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1.
Chinese Journal of Otorhinolaryngology Head and Neck Surgery ; (12): 476-480, 2023.
Article in Chinese | WPRIM | ID: wpr-986915

ABSTRACT

Objective: To observe the clinical effect of auricle reconstruction in adult patients with microtia and summarize the experience. Methods: Clinical data of adult patients with microtia who underwent total auricle reconstruction using the modified Nagata's two stage for microtia reconstruction from June 2016 to June 2021 were analyzed. A total of 41 adult patients (42 ears) with microtia were enrolled, including 30 males and 11 females, with the median age at the time of surgery of 37 years. Autogenous costal cartilage was used as the auricular framework for all patients in this group. The first stage surgery was performed according to the modified Nagata's two stage for microtia reconstruction procedure,cartilage auricular framework carving was performed by different methods according to the ossification state of adult costal cartilage. Six months following the primary operation, ear elevation and cranioauricular angle formation, retroauricular facial flap transfer and medium-thick skin grafting were performed in the second stage. Results: All patients successfully completed two stage operation. During the follow-up of 3 months and 24 months, all the 41 patients were satisfied with the morphology of reconstructed auricle. Conclusion: According to the costal cartilage status of adult patients, different costal cartilage carving techniques can be used for total auricle reconstruction to obtain ideal surgical results.


Subject(s)
Male , Female , Humans , Adult , Surgical Flaps , Congenital Microtia/surgery , Plastic Surgery Procedures , Ear, External/surgery , Ear Auricle/surgery
2.
Chinese Journal of Medical Genetics ; (6): 1292-1295, 2023.
Article in Chinese | WPRIM | ID: wpr-1009292

ABSTRACT

OBJECTIVE@#To analyze the genetic characteristics of a child with Meier-Gorlin syndrome (MGS) due to a homozygous variant of the ORC6 gene.@*METHODS@#A child who was admitted to the Children's Hospital Affiliated to Soochow University on March 25, 2019 due to growth retardation was selected as the study subject. Clinical data of the child was collected. Whole exome sequencing was carried out for the child. Candidate variant was validated by Sanger sequencing and bioinformatic analysis.@*RESULTS@#The child, a 8-year-and-3-month-old male, has featured short stature, small ears, bilateral cryptorchidism and patellar dysplasia. His parents were of first cousins. The child was found to harbor a homozygous c.712A>T (p.K238*) missense variant of the ORC6 gene, which may lead to premature termination of protein translation. Sanger sequencing confirmed that both of his parents were heterozygous carriers. Based on the guidelines from the American College of Medical Genetics and Genomics, the variant was classified as pathogenic (PVS1_Moderate+PM2_Supporting+PM3+PP3+PP4).@*CONCLUSION@#The homozygous c.712A>T (p.K238*) variant probably underlay the MGS in this child.


Subject(s)
Humans , Infant , Male , Computational Biology , Congenital Microtia/genetics , Dwarfism/genetics , Growth Disorders/genetics , Origin Recognition Complex/genetics
3.
Chinese Journal of Contemporary Pediatrics ; (12): 614-619, 2022.
Article in Chinese | WPRIM | ID: wpr-939637

ABSTRACT

Microtia is the second most common maxillofacial birth defect in neonates and has an prevalence rate of 3.06/10 000 in China, and 20%-60% of microtia cases is associated with a certain type of syndrome. This article elaborates on the clinical phenotypes and genetic characteristics of three microtia-associated syndromes (MASs) with high prevalence, high incidence rate of ear deformity, and definite genetic etiology, i.e., oculo-auriculo-vertebral spectrum, branchio-oto-renal spectrum disorder, and Treacher-Collins syndrome, and summarizes another three common MASs, so as to provide a reference for the genetic diagnosis of neonatal MAS.


