Herlitz epidermolysis bullosa in the paediatric patient: Anaesthetic implications
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.
References
1. Iohorn G, Lyons B. Anaesthesia for children with epidermolysis bullosa: a review of 20 years' experience. Eur J Anaesthesiol. 2001;18:745-54.
2. Fine JD, Bauer EA, Briggaman RA, Carter DM, Eady RA, Esterly NB, et al. Revised clinical and laboratory criteria for subtypes of inherited epidermolysis bullosa. A consensus report by the Subcommittee on Diagnosis and Classification of the National Epidermolysis Bullosas Registry. J Am Acad Dermatol. 1991;24:119-35.
3. Marinkovich MP. Update on inherited bullous dermatoses. Dermatol Clin. 1999;17:473-85.
4. Herod J, Denyer J, Goldman A, Howard R. Epidermolysis bullosa in children: pathophysiology, anaesthesia and pain management. Pediatr Anaesth. 2002;12:388-97.
5. Griffin RP, Mayou BJ. The anaesthetic management of patients with dystrophic epidermolysis bullosa. A review of 44 patients over a 10 year period. Anaesthesia. 1993;48:810-5.
6. Basarab T, Dunnill MG, Eady RA, Russell-Jones R. Herlitz junctional epidermolysis bullosa: a case report and review of current diagnostic methods. Pediatr Dermatol. 1997;14:307-11.
7. Hauschild R, Wollina U, Bruckner-Tuderman L. Junctional epidermolysis bullosa gravis (Herlitz): diagnostic and genetic aspects. J Eur Acad Dermatol Venereol. 2001;15:73-6.
8. Fine JD, Eady RA, Bauer EA, Briggaman RA, Bruckner-Tuderman Christiano A, et al. Revised classification system for inherited epidermolysis bullosa: report of the second international consensus meeting on diagnosis and classification of epidermolysis bullosa. J Am Acad Dermatol. 2000;42:1051-66.
9. Goldschneider K, Lucky AW, Mellerio JE, Palisson F, del Carmen Viñuela Miranda M, Azizkhan RG. Perioperative care of patients with epidermolysis bullosa: proceedings of the 5th international symposium on epidermolysis bullosa, Santiago, Chile, December 4-6, 2008. Pediatr Anesth. 2010;20:797- 804.
10. James I, Wark H. Airway management during anesthesia in patients with epidermolysis bullosa dystrophica. Anesthesiology. 1982;56:323-6.
11. Fröhlich S, O'ullivan E. Airway management in adult patients with epidermolysis bullosa dystrophica: a case series. Anaesthesia. 2011;66:842-3.
12. Parodi-Díaz EM, Garcia-Vega MI, Gilsanz-Rodríguez F. Implicaciones anestésicas de la epidermólisis ampollosa. Act Anest Reanim. 2004;14:33-9.
13. Ho AM, Tsui CS. Epidermolysis bullosa in a newborn. Anesthesiology. 2012;116:925.
14. Ames WA, Mayou BJ, Williams KN. Anesthetic management of epidermolysis bullosa. Br J Anaesth. 1999;82:746-51.
15. Karabiyik L, Güngör I, Alp GG, Kaya K. Anesthetic management of epidermolysis bullosa: a review and report of two cases. Acta Anaesthesiol Belg. 2009;60:51-4.
16. White R. A multinational survey of the assessment of pain when removing dressings. Wounds UK. 2008;4.
17. Davies P, Rippon M. Evidence review: the clinical benefits of Safetac technology in wound care. J Wound Care. 2008; Suppl.:3-31.
18. White R, Morris C. Mepitel: a non-adherent wound dressing with Safetac technology. Br J Nurs. 2009;18:58-64.
19. Yonker-Sell AE, Connelly LA. Twelve hour anaesthesia in a patient with epidermolysis bullosa. Can J Anaesth. 1995;42:735-9.
20. Spielman FJ, Mann ES. Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa. Can Anaesth Soc J. 1984;31:549-51.
21. Goldschneider KR, Lucky AW. Pain management in epidermolysis bullosa. Dermatol Clin. 2010;28:273-82.
22. Allegaert K, Naulaers G. Gabapentin as part of multimodal analgesia in a newborn with epidermolysis bullosa. Paediatr Anaesth. 2010;20:972-3.
23. Howard R, Carter B, Curry J. Good practice in postoperative and procedural pain management: medical procedures. Association of paediatric anaesthetists of Great Britain and Ireland. Paediatr Anaesth. 2008;18(S1):19-35.
24. Uitto J, Eady R, Fine JD, Feder M, Dart J. The DEBRA international visioning/consensus meeting epidermolysis bullosa: summary and recomendations. J Invest Dermatol. 2000;114:734-7.
