Anesthesia of child with anhydrotic ectodermic dysplasia with multiminicore myopathy

Purpose: To report the perioperative management of anesthesia and analgesia in a child presenting with the association of multiminicore myopathy (MMM) and anhydrotic ectodermic dysplasia (AED). Clinical features: An eight-year-old girl was admitted for elective orthope dic surgery of-the lower limbs. AED is a congenital dermatosis characterize d by the absence of sweating and subsequent problems with thermoregulation; in addition, maxillary hypoplasia and abnormal teeth can render intubation difficult. MMM is a rare congenital myopathy characterized by proximal mus cle weakness, stable in time or with a slow and progressive evolution. It c an involve respiratory muscles and be associated with severe cardiomyopathy . Moreover, MMM shares some characteristics with Central Core Disease which is known to be associated with malignant hyperthermia. Since depolarizing muscle relaxants and halogenated agents could not be use d, a combined propofol-based intravenous anesthesia with lumbar epidural an algesia was chosen. This combination provided stable anesthesia, smooth rec overy and excellent analgesia during and after the operation, without compl ications. Temperature was monitored closely during surgery and in the posto perative period. Conclusions: The association of MMM and AED requires rapid distinction betw een hyperthermia secondary to anhydrosis and malignant hyperthermia. The ma nagement should provide a << trigger-free >> anesthetic and optimal postope rative analgesia without sedation. if appropriate for the surgical procedur e, a combination of general with regional anesthesia is particularly attrac tive in achieving these objectives.