Charcot-Marie-Tooth disease: peripartum management of two contrasting clinical cases

Summary 

Charcot-Marie-Tooth disease is a rare hereditary motor and sensory demyelinating polyneuropathy with potentially severe and debilitating peripheral symptoms. Respiratory muscles and vertebral anatomy can be affected, both of which may have significant impact on any planned or unplanned anaesthetic intervention during labour. We describe two cases at opposite ends of a wide spectrum of disease severity. The first case illustrates an approach to the management of a mildly affected patient who was permitted to labour normally, but nevertheless needed a detailed antenatal plan in order to allay her anxiety and prepare for potentially complicated labour analgesia and operative delivery. Spontaneous delivery of a healthy infant did not require anaesthetic intervention during labour. The second woman had severe scoliosis and marked respiratory impairment and required non-invasive ventilatory support for one week before scheduled caesarean section. A single-shot spinal anaesthetic was used as a spinal catheter could not be sited. This produced a high block for which a brief period of respiratory assistance was required. Perioperative management and subsequent high dependency care are discussed.

Keywords: Charcot-Marie-Tooth disease, Complications, Labour, Analgesia, Caesarean section, Spinal anaesthesia, Scoliosis