The role of MRI in the prenatal diagnosis and classification of fetal microtia.

OBJECTIVE: To investigate the role of MRI in the diagnosis and classification of fetal microtia. METHODS: Ninety-five fetuses with suspected microtia based on ultrasound and MRI performed within 1 week were enrolled in this study. The diagnosis based on MRI was compared with postnatal diagnosis. Among the microtia cases suspected on the basis of MRI, mild and severe cases were further classified. In addition, external auditory canal (EAC) atresia was evaluated by MRI in 29 fetuses with a gestational age > 28 weeks, and the accuracy of MRI in the diagnosis and classification of microtia was determined. RESULTS: Of 95 fetuses, 83 were considered to have microtia on the basis of MRI, 81 were confirmed to have microtia, and 14 were found to be normal according to postnatal diagnosis. Among 190 external ears in 95 fetuses, 40 ears were suspected to have mild microtia, and 52 ears were suspected to have severe microtia on the basis of MRI. According to the postnatal diagnosis, mild and severe microtia were confirmed in 43 and 49 ears, respectively. Among the 29 fetuses with a gestational age > 28 weeks, 23 ears were suspected to have EAC atresia according to MRI and 21 ears were ultimately confirmed to have EAC atresia. The accuracy of MRI in diagnosing microtia and EAC atresia was 93.68% and 93.10%, respectively. CONCLUSION: MRI shows good performance in diagnosing fetal microtia and has the potential to evaluate its severity on the basis of classification and EAC status. CLINICAL RELEVANCE STATEMENT: This study was aimed at investigating the role of MRI in the diagnosis and classification of fetal microtia. MRI shows good performance and can help evaluate microtia severity and EAC atresia, thus allowing for better clinical management. KEY POINTS: • MRI is a useful adjunct to prenatal ultrasound. • MRI has a higher accuracy rate than ultrasound in diagnosing fetal microtia. • The accurate classification of fetal microtia and the diagnosis of external auditory canal atresia through MRI may help guide clinical management.

Correlations Between Mandible Condylar Structures and External Ear Deformities in Hemifacial Microsomia With Three-Dimensional Analysis.

ABSTRACT: This study aimed to explore correlations between mandible and ear deformities and quantitative volumetric relations between condylar structures and external ear in hemifacial microsomia. The authors reconstructed three-dimensional craniofacial models from 212 patients with unilateral hemifacial microsomia (the unaffected side as the controls). Patients were evaluated by Pruzansky-Kaban and Marx classification, and divided into 3 age groups (0-6, 7-12, and >12 years of age). The mandible condylar structures, including condyle and the condylar skeletal unit, were selected (except the classification of the mandibular or ear deformities (M3)). Along with the external ear (except the classification of the mandibular or ear deformities (E4)), their volumes were measured and analyzed. Spearman correlation coefficient analysis was applied. There was a positive correlation between the mandible and ear deformities (r = 0.301, P   <  0.001). Either between the condyle and external ear ( P  = 0.071-0.493) or between the condylar unit and external ear ( P  = 0.080 - 0.488), there were no volumetric relations on the affected side, whereas on the unaffected side were (r = 0.492-0.929 for condyle, r = 0.443-0.929 for the condylar unit, P  < 0.05). In most cases, the condylar structures of the classification of the mandibular or ear deformities (M2b) were significantly smaller than the classification of the mandibular or ear deformities (M2a). Results suggested deformities of mandibular condylar structures and ear did not correlate, although deformities of mandible and ear did. The condylar deformity might develop independently from microtia and be more severe within relatively more abnormal temporomandibular joints.

Sonography of solitary fibrofolliculoma with histologic correlation.

We present a case of 15-year old male with solitary fibrofolliculoma on the ear and we demonstrate the use of ultrasound in outlining the features of this rare benign skin tumor with histological correlation. Fibrofolliculoma can be associated with a rare syndrome known as Birt-Hogg-Dubé which affects the skin, lungs and kidneys.

Anatomy and external landmarks of the superficial temporal artery using 3-dimensional computed tomography.

