Intra- und postoperative Analgesie mit Kaudalkatheter bei einem Kind mit Schwartz-Jampel-Syndrom

The Schwartz-Jampel syndrome, also known as myotonia chondrodystrophica, is a rare autosomal recessive disorder characterized by bone dysplasia, growth retardation and generalized myotonia. Laryngoscopy and intubation may be difficult because of micrognathia and limited mouth opening due to myotonia of the masseter muscles. As regional anaesthesia reduces myotonic contractions and avoids administration of opioids causing respiratory depression, it appears to be the ideal method for postoperative analgesia. We report on a 5-year-old girl who underwent osteotomy of both hips and received intraoperative and postoperative analgesia via a caudal catheter. Excellent analgesia without myoclonic episodes could be achieved by continuous infusion of ropivacaine.