Anesthetic Management of a Patient with Epidermolysis Bullosa for Esophageal Dilatation: A Case Report
Ersin Kandemir, Begüm Gökdemir, Deniz Kaya, Figen Leblebici, Nedim Çekmen
Başkent Üniversitesi Ankara Hastanesi
Giriş: Epidermolysis bullosa (EB) is a mucocutaneous disorder characterized by fragility of skin and mucosal membranes to the mechanical stress resulting formation of blisters, erosions and scarring. (1) Anesthetic management of these patients can be challenging as all the practices done perioperatively (transporting, positioning, monitoring, airway management etc.) may cause harm to patient. (2, 3) We present a case report of an esophageal dilatation procedure for an 18-year-old female with dystrophic EB.
Olgu: She was suffering from dysphagia. Her weight was 42 kg. She had history of multiple esophageal dilatations and a microstomia operation. Both of her hands had pseudo-syndactyly deformities. (Figure 1) Her mouth opening was narrow. (Figure 2) In operating room, she moved from sketcher to operating table without assistance. All contact points were padded. Her right arm was wrapped with cotton gauzes and a blood pressure cuff was placed. Soft silicone adhesive dressings were used to secure monitoring units. All vital signs were normal. IV cannula inserted under guidance of ultrasonography. (Figure 3) After induction with Propofol 2 mg/kg, Lidocaine 1 mg/kg, Fentanyl 1 mcg/kg and Rocuronium 0.9 mg/kg; preoxygenated with a face mask for 3 minutes. A 6.0 mm endotracheal tube with stylet inserted into trachea under the guidance of video laryngoscopy. (Figure 4) Maintenance of anesthesia was provided with sevoflurane administration and remifentanil 0.05-0.1 mcg/kg/min IV infusion. Methylprednisolone 1 mg/kg was given. The procedure was done without complications. After discontinuation of sevoflurane and remifentanil, Sugammadex 2 mg/kg was given for reversal of neuromuscular blockade. She was extubated awake after following commands. There were no signs of airway edema, obstruction. She was discharged from hospital on the same day.
Tartışma ve Sonuç: Patients with EB may need surgeries or procedures for various reasons. Any kind of mechanical stress needs certain attention and anesthetic management plan to perform these procedures without complications.
|