PURPOSE: The purpose of this study was to analyze the anatomical variability of the superficial temporal artery (STA) and to provide an easy visual landmark to find the STA and its branches to facilitate its surgical access. METHODS: A retrospective study was conducted on 57 patients who underwent a head and neck computed tomography with contrast injection. A visual landmark running from the tragus to the corner of the eye was used: the "eye-tragus-line" (ETL). On the ETL, the distance between the tragus and the STA was measured. The length of the STA main branch, its parietal and frontal branch and the angle of the STA and its branches with the ETL were measured. The division of the STA was studied as above/at the same level/below the zygomatic arch (ZA) and the ETL. RESULTS: The STA division was located above the ZA in 61.54% of cases, at the same level in 26.92% of cases and below in 11.54% of cases. Regarding the ETL, 93.27% of the STA divisions were located above the ETL, 5.77% at the same level and 0.96% below. On the ETL, the STA was located 15.55 ± 4.5 mm in front of the tragus. CONCLUSION: This study allowed to define an easy visual landmark: the ETL running from the tragus to the corner of the eye. The STA main branch was located 15.55 ± 4.5 mm of front of the tragus on the ETL. The STA division was nearly always located above the ETL (99.04%). Furthermore, this study provides a statistical representation of the anatomy of the STA and its branches.

Ethyl Alcohol Versus Botulinum Toxin A: A Comparative Study of the Visual and Histopathological Outcomes in the Rabbit Anterior Auricular Muscle Model.

BACKGROUND: Botulinum toxin has long been known for its paralytic effects at the neuromuscular junction. Although it has been widely used for vascular and nervous tissues, there has been no study of the aesthetic effects of the application of ethanol to muscle tissues to date. OBJECTIVE: The authors aimed to demonstrate the effects of the application of ethanol to muscle tissues after an intramuscular injection and to compare the effects of botulinum toxin A (BTA) and ethanol. METHODS AND MATERIALS: A total of 28 rabbits were divided into 4 groups (n = 7 each). Botulinum toxin A (5 units) and different concentrations of ethanol (5 cc) were injected into the left and right anterior auricular muscles of all rabbits, respectively. Ear ptosis was assessed, and histopathological examination was performed after all rabbits were euthanized in the eighth week. RESULTS: Muscle function was affected earlier in ethanol-treated ears than in botulinum-treated ears; however, the ptotic effect lasted for a significantly shorter duration in ethanol-injected ears than in BTA-applied ears. CONCLUSION: Ethanol can block muscle function reversibly and can serve as an alternative to BTA, particularly when rapid results are desirable.

The Study of Using 3D Scan Technique to Evaluate the Expanding Method of Ear Reconstruction Before Operation.

PURPOSE: Three-dimensional scanning technology was used to measure the expansion of the area and size of auricular skin to meet the normal standard of the external ear before ear reconstruction among microtia patients. MATERIALS AND METHODS: The skin surface area of microtia patients was measured by three-dimensional scanner: the surface area (S), vertical length (A), vertical curve length (B), transverse length (C), transverse curve length data (D), and then taking the average. Corresponding measurements in healthy adults were also obtained: surface area (S0), the vertical curve length (B0), and transverse curve length (D0) of the normal external ear were obtained by scanning normal adult male ears with reference to the range of the vertical length and the transverse straight length. Mean surface area (S and S0), vertical curve length (B and B0), and transverse curve length (D and D0) were compared between microtia patients and healthy adults. RESULTS: The surface area, vertical curve length, and transverse curve length were statistically significantly higher among healthy adults. CONCLUSIONS: With the amount of expanded water injection of 120-130 ml, the expanded skin still does not reach the standard of the normal external ear in terms of skin surface area and size. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

Validity and reliability of three-dimensional costal cartilage imaging for donor-site assessment and clinical application in microtia reconstruction patients: A prospective study of 22 cases.

