title: [Anaesthetic management in a paediatric patient with a difficult airway due to epidermolysis bullosa dystrophica].
abstract: Dystrophic epidermolysis bullosa (DEB) is a rare inherited disorder characterized by blistering after minimal trauma. These blisters tend to form dystrophic scars, leading to limiting and life-threatening sequelae. The anaesthetic management of patients with DEB is a challenge, even for the most experienced anaesthesiologists, but basic principles can help us prepare the plan of care. The main goals are to prevent trauma/infection of skin/mucous, and to establish a secure airway without causing bullae. Patient positioning and the instruments used to monitor vital signs and administering anaesthetic agents can cause new lesions. It is advisable to lubricate the instruments and to avoid adhesive material and shearing forces on the skin. Besides the implications of the comorbidities, there is a potential difficult intubation and difficult vascular access. Acute airway obstruction can occur due to airway instrumentation. We report the case of a patient diagnosed with EBD difficult airway and undergoing correction of syndactylyl and dental extractions.
authors.affiliation: UGC Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario Virgen Macarena, Universidad de Sevilla, Sevilla, España. Electronic address: eva_bg@msn.com.
authors.forename: E
authors.initials: E
authors.lastname: Blázquez Gómez
authors.affiliation: Servicio de Anestesiología, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, España.
authors.forename: A
authors.initials: A
authors.lastname: Garcés Aletá
authors.affiliation: Servicio de Anestesiología, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, España.
authors.forename: E
authors.initials: E
authors.lastname: Monclus Diaz
authors.affiliation: Servicio de Anestesiología, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, España.
authors.forename: F
authors.initials: F
authors.lastname: Manen Berga
authors.affiliation: Servicio de Cirugía Pediátrica, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, España.
authors.forename: L
authors.initials: L
authors.lastname: García-Aparicio
authors.affiliation: UGC Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario Virgen Macarena, Universidad de Sevilla, Sevilla, España.
authors.forename: A
authors.initials: A
authors.lastname: Ontanilla López
mesh_terms: D000293:Adolescent; D058109:Airway Management; D000402:Airway Obstruction / Q000209:etiology*; D000769:Anesthesia, Inhalation / Q000379:methods*; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D001279:Atracurium / Q000008:administration & dosage / Q000031:analogs & derivatives; D002405:Catheterization, Central Venous / Q000379:methods; D002921:Cicatrix / Q000209:etiology; D019468:Disease Management; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000473:pathology; D005283:Fentanyl / Q000008:administration & dosage; D006801:Humans; D007431:Intraoperative Complications / Q000517:prevention & control; D008297:Male; D008738:Methyl Ethers / Q000008:administration & dosage; D011183:Postoperative Complications / Q000517:prevention & control; D012121:Respiration, Artificial; D000077149:Sevoflurane; D013576:Syndactyly / Q000601:surgery; D014081:Tooth Extraction
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D001279:Atracurium; D000077149:Sevoflurane; C101584:cisatracurium; D005283:Fentanyl
keywords: Airway management; Anaesthesia; Anestesia; Dystrophic epidermolysis bullosa; Epidermólisis ampollosa distrófica; Manejo de la vía aérea