Subject(s)
Humans , Infant, Newborn , China , Congenital Microtia/genetics , Phenotype , Prevalence , Syndrome
4.
Rev. Assoc. Méd. Rio Gd. do Sul ; 65(4): 01022105, OUT-DEZ 2021.
Article in Portuguese | LILACS | ID: biblio-1391963

ABSTRACT

A macrostomia congênita, também denominada fissura facial transversa, horizontal ou lateral, constitui 1,5% de todas as fissuras. A expressão clínica variável envolve desde uma forma discreta até casos graves com malformações desfigurantes. Deformidades da orelha externa podem estar presentes, desde excesso de pele até ausência do pavilhão. A macrostomia congênita deve ser submetida a uma investigação sistemática clínica e de imagem para definir o âmbito das anomalias, uma vez que a doença é rara, complexa e manifesta-se fenotipicamente de maneiras distintas. Este presente artigo relata um caso de macrostomia congênita associada à microtia unilateral.


Congenital macrostomia, also called transverse, horizontal, or lateral facial cleft, constitutes 1.5% of all clefts. The variable clinical expression ranges from mild to severe cases with disfiguring malformations. External ear deformities can be present, from excess skin to the absence of the pinna. Congenital macrostomia must be submitted to a systematic clinical and imaging investigation to define the scope of the anomalies, since the disease is rare, complex and manifests itself phenotypically in different ways. This article reports a case of congenital macrostomia associated with unilateral microtia.


Subject(s)
Humans , Adult , Congenital Microtia , Craving , Macrostomia
5.
Chinese Journal of Otorhinolaryngology Head and Neck Surgery ; (12): 643-647, 2021.
Article in Chinese | WPRIM | ID: wpr-942492

ABSTRACT

Objective: To observe the treatment effect of hemifacial dysplasia by injecting transplantation of autologous dermis and fat granules in the second stage surgery for total auricle reconstruction. Methods: From March 2013 to March 2018, 57 patients with unilateral microtia and mild-to-moderate hemifacial dysplasia were divided into concurrent treatment group (32 cases, including 13 females and 19 males and aged 6-33 years old with an average age of 12.5 years) and traditional treatment group (25 cases, including 10 females and 15 males and aged 6-21 years old with an average age of 11.3 years) according to the different surgical methods. Modified Nagata method of auricular reconstruction was chosen, in the second stage surgery (cranial ear angle plasty), patients in concurrent treatment group received the treatment of hemifacial dysplasia with autologous dermal and fat injection transplantation at the same time; Patients in traditional treatment group only received cranial ear angle plasty. Statistical analysis of the two groups of patients was carried out for the average operation time, the average length of hospital stay, the incidence of common complications and postoperative satisfaction rate. SPSS 21.0 software was used for statistical analysis. Results: The mean operation time of the concurrent treatment group (282.0±3.4)min was longer than that of the traditional treatment group (243.0±3.1)min, and the difference was statistically significant (t=9.884, P<0.05). There were no statistically significant differences in the average length of stay between the the concurrent treatment group (9.4±0.3)d and the traditional treatment group(9.5±0.2)d, t=0.256, P>0.05. There were no statistically significant differences in the incidence of common surgical complications between the concurrent treatment group (12.5%, 4/32) and the traditional treatment group(12.0%, 3/25), χ2=0, P>0.05. Postoperative satisfaction rate of the concurrent treatment group(90.6%, 29/32) was significantly higher than that of the traditional treatment group(56.0%, 14/25), the difference was statistically significant (χ2=9.081, P<0.05). Conclusions: Auricular reconstruction with treatment of hemifacial dysplasia should not significantly increase the average length of stay and the incidence of common complications compared with auricular reconstruction alone. Although the operation time is slightly extended, the scheme of concurrent treatment can reduce the times of operations, save medical resources and increase the satisfaction rate of patients.


Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Young Adult , Congenital Microtia/surgery , Ear Auricle/surgery , Ear, External/surgery , Operative Time , Plastic Surgery Procedures , Treatment Outcome
6.
Chinese Journal of Otorhinolaryngology Head and Neck Surgery ; (12): 346-350, 2021.
Article in Chinese | WPRIM | ID: wpr-942439

ABSTRACT

Objectives: To evaluate the therapeutic effects of unilateral/bilateral bone conduction hearing rehabilitation in patients with bilateral microtia accompanied with severe conductive hearing loss following staged auricle reconstruction and bonebridge implantation. Methods: Thirty-two patients, including 20 males and 12 females, with an average age of 11.8, who received surgical treatment in Peking Union Medical College Hospital (PUMCH) from March, 2016 to January, 2020 with bilateral microtia-atresia were included. Hearing thresholds, speech perception and high-resolution CT of the temporal bone were evaluated prior to surgery and individualized surgery plans (staged auricle reconstruction and bonebridge implantation) were made. Hearing thresholds and speech perception in quiet and noise (SNR = 5 dB) using unilateral Bonebridge were tested two weeks after the implantation surgery when the Bonebridge was activated and at 3th, 6th, 12th month after activation. Hearing thresholds and speech perception were also tested at least three months after the activation of the Bonebridge under three conditions: unaided, unilateral Bonebridge, and bilateral bone conduction hearing devices (Bonebridge plus contralateral ADHEAR). The international hearing aid assessment questionnaire (IOI-HA) and Glasgow children's benefit questionnaire were used to evaluate the subjective benefits of the patients. SPSS 21.0 software was used for statistical analysis. Results: Among these 32 patients, nine were conducted Bonebridge implantation surgery before auricle reconstruction, six were simultaneously with auricle reconstruction and 17 were implanted after auricle reconstruction surgery. Compared with unaided, the mean hearing thresholds (0.5, 1, 2, and 4 kHz) and speech perception following unilateral BCHD and bilateral BCHD attachment were improved significantly (P<0.05 each). The speech perceptin in noise of bilateral BCHD was better than unilateral (P<0.05 each). The modified questionnaire revealed high levels of patient satisfaction following use of both unilateral and bilateral devices. Conclusions: Individulized surgical procedures involving auricle reconstruction and Bonebridge implantation are safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems. Speech perception in noise is better following bilateral BCHD than unilateral BCHD attachment.


Subject(s)
Child , Female , Humans , Male , Bone Conduction , Congenital Microtia/surgery , Ear, External , Hearing Aids , Hearing Loss, Conductive/surgery , Speech Perception
7.
Chinese Journal of Otorhinolaryngology Head and Neck Surgery ; (12): 333-339, 2021.
Article in Chinese | WPRIM | ID: wpr-942437

ABSTRACT

Objective: To investigate the clinical effects of single-stage auricular reconstruction and hearing rehabilitation in children with microtia and external auditory canal atresia. Methods: Sixty eight cases of microtia with external auditory canal atresia (53 males and 15 females, age from 7 to 12 years, with a median age of 8.8 years), who received operations in Shanghai Ninth People's Hospital, Shanghai Jiaotong University School of Medicine from July 2017 to December 2019 were collected.A total of 28 cases received auricle reconstruction with high-density polyethylene (Medpor) framework and hearing reconstructions, among which 20 patients received the traditional external auditory canal and middle ear repair (EACR), and eight patients were implanted bone conduction device bone bridge(BB) simultaneously.In the control group, 40 patients only received Medpor frame implantation for auricle plasty. Postoperative changes in auricle morphology and auditory function and postoperative complications were evaluated. Results: After three to thirty months follow-ups, the auricles shape recovered well in all three groups. The average scores of 14 fine structures in the auricles were 9.43(EACR) and 10.67(BB) points. The average score of auricle symmetry were 6.83(EACR) and 6.00(BB) points. There was no significant difference compared to the auricle reconstruction group (8.23/6.20 points). P>0.05. After surgery, the average hearing improvement in the BB group was 43.33 dB HL and the average speech recognition threshold declined 42.28 dB HL. In the EACR group, the average hearing improvement was 4.13 dB HL and the average speech recognition threshold declined 11.36 dB HL. No vertigo, tinnitus, cerebrospinal fluid leakage and other complications occurred in all the patients. In the EACR group, sensorial hearing loss, auricle stent fracture, ear canal restenosis and ear canal atresia occurred in one patient respectively. In the auricle group, one auricle stent exposure and one facial branch nerve injury occurred. Nearly ten patients had difficulty in hair growth at scalp incisions. Conclusions: The operation of single-stage auricular reconstruction and hearing rehabilitation for microtia is feasible. The methods of hearing reconstruction should be determined by evaluating the development of the inner and middle ear of the patients. For those with poor mastoid development, bone bridge implantation is recommended to achieve a stable and significant hearing effect.