OBJECTIVES: This study assesses the ability to reconstruct costal cartilage images by using three-dimensional visualisation software (Mimics) based on semi-automated segmentation algorithm and to investigate its reliability and validity with an anthropometric analysis. DESIGN: Observational prospective study. SETTING: Plastic surgery department of a tertiary hospital. PARTICIPANTS: Twenty-two microtia patients who underwent autologous ear reconstruction. MAIN OUTCOME MEASURES: Preoperative thoracic computed tomography data were processed to Mimics software for three-dimensional costal cartilage imaging. The length, width, thickness and volume of the 9th costal cartilages were calculated from these images and compared with the direct measurements (DM) obtained intraoperatively. RESULTS: The intra-examiner reliability and inter-examiner reliability were high in terms of all four measurements (intraclass correlation coefficients, ICC: 0.876-0.984). There were no significant differences between image-based anthropometry and DM in the linear measurements except for the volume (P < .05). The mean volume calculation error of Mimics was -0.08 ± 0.13 mL. No correlation was found between the anthropometric variables and the absolute errors (P > .05). Furthermore, Bland-Altman plots were used to evaluate the agreement between the two methods. CONCLUSIONS: Despite a very small error was found in volume calculation, Mimics software was accurate and reliable in linear calculation. Three-dimensional costal cartilage imaging was found to be an efficient tool for morphological evaluation of costal cartilages. We believe that with the application of individualised cartilage models based on three-dimensional printing, the use of customised ear framework carving will be practicable in surgical training.

3D Ultrasound Evaluation of the Fetal Outer Ear: Novel Biometry Ratio and Comparison of Different Surface Display Modes.

BACKGROUND: The examination of the fetal ear is a promising but still challenging approach in prenatal diagnosis. OBJECTIVES: This study investigated a novel ear length/width ratio based on anatomical landmarks. Additionally, we compared different 3D ultrasound surface rendering modes regarding their potential to depict detailed structures of the outer ear. METHOD: We measured both the ear length and width of 118 fetal ears from 20 to 40 weeks of gestation to establish a length/width ratio. Additionally, we rendered the volumes in three different surface display modes and one adapted light position. Each image was scored regarding the visibility of distinct structures of the ear relief and indicator scores were evaluated for each mode. RESULTS: The median of the length/width ratio was 1.9 with a slight decline over the gestational period. The overall visibility of the ear structures differed noticeably between the four surface display modes (p < 0.001). The post hoc comparison showed that the display mode "TrueVue" resulted in the highest indicator scores. CONCLUSION: The length/width ratio based on anatomical landmarks of the ear could prospectively be used as a marker in syndrome detection. The study showed a superiority of the surface display mode "TrueVue" for examination of the detailed ear structures.

Midterm and long-term results of ethanol embolization of auricular arteriovenous malformations as first-line therapy.

OBJECTIVE: The objective of this study was to assess the midterm and long-term results of patients with auricular arteriovenous malformations (AVMs) treated by ethanol embolization. METHODS: Medical records of 35 patients (20 male, 15 female; age range, 10-59 years) with auricular AVMs between 2006 and 2016 were reviewed. The short-term results of 4 of the 35 patients were reported in 2009. They were included in this study with updated follow-up data. The data from the 31 new included patients have not been reported previously. Ethanol embolization was performed with a direct puncture approach. The nidus was eradicated by bolus injection of ethanol with manual compression whenever possible. Treatment outcomes were classified into four categories by assessing the resolution of symptoms as well as the degree of nidus devascularization between the baseline and follow-up angiography studies. RESULTS: A total of 86 embolization sessions were performed in 35 patients (mean, 2.5 ± 1.3 sessions). The dosage of ethanol used per single session was 12.0 ± 4.5 mL (range, 6.0-24 mL). All patients received post-treatment clinical follow-up (mean, 40.7 ± 25.8 months), and 28 patients received post-treatment imaging follow-up (mean, 34.3 ± 25.5 months). Ethanol embolotherapy was effective in all patients. Control was achieved in 16 patients (45.7%), and improvement was achieved in 18 patients (51.4%). One patient experienced recurrence. A total of 13 minor complications and 2 major complications occurred in 12 patients during the 86 treatment sessions (12/35 [34.2%]; 15/86 [17.4%]). All the complications resolved spontaneously. CONCLUSIONS: The midterm and long-term results of this study demonstrate that ethanol embolization alone is an effective option for auricular AVMs as first-line therapy with a mild risk of minor and major complications.

