General anaesthesia management with larengeal mask airway in a patient with Epidermolysis Bullosa: Case report
Fulya Yılmaz, Burcu Ulugölge,
Sağlık Bilimleri Üniversitesi, İzmir Bozyaka Eğitim Ve Araştırma Hastanesi, Anesteziyoloji Ve Reanimasyon Kliniği İzmir
INTRODUCTION:Epidermolysis bullosa (EB) is a rare (1/17000-1/300000) genetic disease which is characterized by an abnormal collagen and extreme fragility of skin and mucous membranes. Anaesthestic management of these patients requires attention: Co-morbidities, monitorization, venous cannulation, airway management, anaesthesia management, surgical concerns, pain management. We report a case with EB, managed uneventfully with general anaesthesia using larengeal mask airway. CASE REPORT:A 24-year-old woman (35 kg, 140 cm) with epidermolysis bullosa presented to operation room for local resection of mass on tuberositas tibia. Besides anemia and history of using drugs for EB, she had no other medical or surgical history. Significant preoperative findings were generalized scars, bed sores, joint contractures, amputation of fingers, poor oral hygine, microstomia, limited mouth opening and limited neck extension. No premedication was given on the operation day. The patient positioned herself on the operation table in a supine position. She was monitored with clip-on pulseoximeter, non-invasive blood pressure with padding underneath the cuff (measured when required), ECG was not monitored to avoid skin touch. Peripheric intravenous line was achieved (20 gauge cannula) on right tibia and was secured with gauze. Preoxygenation was followed by induction with propofol (80 mg) under gentle mask ventilation. Lumbricated larengeal mask airway No: 3 was used as a airway device. Her eyes were not closed spontaneously because of scars, so eyes were cared with gel to prevent ophthalmic complications. Anaesthesia was maintained with oxygen,air (50%:50%) sevoflurane and remifentanil infusion. She was extubated at the end of the surgery. Postoperative analgesia was provided with IV contramal. Postoperatively, oral examination was normal. Patient was observed for 2 hours in recovery room for signs of respiratory complications. CONCLUSION:Anesthesia management of EB cases requires features at all stages of anesthesia management. A multidisciplinary approach and careful planning are essential for safely management of surgery.
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