Herlitz epidermolysis bullosa in the paediatric patient: Anaesthetic implications

  • J.C. Galán Gutiérrez Anaesthesiology and Reanimation Service, Hospital Universitario Central de Asturias, Oviedo, Spain
  • M.A. Martínez Suárez Anaesthesiology and Reanimation Service, Hospital Universitario Central de Asturias, Oviedo, Spain
  • B. Tobera Noval Gynaecology and Obstetrics Service, Hospital de Cabueñes, Gijón, Spain
  • R. Avello Taboada Anaesthesiology and Reanimation Service, Complejo Hospitalario de Burgos, Spain
Keywords: Epidermolysis Bullosa, Anesthesia, Infant, Newborn, Anestesiology, Perioperative Period

Abstract

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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How to Cite
1.
Galán Gutiérrez J, Martínez Suárez M, Tobera Noval B, Avello Taboada R. Herlitz epidermolysis bullosa in the paediatric patient: Anaesthetic implications. Colomb. J. Anesthesiol. [Internet]. 2014Apr.1 [cited 2021Jun.24];42(2):132 -135. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/602

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Published
2014-04-01
How to Cite
1.
Galán Gutiérrez J, Martínez Suárez M, Tobera Noval B, Avello Taboada R. Herlitz epidermolysis bullosa in the paediatric patient: Anaesthetic implications. Colomb. J. Anesthesiol. [Internet]. 2014Apr.1 [cited 2021Jun.24];42(2):132 -135. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/602
Section
Case Report / Case Series

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