Anesthesia for balloon dilatation of esophageal strictures in children with epidermolysis bullosa dystrophica: from intubation to sedation

Abstract

Dystrophic epidermolysis bullosa is a rare disease mainly affecting small children. They often have to undergo different surgical procedures, for example balloon dilatation following esophageal strictures to maintain oral intake and prevent malnutrition. So far, no specific treatment is available for patients with any forms of epidermolysis bullosa. However, more and more minimal invasive surgical techniques are used to manage symptoms of the disease, for example endoscopic balloon dilatation for severe esophageal strictures. In this article implications for the anesthetic management of children with dystrophic epidermolysis bullosa are described. General anesthesia and sedation techniques are feasible if specific prerequisites are fulfilled. The team providing anesthesia and endoscopy must be familiar with the nature and disabilities of this disease. Coordinated care of the involved disciplines is crucial for all planned procedures regarding perioperative management.