Anaesthetic management in a paediatric patient with a difficult airway due to epidermolysis bullosa dystrophica

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 exp...

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Bibliographic Details
Published in:	Revista española de anestesiología y reanimación Vol. 62; no. 5; p. 280
Main Authors:	Blázquez Gómez, E, Garcés Aletá, A, Monclus Diaz, E, Manen Berga, F, García-Aparicio, L, Ontanilla López, A
Format:	Journal Article
Language:	Spanish
Published:	Spain 01-05-2015
Subjects:	Adolescent Airway Management Airway Obstruction - etiology Anesthesia, Inhalation - methods Anesthetics, Inhalation - administration & dosage Atracurium - administration & dosage Atracurium - analogs & derivatives Catheterization, Central Venous - methods Cicatrix - etiology Disease Management Epidermolysis Bullosa Dystrophica - complications Epidermolysis Bullosa Dystrophica - pathology Fentanyl - administration & dosage Humans Intraoperative Complications - prevention & control Male Methyl Ethers - administration & dosage Postoperative Complications - prevention & control Respiration, Artificial Syndactyly - surgery Tooth Extraction Anaesthesia Epidermólisis ampollosa distrófica Manejo de la vía aérea Anestesia Airway management Dystrophic epidermolysis bullosa
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Summary:	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.
ISSN:	2340-3284
DOI:	10.1016/j.redar.2014.08.003