Anesthetic Approach to a Patient with Epidermolysis Bullosa: A Case Report

Year 2015, Volume: 5 Issue: 3, 192 - 195, 28.04.2015

Ersin Köksal , Nurullah Yılmaz Cengiz Kaya Yasemin Üstün Ali Şahinoğlu Fatih Özkan

https://doi.org/10.16899/ctd.94038

Abstract

Epidermolysis Bullosa (EB) is characterized by fragility and formation of blisters in the skin and mucosal membranes. Due to blisters on the airway and occurring new blisters as a result of anesthetic or surgical procedures may complicate anesthesia management. In this case report, we present the anesthetic approach of a 6 years old case with EB who underwent reconstructive surgery. After premedication and optimal monitorization, intravenous (IV) anesthesia and analgesia was administered. Associated with the oropharyngeal lesions and limitation to reach an adequate mouth opening, we preferred the facemask to maintain ventilation. Neither new lesions nor complications were detected during or after surgery.

 

Keywords

anesthesia; epidermolysis Bullosa; preoperative evaluation

Epidermolizis Büllozalı Hastada Anestezik Yaklaşım: Olgu Sunumu

Abstract

Epidermolizis bülloza (EB); cilt ve mukozalarda frajilite ve bül oluşumuyla karakterize bir hastalıktır. Özellikle havayolundaki büller, yapılacak girişimlere bağlı yeni bül oluşumu anestezi yönetimini güçleştirir. Bu sunumda rekonstrüktif cerrahi planlanan, 6 yaşındaki, EB tanılı olgunun anestezik yönetimi anlatıldı. Anestezi ve analjezi optimal monitörizasyon ve girişim sonrası intravenöz (iv) yoldan sağlandı. Ventilasyon ise orofaringeal lezyonların varlığı ve ağız açıklığındaki kısıtlılık nedeniyle yüz maskesi ile gerçekleştirildi. Yeni lezyon veya komplikasyon oluşmadı

Keywords

anestezi; epidermolizis bülloza; preoperatif değerlendirme

References

• Fine JD: Inherited epidermolysis bullosa: Past, present, and future. Ann N Y Acad Sci 2010; 1194: 213-22.
• Paller AS, Mancini AJ. Bullous disorders of childhood. Hurwitz Clinical Pediatric Dermatology. 4th ed. China: Elsevier Saunders; 2011; 303-313.
• Karaduman A. Inherited bullous diseases. Turkdem 2011; 45: 81-86.
• Saraf SV, Mandawade NJ, Gore SK, Padhye UD, Pereira CS. Epidermolysis bullosa: Careful monitoring and no touch principle for anesthesia management. J Anaesthesiol Clin Pharmacol. 2013; 29: 390-3.
• Fine JD. Review: Inherited epidermolysis bullosa. Orphanet J Rare Dis. 2010; 12: 1-17.
• Fine JD, Mellerio JE: Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009; 61: 367-84; quiz 385-6.
• Fine JD, Mellerio JE: Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol 2009; 61: 387-402; quiz 403-4.
• Furukawa LK, Krane E. Guidelines for the Anesthetic Management of Epidermolysis Bullosa (EB). Division of pediatric anesthesia and pain management. Lucile Salter Packard Children's Hospital. 2012; 1-20.
• Alkan M, Pampal HK, Demirel CB, Akbas B. Anaesthetic management for laparoscopic surgeries of patient with epidermolysis bulloza: Case report. J Med Res 2011; 9: 198 -201.
• Thomas J. Epidermolysis bullosa in children: Pathophysiology, anaesthesia and analgesia. S Afr J Anaesthesiol Analg. 2010; 16: 12-15.
• Ames WA, Mayou BJ, Williams K. Anaesthetic management of epidermolysis bullosa. Br J Anaesth. 1999; 82: 746-51.
• Sertoz N, Ayanoglu HO. Anesthetic management in a patient with epidermolysis bullosa. J Turk Anaesth Int Care. 2010; 38: 222-27.