Subject(s)
Child , Female , Humans , Male , China , Congenital Microtia/surgery , Hearing , Hearing Loss/rehabilitation , Polyethylenes , Plastic Surgery Procedures
8.
Clinical and Experimental Otorhinolaryngology ; : 176-180, 2019.
Article in English | WPRIM | ID: wpr-763302

ABSTRACT

OBJECTIVES: The aim of this study was to analyze the incidence of inner ear anomalies in patients with unilateral congenital aural atresia (CAA) combined with microtia. METHODS: We retrospectively reviewed 61 patients with unilateral CAA combined with microtia who underwent high-resolution temporal bone computed tomography (TBCT) and hearing examination. Inner ear anomalies were analyzed using TBCT and evaluated according to the Jahrsdoerfer grading system, Marx classification, and extent of inferior displacement of the mastoid tegmen. RESULTS: Inner ear anomalies were observed in 14 patients (23.0%). Lateral semicircular canal (LSCC) dysplasia was the most common inner ear anomaly, with an incidence of 16.4%. The incidence was significantly higher on the pathologic side than on the unaffected side (P=0.002). All vascular anomalies were observed in the high-riding jugular bulb, with an incidence of 24.6%. The incidence was significantly higher on the pathologic side than on the unaffected side (P<0.001). LSCC dysplasia was significantly more common in patients with a lower Jahrsdoerfer score (odds ratio, 0.66; P=0.004). CONCLUSION: The incidence of inner ear anomalies was relatively high in patients with unilateral CAA combined with microtia; LSCC dysplasia was the most common anomaly and the probability of coexistence was higher in patients with a lower Jahrsdoerfer score.


Subject(s)
Humans , Classification , Congenital Microtia , Ear, Inner , Hearing , Incidence , Mastoid , Retrospective Studies , Semicircular Canals , Temporal Bone
9.
Archives of Craniofacial Surgery ; : 79-82, 2018.
Article in English | WPRIM | ID: wpr-713275

ABSTRACT

Formation of an ideally-shaped tragus remains one of the most challenging issues during staged tragus reconstruction in microtia patients. The authors describe a new method used to treat a unique case of concha-type microtia in which the 10-year-old male patient had only a portion of pre-existing cartilage at the tragus site. An anomalous skin lump was also present. During the initial stages of the reconstruction, the two-stage Nagata method was used for surgical correction of the microtia. An autologous rib cartilage graft was used to form the ear framework. A temporoparietal fascia flap was also constructed. Remnant skin tags and anomalous cartilage that accompany microtia are usually removed during microtia repair. However, the cartilage and skin lump were preserved during the reconstruction. The skin lump was later used to form a vascularized chondrocutaneous island flap that supplemented the portion of cartilage during tragus formation. The result was a new tragus that was satisfactorily improved in both size and shape. Patients with concha-type microtia may benefit from the use of this new method for tragus formation.


