Abstract ID: A46
Abstract Title: Continuous caudal anesthesia for an eight week old baby with severe Junctional Epidermolysis Bullosa (JEB)
Poster Type: Either
ABSTRACT BODY
Introduction:Epidermolysis bullosa (EB) is a group of mechanobullous disorders characterized by excessive susceptibility of the skin and mucosa to separate from underlying tissues and form bullae. There are three major subtypes (dystrophic EB, EB simplex and junctional EB) based on genetics, clinical features and histology. JEB is associated with severe panmucosal involvement (skin, respiratory, genitourinary and gastrointestinal)(1). We present the anesthetic management of a patient who had a rare form of JEB associated with pyloric atresia (PA-JEB).
Case report:Our patient (AN) was born prematurely at 34 weeks with a birth weight of 1900 gms. Apart from multiple ulcerated skin lesions she had pyloric stenosis that was repaired on day of life # 2. Due to airway involvement she had recurrent bouts of stridor and hypoxemia. At 7 weeks, she developed seizures and lethargy due to sagittal sinus thrombosis because of dehydration. She presented to the operating room requiring placement of femoral venous access. Because of the existing laryngeal blisters and mucositis, we chose to avoid tracheal intubation. Our choice of anesthetic was a continuous caudal infusion of 3% chloroprocaine for the duration of the surgery. The operating room was prepared with radiant warming lights. To reduce skin damage, she was placed on a crease-free gel roll with foam padding of extremities. No EKG stickers were applied. A blood pressure cuff was placed on the lower extremity after the skin was covered with paraffin gauze and cycled once every 15 minutes. An existing scalp intravenous catheter was used to give fluids.Following preparation of the skin with chlorhexidine spray, an 18 G intravenous catheter was inserted into AN’s caudal space. A 20 G epidural catheter was threaded and advanced to the upper lumbar level. Instead of taping , the catheter was secured by sterile petroleum gauze applied to the back. After negative aspiration from the catheter, a dose of 2cc/kg chloroprocaine bolus was given over 20 minutes and a 2cc/kg/hr infusion was started. To avoid irritating her face, we provided oxygen blowby under the drapes. Temperature monitoring was done every 20 minutes from the axilla using a digital temperature probe. A.N did not receive opioids or benzodiazepines during the procedure. To avoid placing the discharging pad on the patient’s skin, the surgeons used bipolar diathermy. The catheter was removed at the end of the one-hour surgery. No new lesions developed secondary to this procedure.
Discussion: Anesthesiologists caring for children with EB should avoid frictional mechanical forces that cause new bullae to form. The choice of anesthetic technique depends upon the patient’s disease and severity of existing lesions. Although general endotracheal anesthesia has been attempted successfully in other subtypes of EB (2, 3), airway manipulation should be avoided in JEB. Subcutaneous infiltration of local anesthetic is avoided because of the pathology of the disease. Therefore, neuraxial or regional blockade is an excellent option. Techniques like catheter placement are ideal for long-term postoperative pain control since oral and rectal routes are unavailable.
References:
1. Herod J. et al,Pediatr Anesth 2002, 2. Iohom G. et al., Euro Acad of Anesth 2001
3. Benavente M.A et al., Paediatr Anaesth 2003
ATTACHED FILES
Reg Anesth Pain Med 2004; 29(2):A46