CMT disease: peripartum management of two contrasting clinical cases

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Int J Obstet Anesth. 2007 Jan 31

Charcot-Marie-Tooth disease: peripartum management of two

contrasting clinical cases.

Greenwood JJ, WE.

Department of Anaesthesia, Derby City General Hospital, Derby, UK.

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.