Renal ultrasound abnormalities in children with syndromic and non-syndromic microtia.

OBJECTIVE: Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia. METHODS: We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fisher's exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings. RESULTS: The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings. CONCLUSION: Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.

Surgical-Based Classification for Microtia.

The known classifications for microtia have been cumbersome and difficult to apply in daily consultation. The lack of a progressive surgical behavior according to each degree of affection also contributes to confusion and in a lower success rate in clinical application. The authors propose a progressive surgical classification that takes into consideration the principal anatomical defect and the corresponding correction with modern techniques, having eliminated from previous classifications, those elements that are now considered deformations of the ear, capable of conservative treatment in early stages of life.

Microtia: definición, clasificación y tratamiento: revisión de la literatura / Microtia: definition, classification and treatment: Literature review

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.

Phenotypic sub-grouping in microtia using a statistical and a clinical approach.

The clinical presentation of microtia varies widely from minimal morphological abnormalities to complete absence of the ear. In this study we sought to identify and characterize sub-groups of microtia using a statistical and a clinical approach. Photographs of 86 ears were classified in relation to all the external ear components. We used cluster analysis and rater's clinical opinion to identify groups with similar phenotypes in two separate analyses. We used Cramer's Phi coefficient of association to assess the similarity among the clinician's groupings as well as among the statistical sub-phenotypic groups and each of the clinician's groupings. The cluster analysis initially divided the 86 ears into a more and a less severe group. The less severe group included two sub-groups that included ears classified as normal and a group that had very few anomalous components. The group of 48 more affected ears all had abnormalities of the helix crus; antihelix-stem, -superior crus and -inferior crus; and antitragus. These were further divided into 4 sub-phenotypes. There was a moderate degree of association among the raters' groupings (Cramer's Phi: 0.64 to 0.73). The statistical and clinical groupings had a lower degree of association (Cramer's Phi: 0.49 to 0.58). Using standardized characterization of structural abnormalities of the ear we identified six distinct phenotypic groups; correlations with clinicians' groupings were moderate. These clusters may represent groups of ear malformations associated with the same etiology, similar time of insult or target cell population during embryonic development. The results will help inform investigations on etiology.

The importance of costal cartilage framework stabilization in microtia reconstruction: anthropometric comparison based on 216 cases.

This study explored anthropometric changes in the reconstructed auricle and the contralateral normal ear in a series of 216 microtia patients using different stabilization methods. Our main personal modifications concerning the preparation of the framework were the following: 1. The individualized framework grafting was based on patients with different ages and different degrees of the strength and thickness of the rib cartilage. 2. The framework was stabilized as a "C" shape by using a piece of cartilage or suture to reinforce the two end points of the "C". In group A (the thickness of cartilage was more than 5 mm), a block of residual cartilage fixed by wire was added between the tragus and the base frame of the inferior crus by the modified method but was not applied in the original method. In group B (the thickness of cartilage was less than 5 mm), a 4-0 braided suture was used to reinforce the two structures but was not used in the original method. No significant differences were found in the height or width measurements of the cartilage framework and the contralateral normal side in either group at the time of implantation. At the follow-up, the height and width measurements were obviously increased in both groups operated on by the original method compared with the initial implanted or contralateral normal measurements. There were no significant differences in the height or width measurements by the modified method in either group. The authors' techniques produced acceptable results and generated some useful parameters for the growth study of the reconstructed auricle and the contralateral normal ear. The modifications in framework stabilization allow a harmonious outline of the reconstructed auricle to be attained, which is almost symmetrical to the contralateral normal auricle.