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"autoresLista" => "Mainak Dutta, Indranil Chatterjee" "autores" => array:2 [ 0 => array:4 [ "nombre" => "Mainak" "apellidos" => "Dutta" "email" => array:1 [ 0 => "duttamainak@yahoo.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Indranil" "apellidos" => "Chatterjee" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Department of Otorhinolaryngology and Head-Neck Surgery, Medical College and Hospital, Kolkata, Kolkata, 700073, West Bengal, India" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Pediatric Surgery, Medical College and Hospital, Kolkata, Kolkata, 700073, West Bengal, India" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Macrostomia: la característica definitoria del espectro oculo-auriculo-vertebral" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 767 "Ancho" => 950 "Tamanyo" => 48130 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Macrostomia is an extremely rare pharyngeal arch dysmorphogenesis (∼1:300,000 live births), characterized by facial cleft (Tessier soft-tissue cleft group 7) where the oral commissure extends laterally with increasing severity through orbicularis oris, buccinator, and masseter (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>; Pre-operative, and following partial surgical repair, respectively). The cleft approximates the imaginary line joining tragus with the original coordinates for oral commissure, representing the line of postero-anterior fusion between maxillary and mandibular processes, failure to which leads to macrostomia. The condition is classically associated with appendages in the pre-auricular region, often involving the imaginary fusion line (arrows). While the maxillary and mandibular processes originate from first pharyngeal arch, the pre-auricular appendages result from duplication/excesses of first and second arches. Thus, pharyngeal arch dysmorphogenesis as the putative explanation of macrostomia and pre-auricular appendages makes the condition eligible for the oculo-auriculo-vertebral spectrum (OAVS). OAVS is a broad, complex family of heterogeneous multisystem disorders, predominated by craniofacial defects related to first and second arches. Microtia (including pre-auricular appendages), with hemifacial microsomia, constitute its minimum diagnostic criterion, and is the connecting link among other syndromic phenotypes, like the Goldenhar. The phenotype and embryology makes macrostomia an important component of OAVS. However, according to the EUROCAT (<a id="intr0005" class="elsevierStyleInterRef" href="http://www.eurocat-network.eu/">http://www.eurocat-network.eu/</a>), it is often not systematically recorded in some registries, might be because it is seldom encountered in routine clinical practice. For clinical otolaryngologists and pediatricians, it is therefore important to recognize this entity within the OAVS rather than a discrete clinical occurrence. This will ensure exploring other congenital stigmata in the head-neck, auditory and skeletal systems, and would help in providing optimum management and parental counseling.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Informed consent</span><p id="par0010" class="elsevierStylePara elsevierViewall">The authors have taken informed consent in written from the guardians of the child seeking permission to reproduce the clinical photographs that could reveal his identity.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Financial disclosure</span><p id="par0015" class="elsevierStylePara elsevierViewall">None declared.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflict of interests</span><p id="par0020" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Informed consent" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Financial disclosure" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflict of interests" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:2 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 767 "Ancho" => 950 "Tamanyo" => 48130 ] ] ] 1 => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 599 "Ancho" => 950 "Tamanyo" => 80235 ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/21735735/0000007000000001/v1_201902080719/S2173573519300079/v1_201902080719/en/main.assets" "Apartado" => array:4 [ "identificador" => "5869" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Images in otorhinolaryngology" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/21735735/0000007000000001/v1_201902080719/S2173573519300079/v1_201902080719/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173573519300079?idApp=UINPBA00004N" ]