Subject(s)
Child , Humans , Male , Cartilage , Congenital Microtia , Ear , Ear Auricle , Fascia , Methods , Ribs , Skin , Surgical Flaps , Transplants
10.
The Medical Journal of Malaysia ; : 135-137, 2017.
Article in English | WPRIM | ID: wpr-630945

ABSTRACT

A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication. KEY WORDS: Microtia; canal atresia; cochleovestibular nerve aplasia; inner ear anomaly


Subject(s)
Congenital Microtia
11.
Chinese Journal of Medical Genetics ; (6): 68-72, 2017.
Article in Chinese | WPRIM | ID: wpr-345322

ABSTRACT

<p><b>OBJECTIVE</b>To identify the genetic cause for a 11-year-old Chinese boy with Meier-Gorlin syndrome (MGS).</p><p><b>METHODS</b>Chromosomal microarray analysis (CMA) was used to detect potential variations, while whole exome sequencing (WES) was used to identify sequence variants. Sanger sequencing was used to confirm the suspected variants.</p><p><b>RESULTS</b>The boy has featured short stature, microtia, small patella, slender body build, craniofacial anomalies, and small testes with normal gonadotropin. A complete uniparental disomy of chromosome 16 was revealed by CMA. WES has identified a novel homozygous mutation c.67A>G (p.Lys23Glu) in ORC6 gene mapped to chromosome 16. As predicted by Alamut functional software, the mutation may affect the function of structural domain of the ORC6 protein.</p><p><b>CONCLUSION</b>The patient is probably the first diagnosed MGS case in China, who carried a novel homozygous mutation of the ORC6 gene and uniparental disomy of chromosome 16. The effect of this novel mutation on the growth and development needs to be further investigated.</p>


Subject(s)
Child , Humans , Male , Base Sequence , Chromosomes, Human, Pair 16 , Genetics , Congenital Microtia , Genetics , Family Health , Fathers , Growth Disorders , Genetics , Heterozygote , Micrognathism , Genetics , Mutation , Origin Recognition Complex , Genetics , Patella , Congenital Abnormalities , Polymerase Chain Reaction , Methods , Sequence Analysis, DNA , Methods , Uniparental Disomy , Genetics
12.
Archives of Craniofacial Surgery ; : 287-291, 2017.
Article in English | WPRIM | ID: wpr-134109

ABSTRACT

Preauricular sinus is a congenital malformation that is very commonly encountered among the general population and it has especially high prevalence among Asians when compared to other ethnic groups. It can often go unnoticed or easily overlooked by the patient or even by doctors because most of them are asymptomatic and is most of the time only a tiny pit that can be trivial in terms of aesthetics. We report a very rare and unique case that has no precedence what so ever; hence no reported case in the literature: an ectopic preauricular sinus in a facial cleft and microsomia patient.


Subject(s)
Humans , Asian People , Branchial Region , Congenital Microtia , Craniofacial Abnormalities , Esthetics , Ethnicity , Prevalence
13.
Archives of Craniofacial Surgery ; : 287-291, 2017.
Article in English | WPRIM | ID: wpr-134108

ABSTRACT

Preauricular sinus is a congenital malformation that is very commonly encountered among the general population and it has especially high prevalence among Asians when compared to other ethnic groups. It can often go unnoticed or easily overlooked by the patient or even by doctors because most of them are asymptomatic and is most of the time only a tiny pit that can be trivial in terms of aesthetics. We report a very rare and unique case that has no precedence what so ever; hence no reported case in the literature: an ectopic preauricular sinus in a facial cleft and microsomia patient.


Subject(s)
Humans , Asian People , Branchial Region , Congenital Microtia , Craniofacial Abnormalities , Esthetics , Ethnicity , Prevalence
14.
Arq. bras. med. vet. zootec ; 68(3): 683-686, ilus
Article in Portuguese | LILACS, VETINDEX | ID: lil-785711

ABSTRACT

Descreve-se o caso de um cão, Pinscher, fêmea de 11 meses de idade, com alteração anatômica da orelha do lado direito. Após avaliação, foi realizado o diagnóstico clínico e o radiográfico de microtia com atresia do canal auditivo do lado direito. Como o animal não apresentava sinais de alterações no sistema vestibular ou otite, optou-se pelo acompanhamento clínico do caso. A microtia, caracterizada pela hipoplasia parcial ou completa da pina, assim como outras anomalias do conduto auditivo, é raramente descrita em medicina veterinária. De acordo com a revisão de literatura realizada, este é o primeiro relato ocorrido no Brasil de um cão com microtia e atresia congênita do canal auditivo sem a associação de síndrome vestibular.(AU)


