Oromandibular-limb hypogenesis syndromes (OLHS): A challenge for airway management

Villabona, G.; Melgosa, I.; Iglesias, F.; González, A.M.

doi:10.1016/j.redare.2020.07.010

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Dentoskeletal deformity and left septal deviation." ] ] ] "textoCompleto" => "Oromandibular-limb hypogenesis syndrome (Moebius syndrome; Hanhart syndrome, Charlie M syndrome; hypoglossia-hypodactyly syndrome) involves several congenital malformations, typically: microglossia, mandibular micrognathia and finger abnormalities. We present the case of a 7-year-old patient, weight 25 kg, diagnosed with Hanhart syndrome. He presented craniofacial deformity, micrognathia, mandibular hypoplasia (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), retraction of the lower lip (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), bifid uvula, undeveloped tongue, dentoskeletal deformity and left septal deviation (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>), adactylia of the second and third fingers on the right hand and the second, third and fourth fingers on the left hand, and limited oral opening (12 mm, forced to 22 mm). We performed 2 interventions with airway management using deep sedation under spontaneous ventilation using midazolam (0.05 mg/kg iv), atropine (0.01 mg/kg) and a propofol: ketamine combination (8:4 mg/ml) administered in a 0.25 ml/kg/2 min bolus and perfusion of 0.2 ml/kg/h. The first intervention was performed with videolaryngoscope-assisted right nasal intubation, and the second with right nasal fibreoptic bronchoscopy.<elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Villabona G, Melgosa I, Iglesias F, González AM. Síndromes de hipogenesia dactilooromandibular (OLHS): un reto para el manejo de la vía aérea. Rev Esp Anestesiol Reanim. 2021;68:493–494." ] ] "multimedia" => array:3 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 865 "Ancho" => 900 "Tamanyo" => 100353 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "Multislice computed axial tomography with 3D reconstruction. 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ISSN: 2341-1929

Revista Española de Anestesiología y Reanimación (REDAR) -the Spanish Journal of Anesthesiology and Resuscitation- is the scientific body of the Spanish Society of Anesthesiology, Resuscitation and Pain Therapy. This is a monthly journal (10 issues) that publishes scientific articles of all areas within its specialty: clinical anesthesia, resuscitation-intensive medicine and critical care, diagnosis and treatment of acute and chronic pain, urgent care and emergencies and studies on basic science and related fields. The journal accepts work in both Spanish and English.

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