Anatomical Uniqueness of Ear Morphology: A Novel Metrical Approach through Three-Dimensional Superimposition.

Uniqueness of ear morphology has been a widely debated issue in cephaloscopy, but past studies used only two-dimensional approaches. In the current investigation, the right and left ears of 10 healthy adults were imaged twice by stereophotogrammetry at the interval of a few seconds. The ear images obtained from the two acquisitions were superimposed both within subject (group of matches) and among subjects (group of mismatches). A point-to-point root mean square distance was calculated between the two three-dimensional models. Differences according to side and group were assessed by two-way analysis of variance. In total, 200 superimpositions were performed. On average, the point-to-point root mean square distance was 0.31 mm in cases of matches and 1.43 mm in cases of mismatches: differences were statistically significant (p < 0.01). Results provided quantitative data for the assessment of uniqueness of ear morphology, highlighting differences based on their three-dimensional morphology.

Assessment of Masses of the External Ear With Diffusion-Weighted MR Imaging.

PURPOSE: To assess masses of the external ear with diffusion-weighted MR imaging. MATERIAL AND METHODS: Retrospective analysis of 43 consecutive patients with soft tissue mass of the external ear. They underwent single shot diffusion-weighted MR imaging of the ear. The apparent diffusion coefficient (ADC) value of the mass of the external ear was calculated. The final diagnosis was performed by biopsy. The ADC value correlated with the biopsy results. RESULTS: The mean ADC value of malignancy (=27) of external ear (0.95 ±â€Š0.19 × 10 mm/s) was significantly lower (p = 0.001) than that of benign (n = 16) lesions (1.49 ±â€Š0.08 × 10 mm/s). The cutoff ADC used for differentiation of malignancy from benign lesions was 1.18 × 10 mm/s with an area under the curve of 0.959, an accuracy of 93%, a sensitivity of 92%, and specificity of 93%. There was a significant difference in the ADC of well and moderately differentiated malignancy versus poorly and undifferentiated squamous cell carcinoma (p = 0.001), and stages I and II versus stages III and IV (p = 0.04) of squamous cell carcinoma. CONCLUSION: ADC value is a non-invasive promising imaging parameter that can be used for differentiation of malignancy of the external ear from benign lesions, and grading and staging of squamous cell carcinoma of the external ear.

[The facial nerve study of multi-slice spiral computed tomography in congenital abnormality of external and middle ear].

Objective:Images of temporal bone are obtained on Siemens 64 slices spiral CT scanner with high resolution scanning to describe the course of facial nerve for better preoperative evaluation of CAEMA.Method:To describe the course of facial nerve on rectangular coordinate system from three silce on HRCT, 29 patients(35 ears) are enrolled in our study in ENT department from November 2005 to March 2007 in the second affiliated hospital of sun yan sen university, who are all diagnosed not acquired deformity but CAEME, associated with no congenital deformity syndromes and no middle ear or mastoid operations before. Patients with bilateral ears deformity are 6, and unilateral ears deformity are 23 (13 right and 10 left) in the study group. The control group is the normal ears of unilateral ear deformity.Result:In the study group, the shortest distance from FN tympanic segment to oval window is shorter than that of the normal group (P< 0.05). In CAEME of unilateral ears the FN mastoid segment displaces anteriorly about 2.7 mm, and in CAEME of bilateral ears displaces 3.0mm, compared with the normal ears (P< 0.05). The deformity degree of auricle has correlations with anteriorly displacement of facial nerve mastoid segment (P< 0.05).Conclusion:The FN mastoid segment is anteriorly displacement in both bilateral and unilateral ear deformity. The deformity degree of auricle has correlations with anteriorly displacement of facial nerve mastoid segment. The lateral displacement of facial nerve doesn't occur usually in CAEME. The shortest distance between oval window and tympanic segment of FN become shorter than normal ears.

[Analysis of clinical diagnosis and treatment in patients with microtia in Klippel-Feil syndrome].

