ACTA FAC. MED. NAISS. 2005; 22 (3): 157-160

Ivana Budić, Dejan Novaković, Vesna Marjanović, Zoran Marjanović, Ružica Milićević, Stevan Jovžić, Danijela Đerić
Clinic for Pediatric Surgery and Orthopedics, Clinical Center Niš

SUMMARY

The paper describes the anesthetic management of a neonate with pyloric atresia - junctional epidermolysis bullosa (PA-JEB) syndrome. Anesthesia for the neonate with PA-JEB syndrome can stand for a serious challenge even to the most experienced anesthesiologists. Therefore, a few basic principles will help to organize the necessary procedures. Shearing forces applied to the skin will result in bulla formation, while compressive forces to the skin are tolerated. Adhesive tape, adhesive ECG electrodes, adhesive pulse oximeter probes should not be used under any circumstances. Padding should be profusely used. Face masks should be lubricated with some emollient or coated with several layers of Vaseline gauze. All the instruments places into the mouth (laryngoscope, oropharyngeal airways) must be thoroughly lubricated with water based lubricant - do not lubricate with Lidocaine jelly. Pharyngeal suctioning should be avoided. Tracheal lesions do not appear after intubation probably because the trachea is lined with columnar epithelium. To reduce the risk of new laryngeal bullae formation, a tracheal tube a half to one size smaller than predicted may be necessary. With maximal skin and mucous membrane protection, anesthesia in children with PA-JEB syndrome may be conducted with a few sequelae.

Key words: PA-JEB syndrome, neonate, anesthesia