Manejo anestésico en paciente pediátrico con vía aérea difícil afectado de epidermólisis ampollosa distrófica / Anaesthetic management in a paediatric patient with a difficult airway due to epidermolysis bullosa dystrophica

La epidermólisis ampollosa distrófica (EAD) es una patología hereditaria infrecuente, caracterizada por la formación de ampollas ante mínimos traumatismos, que tienden a formar cicatrices distróficas y llevan a secuelas limitantes y amenazantes para la vida. Es frecuente que los pacientes afectados de EAD precisen diferentes intervenciones quirúrgicas a lo largo de su vida. Los principales objetivos del manejo anestésico son la prevención del traumatismo/infección sobre la piel o las mucosas y el establecimiento de una vía aérea segura sin producir ampollas. El posicionamiento del paciente y el material utilizado para la monitorización de signos vitales y para la administración de agentes anestésicos pueden producir nuevas lesiones. Debe lubricarse todo el material y evitar adhesivos y movimientos de fricción sobre la piel. Estos pacientes suelen presentar múltiples comorbilidades y accesos vasculares y vía aérea difíciles. La formación de nuevas ampollas durante la instrumentación de vía aérea puede llevar a una obstrucción aguda de la misma. Presentamos el caso de un paciente con EAD y vía aérea difícil sometido a corrección de sindactilia y exodoncia múltiple (AU)

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 (AU)

[Anaesthetic management in a paediatric patient with a difficult airway due to epidermolysis bullosa dystrophica]. / Manejo anestésico en paciente pediátrico con vía aérea difícil afectado de epidermólisis ampollosa distrófica.

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.