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Proposed Perioperative Management of A Parturient with Type I Hereditary Angioedema: Multidisciplinary Team Approach
Abstract Number: F-69
Abstract Type: Case Report/Case Series
Hereditary Angioedema (HAE) is a rare, autosomal dominantly inherited blood disorder. Some forms of HAE arise from deficiency or dysfunction of C1 inhibitor (C1INH). HAE, Type I represents approximately 80 to 85% of HAE cases and is characterized by reduced secretion of the C1INH protein. Patients present with episodic attacks of swelling that may affect the face, extremities, genitals, gastrointestinal tract and upper airways. Although the swelling is self-limited and resolves in two to five days without treatment, laryngeal involvement may cause fatal asphyxiation (1,2). Pregnancy and labor in these patients are often challenging requiring close follow up, specific interventions and multidisciplinary planning (3). We describe a case of a 21 year old G1P0 female patient with a documented diagnosis of Type I Hereditary Angioedema and absent C1 inhibitor function who presented for an elective cesarean section. Patient had multiple ER visits and ICU admissions for HAE flare-ups with life threatening airway swelling that required multiple emergent intubations. A multidisciplinary care plan was outlined before delivery, which included personnel from Labor and Delivery, Anesthesiology, Allergy medicine and ENT. We concluded that perioperative administration of C1 inhibitor therapy seems to be essential for short term prophylaxis, with regional anesthesia as a safe option, thus providing surgical anesthesia and avoiding airway manipulation.
1- Tosi M. Molecular genetics of C1 inhibitor. Immunobiology 1998; 199:358.
2- Prada AE, Zahedi K, Davis AE 3rd. Regulation of C1 inhibitor synthesis. Immunobiology 1998; 199:377
3- Nathani F, Sullivan H, Churchill D. Pregnancy and C1 esterase inhibitor deficiency: a successful outcome. Archives of Gynecology and Obstetrics. 2006;274(6):381-4.