Anesthetic implications of a pediatric patient with stiff skin syndrome: A case report

Abstract

Stiff skin syndrome is a rare genetic disorder that is present in infancy or early childhood. It is characterized by hard, inflexible skin and limited joint mobility making anesthetic management of these patients challenging. Their limited neck flexibility and chest wall rigidity make intubation and mask ventilation difficult. Intraoperative positioning can be challenging due to joint contractures and potential entrapment peripheral neuropathy. Even though peripheral intravenous access can be relatively easy, central venous cannulation may be problematic due to the hard skin overlying the entry sites. Our case report details the anesthetic management and considerations of a pediatric patient with stiff skin syndrome.