The presence of micrognathia indicates
potentially difficult airway management. Maintain spontaneous ventilation
and avoid neuromuscular blockers until the airway has been secured. Patient
positioning and vascular access may be difficult due to spasticity and
arthrogryposis. If kyphoscoliosis is clinically significant, preoperative
lung function tests, arterial blood gas analysis, and echocardiography may
be indicated. Regional anesthesia is not contraindicated per se. However,
given the clinical findings, it seems technically challenging (limited
cooperation and difficult positioning). Furthermore, in a progressive
demyelinating disease, the authors would not recommend a central neuraxial anesthesia
technique. Anxiolytic and sedative premedication (careful in patients with
cardiopulmonary compromise due to kyphoscoliosis) and the presence of the
primary caregiver during induction of anesthesia may be helpful. Kyphoscoliosis with
associated respiratory dysfunction increases the risk of postoperative
mechanical ventilation, which should be arranged for in advance.