Anesthetic Implications of a Pediatric Patient with Stiff Skin Syndrome: A Case Report

Stiff Skin Syndrome (SSS) is a rare genetic syndrome characterized by rock-hard, indurated skin and joint contractures that worsen with age. Difficulties with mask ventilation should be expected due to the restrictive effect of the skin overlying the chest and abdomen, especially after muscle relaxation. Endotracheal intubation is often challenging, and advanced devices should be immediately available. SSS patients may experience diffuse entrapment peripheral neuropathy at contracted joints increasing the vulnerability to intraoperative nerve damage. Oral psoralens can cause nausea so antiemetic prophylaxis should be considered for SSS patients on PUVA therapy.