Successful use of ketamin combined with remifentanil in two patients with epidermolysis bullosa

J Clin Exp Invest www.jceionline.org Vol 3, No 3, September 2012 1 Dicle Üniversitesi Anesteziyoloji ve Reanimasyon AD, Diyarbakır, Türkiye 2 Akçaabat Haçkalı Baba Devlet Hastanesi, Anesteziyoloji ve Reanimasyon AD, Trabzon, Türkiye 3 Hacettepe Üniversitesi, Tıp Fakültesi Hastanesi, Anesteziyoloji ve Reanimasyon AD, Ankara, Türkiye Correspondence: İlker Öngüç Aycan, Dicle Üniversitesi Anesteziyoloji ve Reanimasyon AD Diyarbakır, Türkiye Email: ilkeraycan@gmail.com Received: 05.05.2012, Accepted: 28.05.2012 Copyright © JCEI / Journal of Clinical and Experimental Investigations 2012, All rights reserved JCEI / 2012; 3 (3): 3957 Journal of Clinical and Experimental Investigations doi: 10.5799/ahinjs.01.2012.03.0186


INTRODUCTION
There are more than 20 different described subtypes of epidermolysis bullosa.Recessive Dystrophic Epidermolysis Bullosa (RDEB), the most common subgroup of epidermolysis bullosa, is characterized by the presence of extremely fragile skin and painful blister formations in the skin in response to minor trauma, friction or pressure. 1Other common features that can be seen are extremity contractures, severe digit deformities, difficult airways and severe wound infections. 2 The common surgical procedures for patients with RDEB are balloon dilation of esophageal strictures, pseudosyndactyly release with or without skin graft; postsurgical or skin care related dressing changes, percutaneous endoscopic gastrostomy tube placement and circumcision. 1Because of the fragile skin and mucous membranes all of the anesthetic procedures during these operations should be handled gently in these children.For all these reasons, we used a combination of ketamine-remifentanil in order to minimize intervention to airway control.

CASE REPORT
We present the anesthetic management for twin patients with epidermolysis bullosa caried out during the syndactyly operation.15-year-old, 24 kg and 25 kg, twin sisters with epidermolysis bullosa underwent operation for syndactyly.Premedication was done with 0.5 mg/kg nasal midazolam.Routine monitorization was done during the operation.The electrocardiography electrodes were placed with using electrode gel over wet spanches and so they did not directly touch the patients' skin.Pulse oximetry was placed on both patients' anterior part of lower leg after oxytetracycline and polymyxine B eye pomade (Terramycin®, Pfizer, Istanbul, Turkey) was applied and that part was covered with wet spanch.Pulse strength was evaluated by palpating the carotid arteries of the patients instead of using routine non-invasive blood pressure monitorization cuffs.After pomade was applied and covered with wet spanch to the facial parts which could be in contact with facial masks, anesthesia induction was performed with 8% sevoflurane in 50% O 2 -50% N 2 O, gently without touching patients' faces.
After the anesthesia induction, we covered patients' faces with wet spanches and gently put the facial masks on their faces.Intravenous cannulation was done through the anterior part of lower leg and was fixed with only spanches, avoiding plaster usage.We made a small incision in the middle of the spanch to put over the intravenous line from that hole and after covering the iv line with spanches, we fixed it with plasters above the spanches.For the maintenence of anesthesia 2 mg/kg ketamine bolus and later infusion of 1 mg/kg/hr was given intravenously.Also 50 μg fentanyl and 0.1 μg/kg/ min remifentanil infusion was given to both patients.Endotracheal intubation was not done.Because the patients' spontaneous breathing was preserved enough, there was no need for ventilatory support, only O 2 was appropriate.Both operations were ended approximately in 4 hours.At the end of the operations, we stopped ketamine and remifentanil infusions and injected 2 mg intravenous morfin for postoperative pain control.

DISCUSSION
Since epidermolysis bullosa is an incurable disease, the current management is mainly focused on supportive care and prevention of complications. 3,4nder the circumstances, adequate sedation and analgesia while maintaining spontaneous ventilation as in our cases is superior to laringeal mask airway placement or endotracheal intubation.Intubation or LMA placement would increase the risk of damage to mucosal membranes and laringeal bullae formation and direct pressure and friction caused by a facemask can blister or peel off the extensively fragile skin. 2,5The anesthesiologists also try to minimize the trauma to the skin in order to prevent chronic blood loss in these patients who are probably anemic because of this reason. 1 intubation is needed in patients with RDEB, it should not be forgotten that the involvement of the skin and the mucous membranes in the regions of the face, the neck, and the oropharynx can produce considerable difficulties in their intubation because of limited mouth opening due to scarring and contractures at the corners of the mouth. 6nce ketamine allows stable hemodynamics and causes less respiratory depression, it can be the drug of preference in children with epidermolysis bullosa.In a case report of two children with epidermolysis bullosa published by Wu, 2 it was shown that during dressing changes and whirlpool bath propofol and ketamine infusions provided satisfactory sedation with significantly reduced narcotic requirements compared with propofol alone.And they did not observe any side effects like delirium, agitation or hallucinations which are the most common side effects of ketamine.Also we did not observe these side effects during intraoperative and postoperative management of our patients.
We have preferred to use the combination of ketamine with remifentanil and fentanyl in our patients similarly to the combination of ketamine with propofol in the study of Wu. 2 Ketamine may be a good choice for epidermolysis bullosa.
Brachial plexus anesthesia, epidural anesthesia, and spinal anesthesia allow the maintenance of airway patency with minimal epidermal-dermal damage and postoperative pain relief in some study with RDEB. 1 Baloch et al 7 used to spinal anesthesia and epidural analgesia for labour in a pregnant patient.
We preferred oral route for preoperative sedation of our patients.Our patients were 17 years old but also in small children intramuscular or rectal medication should be thought twice because of blister formation or perianal trauma.
Combining ketamine with remifentanil seems to be effective, simple, safe for airway management and practical way to provide general anesthesia and analgesia of EB patients without respiratory depression.