thickness skin grafts, combined with a retroauricular fascial flap, offers a safe, effective, and aesthetically pleasing solution for second-stage auricular projection.
An Overview of the Currently Used Congenital Auricular Anomalies (CAA) Classifications for Surgical Reconstruction: A Scoping Review.
BackgroundCAAs are congenital malformations of the auricle ranging from ear underdevelopment to anotia, lacks standardized classification, impacting our outcome of different reconstruction approaches. This scoping review aimed to explore which CAA classifications are most used in current ear reconstruction practices.MethodsWe conducted a scoping review following the PRISMA guidelines, searching MEDLINE and Embase databases on November 1st, 2023. Studies on CAA reconstruction that included clear descriptions of the used classification published in the past 5 years were included. Studies were appraised using the Joanna Briggs Institute checklist.ResultsOut of 293 screened studies, 45 met inclusion criteria, encompassing 19 case series and 5 cohort studies. Findings revealed a predominant use of the Nagata classification across rib cartilage and alloplastic material reconstructions, despite noted application inconsistencies. Other systems like Marx's, Weerda's, and Meurman's remain underutilized.ConclusionMost studies used the Nagata's classification. Its widespread use underscores the necessity for an easy to use, but standardized classification to improve surgical outcome reporting and assessment accuracy. Further investigation and standardization efforts regarding the Nagata system are recommended.
A systematic review of congenital external ear anomalies and their associated factors.
External ear anomalies may lead to conductive hearing loss with significant childhood disability, psychological distress, anxiety, social avoidance, and behavioral problems. The aim of this study is to compile and review published literature on the frequency of isolated and non-isolated external ear anomalies, their associated factors, and associated malformations/deformations in non-isolated cases. We conducted a systematic review in PubMed, Google Scholar, and Science Direct searching for any type of article (excluding reviews and meta-analyses) reporting isolated and non-isolated external ear anomalies in humans. Two authors extracted the information according to the main variables of interest according to PICO criteria. Details of studied population and main findings were also obtained (malformation type, unilateral or bilateral malformations and associated factors). Twenty-six studies met eligibility criteria to be included in this review. Anotia/microtia was the most reported malformation, more frequently found in males, mostly unilateral; being the right ear the most affected, and more frequent in Hispanic population. Associated factors for external ear anomalies included parental age, maternal education, multiple pregnancies, high maternal body mass index and diabetes, pregnancy, and perinatal complications (low birth weight, prematurity, threatened abortion, etc.), twining, and chemical/drug exposure. The most reported malformations and syndromes associated with congenital external ear defects included: skull/face anomalies, cleft lip/palate, congenital heart defects, musculoskeletal malformations of skull, face and jaw, Treacher-Collins, OAVS (oculo-auriculo-vertebral spectrum), and trisomy 18, 13 and 21. Congenital external ear anomalies can occur isolated or associated with other malformations or syndromes. Environmental, socioeconomic, and cultural factors may partially explain the variation across populations for congenital external ear anomalies. Depending on their type and severity, they can lead to speech impediments and childhood disability, particularly in bilateral cases, highlighting the relevance of early detection and repair to avoid childhood disability.
Association between maternal occupation as a cleaner/maid/janitor during early pregnancy and selected birth defects in the National Birth Defects Prevention Study.
Cleaning work involves potential exposures to physical, chemical, biological and psychosocial hazards, which might increase the risk of birth defects. Yet, there is limited research focused on maternal occupation in cleaning jobs and the risk of birth defects. We investigated the association between maternal occupation in cleaning-related jobs during early pregnancy and selected birth defects in the National Birth Defects Prevention Study, a case-control study from 1997 to 2011. Mothers self-reported job details, which were classified by occupational epidemiologists into cleaner or non-cleaner occupations. Cases were live births or stillbirths with at least one of 19 eligible birth defects ascertained from state surveillance systems, while controls were randomly selected live births without structural defects. We estimated adjusted ORs with 95% CIs from multivariable logistic regression for associations between maternal cleaning occupations and birth defects, controlling for eight covariates identified a priori. Of the 28 936 mothers, 1109 (868 cases/241 controls) were assigned a primary occupational code as a cleaner during early pregnancy. In total, 9 of the 19 included birth defects had elevated ORs (>1.5), ranging from 1.58 for anophthalmia/microphthalmia to 2.65 for oesophageal atresia/stenosis; six had CIs that excluded the null (anencephaly, glaucoma, anotia/microtia, cleft lip and palate, oesophageal atresia/stenosis, small intestinal atresia/stenosis). We observed that maternal occupation in cleaning-related jobs was associated with several specific birth defects in various body systems. Future studies should explore specific chemical and physical exposures under these cleaning occupations and the association with birth defects.
