Search
Presentation Details
| Perioperative Management in Acute Intermittent Porphyria |
| Alexis Garcia Coan, WH Andrew Ryu, Lisa Corbett, Avital O'Glasser Oregon Health and Science University, Portland, OR, United States |
CASE PRESENTATION: A 49-year-old woman with known acute intermittent porphyria (AIP) was seen in pre-operative clinic in anticipation of an L5-S1 bilateral open discectomy. She was very careful to avoid triggers and did not experience daily AIP symptoms. However, periop AIP complications included neurovisceral attacks including abdominal pain, nausea, asthenia[AO1] , and increased bruising typically beginning 6 hours postop. At her hematologist’s recommendation, a recent periop course involved postop admission for a glucose gtt. With this plan, she experienced only mild breakthrough symptoms that improved with increase in the glucose drip rate. Taking heed from this previous surgery, she was admitted overnight after her laminectomy and received a glucose drip (D5 at 50ml/hr). She was discharged POD1 free from neurovisceral attack symptoms. DISCUSSION: Acute intermittent porphyria (AIP) is a rare inheritable caused by a partial deficiency in the enzyme hydroxymethylbilane synthase (HMBS) leading to a build-up of porphobilinogen. Neurovisceral attacks typically manifest as severe diffuse abdominal pain and can be associated with nausea/vomiting or constipation, hypertension and tachycardia, mood/psychiatric disturbance, and hyponatremia. Acute attacks happen due to physiological or environmental stressors, such as medications, infection, and fasting. For this patient, glucose infusions prevented her from entering a catabolic state, thus decreasing the buildup of porphobilinogen and staving off a neurovisceral attack. CONCLUSIONS: This case highlights how early identification of AIP triggers in the pre-operative setting can aid in avoiding the precipitation of a neurovisceral attack in the operative and post-operative settings to keep patients safe and improve their experience. Management might also include scheduling a patient as first case to minimize NPO times and utilizing a glucose drip. Heme arginate can be used for more severe symptoms. This website is a high yield resource for such patients: https://www.drugs-porphyria.org/index2.php?lang=Gbr |