This paper describes an 11-month-old female Pinscher dog, with anatomical changes of the ear on the right side. After physical exam, clinical and radiographic diagnosis of microtia with ear canal atresia on the right side was made. As the animal did not show any signs of vestibular system changes or ear infections, the veterinarians opted for monitoring the patient. Microtia, characterized by the partial or complete hypoplasia of pina, as well as other abnormalities of ear canal are rarely described in veterinary medicine. As far as the author´s knowledge by literature review carried out, this is the first report of a dog with microtia and congenital ear canal atresia not associated with vestibular syndrome in Brazil.(AU)


Subject(s)
Animals , Dogs , Congenital Abnormalities/veterinary , Congenital Microtia/veterinary , Ear Canal/abnormalities , Vestibule, Labyrinth , Anatomic Variation , Ear/abnormalities , Ear/anatomy & histology , Otitis/diagnosis , Otitis/veterinary
15.
Rev. chil. cir ; 68(2): 131-136, abr. 2016. ilus, graf
Article in Spanish | LILACS | ID: lil-784842

ABSTRACT

Aim: To assess the presence of donor site sequel of patients undergoing ear reconstruction with costal cartilage harvest, using supraperichondrial technique. methods: Cross-sectional study; patients under 15 years. The cartilages were harvested with perichondrium. Three observers performed donor site assessment; they were independent to this study (validated Strasser score: deformity, asymmetry, contour and scar). Children and their mothers were applied satisfaction survey. Analysis of the association between variables: nonparametric tests. Results: 19 reconstructions, 18 patients, age 8 (6-15) years, follow-up 50 (14-96) months; number of rib cartilage harvested 3 (2-4) units. Expert assessment: excellent in 2 (10.5%) cases, good in 10 (52.6%), regular in 7 (36.8%). Most mothers and patients rated the result as good. There was no significant difference in the score, with respect to the number of harvested costal cartilages. To separate them by age, poorer results were obtained at surgery before 10 years of age than in older (p < 0.03). Conclusion: Ear reconstruction and supraperichondrial technique for costal cartilage allows obtain good results in donor site. There is high satisfaction when evaluated by the patient and his mother, as well as observers. The result is not dependent on the number of harvested cartilage, but is related to age at surgery, best results were obtained in patients operated over 10 years of age.


Objetivo: Evaluar presencia de secuela en zona dadora de pacientes sometidos a reconstrucción auricular con cartílago costal con técnica suprapericóndrica. material y método: Corte transversal; pacientes menores de 15 años. Cartílagos fueron levantados con pericondrio. Evaluación de zona dadora fue realizada por tres observadores independientes (escala validada de Strasser: deformidad, asimetría, contorno y cicatriz). A niños y madres se les aplicó encuesta de satisfacción. En el análisis de la asociación entre variables se utilizan pruebas no paramétricas. Resultados: 19 reconstrucciones, 18 pacientes, mediana edad 8 (6-15) años, seguimiento 50 (14-96) meses, número de cartílagos costales obtenidos 3 (2-4) unidades. Evaluación de expertos: excelente en 2 (10,5%) casos, bueno en 10 (52,6%) y regular en 7 (36,8%). La mayoría de las madres y pacientes calificó como bueno el resultado. No hubo diferencia significativa en el puntaje con respecto al número de cartílagos costales cosechados. Al separarlos por edad se obtuvo peores resultados en los intervenidos antes de los 10 años que en mayores (p < 0,03). Conclusión: La reconstrucción auricular con cartílago costal y técnica suprapericóndrica, permite obtener buenos resultados en zona dadora. Existe alta satisfacción al ser evaluada por el paciente y su madre, así como por los observadores. El resultado no es dependiente del número de cartílagos; sí en relación a la edad de la cirugía: mejores resultados en pacientes operados sobre los 10 años de edad, lo que concuerda con otros estudios.