Publicações recentes
Association between maternal occupation as a cleaner/maid/janitor during early pregnancy and selected birth defects in the National Birth Defects Prevention Study.
Non-recurrent duplications on chromosome 4p16.1 involving cis-regulatory elements affecting neural crest development in patients with isolated bilateral microtia.
A new species of Anotia Westwood from Bonaire and Curaao (Fulgoromorpha: Fulgoroidea: Derbidae: Otiocerini).
Total Ear Reconstruction with Costal Cartilage in Challenging Cases: Silicone-Induced Vascularized Capsule Technique.
A Modified Method for Ear Projection in Auricular Reconstruction: Split-Thickness Skin Graft Combined with Retroauricular Fascia Flap.
📚 EuropePMC55 artigos no totalmostrando 92
Association between maternal occupation as a cleaner/maid/janitor during early pregnancy and selected birth defects in the National Birth Defects Prevention Study.
Occupational and environmental medicineNon-recurrent duplications on chromosome 4p16.1 involving cis-regulatory elements affecting neural crest development in patients with isolated bilateral microtia.
Human geneticsA new species of Anotia Westwood from Bonaire and Curaao (Fulgoromorpha: Fulgoroidea: Derbidae: Otiocerini).
ZootaxaTotal Ear Reconstruction with Costal Cartilage in Challenging Cases: Silicone-Induced Vascularized Capsule Technique.
Plastic and reconstructive surgeryA Modified Method for Ear Projection in Auricular Reconstruction: Split-Thickness Skin Graft Combined with Retroauricular Fascia Flap.
Plastic and reconstructive surgeryThe Relationship Between Microtia and Complex Chronic Conditions: A Comprehensive Analysis of 20 000 Patients.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationRacial, Ethnic, and Socioeconomic Trends in Microtia and Anotia Care.
The Journal of craniofacial surgeryA systematic review of congenital external ear anomalies and their associated factors.
Frontiers in pediatricsMicrotia Reconstruction: 30-Day Outcomes for Autograft Versus Implant Reconstruction in a National Surgical Database.
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International archives of otorhinolaryngologyAn Overview of the Currently Used Congenital Auricular Anomalies (CAA) Classifications for Surgical Reconstruction: A Scoping Review.
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The Journal of pediatricsRetroposition of the Vestigial Cartilage in Patients With Microtia: A Novel Technique to Enhance Projection of the Reconstructed Ear.
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Birth defects research. Part A, Clinical and molecular teratologyAssociações
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Total Ear Reconstruction with Costal Cartilage in Challenging Cases: Silicone-Induced Vascularized Capsule Technique.
- A Modified Method for Ear Projection in Auricular Reconstruction: Split-Thickness Skin Graft Combined with Retroauricular Fascia Flap.
- An Overview of the Currently Used Congenital Auricular Anomalies (CAA) Classifications for Surgical Reconstruction: A Scoping Review.The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association· 2026· PMID 39846418mais citado
- A systematic review of congenital external ear anomalies and their associated factors.
- Association between maternal occupation as a cleaner/maid/janitor during early pregnancy and selected birth defects in the National Birth Defects Prevention Study.
- Non-recurrent duplications on chromosome 4p16.1 involving cis-regulatory elements affecting neural crest development in patients with isolated bilateral microtia.
- A new species of Anotia Westwood from Bonaire and Curaao (Fulgoromorpha: Fulgoroidea: Derbidae: Otiocerini).
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:93976(Orphanet)
- MONDO:0019780(MONDO)
- GARD:16835(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q15057288(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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