Severe neurologic manifestations in acute intermittent porphyria developed after spine surgery under general anesthesia: a case report

Abstract

Porphyrias are inherited metabolic disorders resulting from a specific enzyme defect in the heme biosynthetic pathway. Porphyrias are induced by various precipitants. Clinical features include abdominal pain, neurologic manifestations, autonomic neuropathy, and mental disturbance. Diagnosis may be delayed because of variable symptoms that mimic other diseases and because of the rarity of of porphyrias. Although most patients with known porphyria can complete anesthesia and surgery safely, undiagnosed porphyric patients are in danger of porphyric crisis due to inadvertent exposure to precipitating drugs and environment. We report a case of a patient who experienced delayed emergence with neurological disturbance after general anesthesia, ultimately diagnosed as acute intermittent porphyria.

Acute intermittent porphyria after right hemi-colectomy

“…Medline search revealed only five cases of AIP after surgery (Table 1) [9], [10], [11], [12], [13]. Other triggers that increase the demand for heme and hence induce AIP include porphyrinogenic drugs, infections, smoking, alcohol intake, fasting, psychological stress, and hormonal changes associated with menstruation and ovulation [1], [2], [3].…”

“…Other differential diagnoses of AIP include psychosis, Guillian Barré syndrome, and encephalitis [7], [11], [13]. Hence, a high index of suspicion is needed to diagnose AIP.…”

“…Most likely, her attack of AIP was precipitated by a combination of (1) hormonal changes during menstruation, (2) epidural analgesia of lidocaine and fentanyl, (3) low-calorie intake during the postoperative period (510 Kcal/day) and (4) surgical site pain after cessation of epidural analgesia [1] , [11] . Medline search revealed only five cases of AIP after surgery ( Table 1 ) [9] , [10] , [11] , [12] , [13] . Other triggers that increase the demand for heme and hence induce AIP include porphyrinogenic drugs, infections, smoking, alcohol intake, fasting, psychological stress, and hormonal changes associated with menstruation and ovulation [1] , [2] , [3] .…”

“…From the surgical point of view, AIP can be mistaken for other causes of abdominal pain such as acute appendicitis, acute cholecystitis, intestinal obstruction, gastric perforation, bowel ischemia, and ovarian torsion [3] , [5] , [6] , [7] , [8] . Other differential diagnoses of AIP include psychosis, Guillian Barré syndrome, and encephalitis [7] , [11] , [13] . Hence, a high index of suspicion is needed to diagnose AIP.…”

“…Acute intermittent porphyria after surgery is very rare with only few case reports in the literature [9] , [10] , [11] , [12] , [13] . We present what we believe is the first case of AIP after right hemi-colectomy.…”