Objective: To analyze the clinical features and diagnostic and therapeutic procedure of microtia in Klippel-Feil syndrome(KFS),and to summarize the experiences on diagnosis and treatment of this kind of rare disease to avoid misdiagnosing. . Methods: Between May 2014 and July 2015,six patients with microtia were diagnosed with suspected cervical vertebral malformation through physical examination and X-ray. Then they underwent additional examinations to evaluate the degree of deformities and relative risks:pure tone test, chest CT,cervical spine CT,temporal CT, echocardiography and ultrasonic on kidney and ureters. Ear reconstruction was performed with soft tissue skin expander and autogenous rib cartilage framework. Results: The six patients were diagnosed as KFS with microtia, which had different degree of cervical fusion and thoracic vertebral fusion. Some of them had rib deformity, scoliosis, congenital renal malformation and so on. Of 6 patients, scar formation occurred in 1 case after ear reconstruction, whose new ears had good position and appearance at 1 month after stage Ⅱ. After operation,6 cases were followed up for 8-20 months (median,12 months),none of them had nerve injury. Five cases had completed the third stage. All of them were well-healed after one month. Three cases were followed up for 3-11 months, the reconstructed ears had a three-dimensional configuration, and the cranioauricular angle of the reconstructed ears were similar to the opposite ears Conclusions: The primary step of comprehensive therapy in microtia with KFS is to diagnose definitely. Enhancing perioperative management can reduce surgery-related risks. It is ought to pay attention to nerve injury in a long-term follow-up.

Oculoauriculofrontonasal Dysplasia Syndrome With Additional Clinical Features.

Oculo-auriculo-vertebral spectrum and frontonasal dysplasia are two well-known examples of dysmorphology syndromes. Oculoauriculofrontonasal syndrome (OAFNS) is a clinical entity involving the characteristics of both OAVS and FND and is thought to be a result of the abnormal development of structures in the first and the second branchial arches, including the abnormal morphogenesis of maxillary processes. Herein we report a case of OAFNS with cliteral hypertrophy, premaxillary teeth, and inguinal hernia, features not previously reported in the literature.

The functional single ventricle: how imaging guides treatment.

Functional single ventricle (FSV) encompasses a spectrum of severe congenital heart disease. Patients with FSV are living longer than decades prior resulting in more frequent imaging both for surgical planning and functional evaluation. At each stage of surgical intervention, imaging plays a critical role in detecting postoperative complications and preprocedural planning. This article describes the unique imaging findings, including complications, that are most important to the referring physician or surgeon at each surgical stage of FSV management. A description of lesions that embody the diagnosis of FSV is also included.

Absence of skin discoloration after transarterial embolization of a subcutaneous auricular arteriovenous malformation with PHIL.

BACKGROUND AND OBJECTIVE: One of the treatment options for arteriovenous malformations consists of embolization, with a choice of various embolic agents, with or without subsequent surgical excision. If embolization is offered without subsequent surgery, the embolic material will stay in situ, in which case the consistency and color become important in superficial lesions. The purpose of this case report is to describe if the use of a novel liquid embolic agent (PHIL) is well suited for treatment of superficial AVMs without subsequent surgery. CASE DESCRIPTION: A 30-year-old male presented with a painful reddish, pulsatile swelling of the left ear that had been present for more than 10 years. Angiography confirmed an arteriovenous malformation supplied by the superficial temporal artery and the posterior auricular artery. The lesion was successfully treated by embolization with PHIL, through the superficial temporal artery. A minute residual shunt, from the posterior auricular artery, was accepted. Immediate disappearance of pulsatile tinnitus was reported. Moreover, return of normal skin color was observed without discomfort from the embolic deposits. This result has been stable throughout one year of clinical follow-up. CONCLUSION: To our knowledge, this is the first case report describing PHIL embolization as a treatment option for superficial arteriovenous malformations without the necessity for subsequent surgery. The white color and rubbery consistency are beneficial characteristics of PHIL in treatment of subcutaneous lesions, especially in cosmetically relevant locations.