Anesthesia for herniotomy in schwartz–jampel syndrome

Abstract EN

Patients with neuromuscular disorders may develop specifi c anaesthetic complications; these include pulmonary insuffi ciency, cardiac arrhythmias, thermoregulatory instability and technical diffi culties with tracheal intubation.

We describe the anaesthetic management of a child with a rare autosomal recessive disorder, Schwartz-Jampel syndrome, characterised by various musculoskeletal disorders and a potential for thermoregulatory dysfunction.