General anesthesia in patients with porphyria

A case report

Abstract

Porphyrias can have cutaneous or extracutaneous manifestations. Among the most relevant skin manifestations, photosensitivity stands out (for this reason they were known as the “vampire disease”), which causes the appearance of blisters in photo-exposed areas, as well as scars, millium cysts, malar hypertrichosis and skin hyperpigmentation. The prototype of the extracutaneous clinic is the acute attack of porphyria, which is characterized by neurological and psychiatric manifestations, to which are added digestive symptoms such as crampy abdominal pain, nausea, vomiting, severe constipation, dark urine. The provocation of a porphyria crisis during anesthesia should be prevented with correct treatment of hypovolemia and fluid and electrolyte disorders, in addition to avoiding fasting, providing 2000 calories / day enterally or with 10% dextrose at high doses (300- 500 g / day) intravenously. The clinical equipment, even the operating room lights, can generate skin changes, so special care is necessary to avoid burns in patients with photosensitivity.