[Anesthetics management in patients with epidermolysis bullosa]. / Condotta anestesiologica in un paziente affetto da epidermolisi bollosa.

The anesthetic management used in a 4-yr-old boy with dystrophic epidermolysis bullosa (DEB) submitted to long-lasting urological surgery is reported. Medical treatment of cutaneous bullae in various stages of healing was undertaken preoperatively. Other preoperative therapies were performed to treat sepsis and to improve poor nutritional state. Different measures were used during monitoring and inhalation induction and maintenance of anesthesia in order to prevent frictional trauma to the skin and oropharyngeal mucosa, leading to blistering. Anesthesia and surgery proceeded uneventfully, while surgical complications and sepsis occurred postoperatively. However the patient was discharged from intensive care unit to surgical ward on 35 postoperative days without evidence of oropharyngeal bullae and with a good improvement of his cutaneous condition. The conclusions are drawn that, even if the patient with DEB represents a challenge to the anesthesiologist, a long-lasting surgical intervention can be performed successfully. The main aspect in the anesthetic management of these patients is the detailed knowledge of all problems that anesthesiologist must face and solve to preserve skin and mucosa integrity, avoiding the risk of severe complications.

[Anesthesiologic management of susceptible children at risk for malignant hyperthermia]. / Condotta anestesiologica in soggetti pediatrici a rischio di ipertermia maligna.

In order to prevent malignant hyperthermia (MH) in children, the authors are currently using a perioperative anaesthetic protocol. In vitro contracture tests with halothane and caffein are performed in selected patients: those with previous signs suggestive of MH, but not severe enough for a definite diagnosis; those with susceptibility to MH in their relatives; those affected by myopathies or other disorders very frequently associated with MH. Even though CK should be considered an unreliable predictor of MH susceptibility, trigger agents are avoided in children with high and constant CK levels. However, prophylactic dantrolene is never employed. In this way, over the last decade no episodes of classic malignant hyperthermia in a large number of children submitted to anaesthesia (more than 50,000) have been observed.