Herlitz epidermolysis bullosa in the paediatric patient: Anaesthetic implications
Epidermolysis bullosa is a group of inherited disorders characterized by blistering of the skin and mucous membranes as a result of friction or minor trauma. Clinical, genetic and histopathological criteria are involved in its diagnosis, distinguishing between simple (SEB), junctional (JEB) and dystrophic (DEB) forms. Among them, the junctional forms, especially de Herlitz JEB type and the one associated with pyloric atresia, pose a major challenge to the anaesthetist, given the high comorbidity.
We report the case of a newborn diagnosed with Herlitz epidermolysis bullosa, taken to nasojejunal transanastomotic tube placement due to congenital intestinal obstruction.
We focused on the main features in the anaesthetic management of these patients considering the preparation of the monitoring and surgical equipment to prevent skin damage by friction and the difficulties establishing venous accesses and airway management.
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