Subject(s)
Humans , Child , Adolescent , Tissue Transplantation/methods , Plastic Surgery Procedures/adverse effects , Costal Cartilage/transplantation , Congenital Microtia/surgery , Cross-Sectional Studies , Surveys and Questionnaires , Follow-Up Studies , Patient Satisfaction , Transplant Donor Site
16.
Korean Journal of Pediatrics ; : S10-S13, 2016.
Article in English | WPRIM | ID: wpr-228473

ABSTRACT

Chromosome 11q13 deletion syndrome has been previously reported as either otodental syndrome or oculo-oto-dental syndrome. The otodental syndrome is characterized by dental abnormalities and high-frequency sensorineural hearing loss, and by ocular coloboma in some cases. The underlying genetic defect causing otodental syndrome is a hemizygous microdeletion involving the FGF3 gene on chromosome 11q13.3. Recently, a new form of severe deafness, microtia (small ear) and small teeth, without the appearance of eye abnormalities, was also reported. In this report, we describe a 1-year-old girl presenting with ptosis of the left upper eyelid, right auricular deformity, high-arched palate, delayed dentition, simian line on the right hand, microcephaly, and developmental delay. In this patient, we identified a deletion in the chromosome 11q13.2-q13.3 (2.75 Mb) region by using an array-comparative genomic hybridization analysis. The deletion in chromosome 11q13 results in a syndrome characterized by variable clinical manifestations. Some of these manifestations involve craniofacial dysmorphology and require a functional workup for hearing, ophthalmic examinations, and long-term dental care.


Subject(s)
Female , Humans , Coloboma , Congenital Abnormalities , Congenital Microtia , Deafness , Dental Care , Dentition , Eye Abnormalities , Eyelids , Hand , Hearing , Hearing Loss , Hearing Loss, Sensorineural , Microcephaly , Nucleic Acid Hybridization , Palate , Tooth , Tooth Abnormalities
17.
Korean Journal of Otolaryngology - Head and Neck Surgery ; : 419-423, 2016.
Article in Korean | WPRIM | ID: wpr-645693

ABSTRACT

The complexity of the auricle makes its reconstruction technically difficult, owing to the convexities and concavities of the underlying cartilage with its tightly adherent and thin overlying skin. This article gives an overview of the current practice in the field of auricular reconstruction. The majority of surgeons who perform auricular reconstruction continues to employ the well-established techniques developed by Brent and Nagata. Surgery takes between two and four stages, with the initial stage being construction of a framework of autogenous rib cartilage which is implanted into a subcutaneous pocket. Several modifications of these techniques have been reported. Understanding the various treatment options and having sufficient practice to increase the surgeon's skills are crucial to providing the patients with the best outcomes possible.


Subject(s)
Humans , Cartilage , Congenital Microtia , Costal Cartilage , Ribs , Skin , Surgeons
18.
Anatomy & Cell Biology ; : 273-280, 2016.
Article in English | WPRIM | ID: wpr-225093

ABSTRACT

Superficial temporal artery (STA) based pedicled fascial flap plays a pivotal role in ear reconstruction for microtia patients. There is paucity of literature on the anatomy of the STA in microtia patients. The present study aimed to describe any possible anatomical variations seen in the STA of patients afflicted with microtia. Pre-operative carotid computer tomographic angiography images of patients under the microtia database of Plastic and Reconstructive Surgery Unit at a tertiary medical centre were selected and 3-dimensionally reconstructed. Measurements were made on the 3D reconstructed computed tomographic angiography images of the STA on both the sides of the microtic ear and the non-microtic ear to assess its various anatomical parameters. We managed to obtain a total of 39 computed tomographic angiography images of STAs for analysis. There was a significant difference in the number of main branches of STA between the two groups (P=0.006). The proportion of ears with 2 main branches was higher in the non-microtia group (89.5%) compared to the microtia group (45.0%). A significant difference was found in the STA diameter between the two groups (P=0.012). The mean diameter of STA in the non-microtia group was larger by 0.4 mm. Furthermore, the median angle of STA was larger on the side of the non-microtic ears compared to that of microtic ears by 24.5°, with a P-value of 0.011. The results of the study may be of clinical importance while planning and performing ear reconstructive surgeries using STA based pedicled fascial flaps.


