Epidermolysis bullosa: Careful monitoring and no touch principle for anesthesia management
A 6-year-old male child, weighing 14 kg; a known case of EB since birth presented with congenital left hand syndactyly []. He was posted for syndactyly release. He required frequent hospital admissions for repeated episodes of chest infection and anemia. Significant preoperative findings were generalized scars, bed sores, pustules, and joint contractures. Airway assessment revealed Mallampatti Class III, microstomia, loose teeth, and poor oral hygiene with thyromental distance 3 cm. Patient also had limited neck extension secondary to scarring [].\nOral antibiotics were administered preoperatively. Informed consent was taken for surgery. Hydrocortisone skin ointment was applied to skin. Intravenous (IV) line 22-G secured with use of EMLA and fixed with vaseline gauze.\nPremedication with IV fentanyl 20 μg, midazolam 0.4 mg, and dexamethasone 2 mg was given. Anticholinergics were avoided. Antacid and antibiotic prophylaxis were given with IV ranitidine 1 mg/kg and cefotaxime 50 mg/kg, respectively. Intraoperative heart rate, rhythm, and oxygen saturation were monitored with lubricated clip on pulse oximeter. Noninvasive blood pressure (NIBP) and electrocardiogram monitoring were avoided []. Patient was positioned supine and pressure points were padded with cotton. Due to anticipated difficult airway, a difficult airway cart was prepared with various size masks, laryngoscope, endotracheal tubes (ETs), ventilating bougie, and stylet. Though supraglotic airway devices were inappropriate in this patient they were kept ready for an emergency airway situation.\nPreoxygenation was followed by induction with IV propofol 20 mg along with oxygen and sevoflurane under gentle mask holding with vaseline gauze. After confirming ventilation, IV atracurium 0.5 mg/kg was given. Minimal pressure for chin lift and head tilt was used for ventilation. Airway was lubricated to prevent mucosal trauma. With use of lubricated Macintosh laryngoscope trachea was intubated with uncuffed ET no. 5 and tube secured with nonadhesive lubricated bandage []. Eye padding was done with cotton to prevent ophthalmic complications.\nAnesthesia was maintained with oxygen, nitrous oxide, and sevoflurane with manual ventilation using Jackson Rees circuit. As rectal suppositories, subcutaneous and intradermal routes are generally not recommended; postoperative analgesia was provided with IV diclofenac sodium 25 mg 8 hourly. Regional block was avoided due to joint contracture and scars, which made location of anatomical landmarks difficult. Syndactyly release was performed without the use of tourniquet uneventfully []. Neuromuscular blockade was antagonized at the end of the procedure, and trachea extubated when adequate signs of spontaneous recovery were evident. Postoperatively, oral examination revealed small intra-oral blister formation not requiring active management. Patient was observed for 2 h in recovery room for signs of respiratory obstruction.