2. Fine JD, Bauer EA, Briggaman RA, Carter DM, Eady RA, Esterly NB, et al. Revised clinical and laboratory criteria for subtypes of inherited epidermolysis bullosa. A consensus report by the Subcommittee on Diagnosis and Classification of the National Epidermolysis Bullosas Registry. J Am Acad Dermatol. 1991;24:119-35.
3. Marinkovich MP. Update on inherited bullous dermatoses. Dermatol Clin. 1999;17:473-85.
4. Herod J, Denyer J, Goldman A, Howard R. Epidermolysis bullosa in children: pathophysiology, anaesthesia and pain management. Pediatr Anaesth. 2002;12:388-97.
5. Griffin RP, Mayou BJ. The anaesthetic management of patients with dystrophic epidermolysis bullosa. A review of 44 patients over a 10 year period. Anaesthesia. 1993;48:810-5.
6. Basarab T, Dunnill MG, Eady RA, Russell-Jones R. Herlitz junctional epidermolysis bullosa: a case report and review of current diagnostic methods. Pediatr Dermatol. 1997;14:307-11.
7. Hauschild R, Wollina U, Bruckner-Tuderman L. Junctional epidermolysis bullosa gravis (Herlitz): diagnostic and genetic aspects. J Eur Acad Dermatol Venereol. 2001;15:73-6.
8. Fine JD, Eady RA, Bauer EA, Briggaman RA, Bruckner-Tuderman Christiano A, et al. Revised classification system for inherited epidermolysis bullosa: report of the second international consensus meeting on diagnosis and classification of epidermolysis bullosa. J Am Acad Dermatol. 2000;42:1051-66.
9. Goldschneider K, Lucky AW, Mellerio JE, Palisson F, del Carmen Viñuela Miranda M, Azizkhan RG. Perioperative care of patients with epidermolysis bullosa: proceedings of the 5th international symposium on epidermolysis bullosa, Santiago, Chile, December 4-6, 2008. Pediatr Anesth. 2010;20:797- 804.
10. James I, Wark H. Airway management during anesthesia in patients with epidermolysis bullosa dystrophica. Anesthesiology. 1982;56:323-6.
11. Fröhlich S, O'ullivan E. Airway management in adult patients with epidermolysis bullosa dystrophica: a case series. Anaesthesia. 2011;66:842-3.
12. Parodi-Díaz EM, Garcia-Vega MI, Gilsanz-Rodríguez F. Implicaciones anestésicas de la epidermólisis ampollosa. Act Anest Reanim. 2004;14:33-9.
13. Ho AM, Tsui CS. Epidermolysis bullosa in a newborn. Anesthesiology. 2012;116:925.
14. Ames WA, Mayou BJ, Williams KN. Anesthetic management of epidermolysis bullosa. Br J Anaesth. 1999;82:746-51.
15. Karabiyik L, Güngör I, Alp GG, Kaya K. Anesthetic management of epidermolysis bullosa: a review and report of two cases. Acta Anaesthesiol Belg. 2009;60:51-4.
16. White R. A multinational survey of the assessment of pain when removing dressings. Wounds UK. 2008;4.
17. Davies P, Rippon M. Evidence review: the clinical benefits of Safetac technology in wound care. J Wound Care. 2008; Suppl.:3-31.
18. White R, Morris C. Mepitel: a non-adherent wound dressing with Safetac technology. Br J Nurs. 2009;18:58-64.
19. Yonker-Sell AE, Connelly LA. Twelve hour anaesthesia in a patient with epidermolysis bullosa. Can J Anaesth. 1995;42:735-9.
20. Spielman FJ, Mann ES. Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa. Can Anaesth Soc J. 1984;31:549-51.
21. Goldschneider KR, Lucky AW. Pain management in epidermolysis bullosa. Dermatol Clin. 2010;28:273-82.
22. Allegaert K, Naulaers G. Gabapentin as part of multimodal analgesia in a newborn with epidermolysis bullosa. Paediatr Anaesth. 2010;20:972-3.
23. Howard R, Carter B, Curry J. Good practice in postoperative and procedural pain management: medical procedures. Association of paediatric anaesthetists of Great Britain and Ireland. Paediatr Anaesth. 2008;18(S1):19-35.
24. Uitto J, Eady R, Fine JD, Feder M, Dart J. The DEBRA international visioning/consensus meeting epidermolysis bullosa: summary and recomendations. J Invest Dermatol. 2000;114:734-7.
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]. 2014 Apr. 1 [cited 2024 Apr. 25];42(2):132-5. 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]. 2014 Apr. 1 [cited 2024 Apr. 25];42(2):132-5. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/602
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Section
Case Report / Case Series
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