Subject(s)
Humans , Angiography , Congenital Microtia , Ear , Plastics , Temporal Arteries
19.
Rev. pediatr. electrón ; 12(3): 13-23, oct. 2015. ilus, tab
Article in Spanish | LILACS | ID: lil-776772

ABSTRACT

El pabellón auricular es una importante unidad estética en el ser humano. Existe un grado variable de malformaciones, en Chile tiene una incidencia de 1 por cada 5600 nacidos vivos. Anatómicamente el pabellón auricular está compuesto por tres estructuras principales: complejo hélix antihélix, concha y lóbulo. Es según la anomalía que se presente en la anatomía externa la forma en que se clasificará, existiendo diversos sistemas para esto, algunos de ellos con utilidad anátomo quirúrgica.El eje principal del tratamiento de estas anomalías es la reconstrucción quirúrgica, teniendo disponible en la literatura variadas técnicas, como son el uso de implantes, reconstrucción protésica y la reconstrucción con injerto autólogo de cartílago costal, entre otras. Reinisch ha sido pionero en el uso de implantes para la reconstrucción, mientras que diversos autores han mostrado resultados satisfactorios con el uso de prótesis osteointegradas. Sin embargo, la técnica más aceptada y utilizada es la reconstrucción con injerto autólogo de cartílago costal, la cual fue descrita en sus inicios por Tanzer, Walton y Beahm, siendo perfeccionada posteriormente por Brent, quien propuso las bases para su desarrollo moderno. En la actualidad los métodos quirúrgicos más aceptados y utilizados son los descritos por Nagata y Firmin, ambos con excelentes resultados.


The ear is an important aesthetic unity in humans. There is a varying degree of malformations, Chile has an incidence of 1 per 5600 live births. Anatomically the pinna is composed of three main structures: helix antihelix complex, shell and lobe. It is according to the anomaly present in the external anatomy how it is classified, various systems exist for this, some of them with anatomic surgical utility. The main focus of treatment of these anomalies is the surgical reconstruction, taking various techniques available in the literature, such as the use of implants, prosthetic reconstruction and reconstruction with autologous rib cartilage graft, among others. Reinisch has pioneered the use of implants for reconstruction, while several authors have shown satisfactory results with the use of bone anchored prosthesis. However, the most accepted and used technique is the reconstruction with autologous rib cartilage graft, which was described at the beginning by Tanzer, Walton and Beahm and was later perfected by Brent, who proposed the foundation for modern development. Currently the most accepted and used surgical methods are described by Nagata and Firmin, both with excellent results.


Subject(s)
Humans , Congenital Microtia/classification , Congenital Microtia/therapy , Congenital Microtia/pathology
20.
Article in English | IMSEAR | ID: sea-158303

ABSTRACT

Microtia is a congenital anomaly of the ear can occur as an isolated birth defect or as part of a spectrum of anomalies or as a syndrome. Microtia is often associated with impaired hearing and or total loss of hearing. Such patients typically require treatment for surgical ear reconstruction and for hearing impairment. Maintenance of ear projection and post auricular sulcus in staged ear reconstruction in microtia is a trying problem. So also is the maintenance of the patency of the external auditory meatus following recanalization and meatoplasty.This case report describes a simple effective way of fabrication of ear splint prosthesis.


Subject(s)
Acrylic Resins , Congenital Microtia/surgery , Ear Deformities, Acquired/surgery , Ear, External/surgery , Humans , Otologic Surgical Procedures/instrumentation , Prostheses and Implants , Splints
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