The Management of Pregnancy with Hereditary Angioedema

Introduction Although a rare disease, hereditary angioedema can be potentially life threatening. It manifests as recurrent episodes of localized edema that can appear in all areas of the body, most frequently at the level of skin, respiratory and gastrointestinal tract. It is due to a deficiency of C1 esterase inhibitor. There is scares data as far as the management of the pregnant patient with hereditary angioedema is concerned. Case presentation Female patient diagnosed clinically with hereditary angioedema and confirmed biologically -C1 esterase inhibitor < 28% (normal level -100%), with a family history of angioedema (mother, aunts, cousins), with recurrent episodes of angioedema that were triggered by effort or by local anesthesia, had a regular obstetrical follow up since the 6th week of pregnancy. During her gestation, she was diagnosed with thrombophilia with multiple heterozygote gene mutation profile that involved: MTHFR C677T, MTHFR, factor XIII V34L, PAI-I 4G/5G, that proved to have a low/medium risk of thromboembolic events. The hematologist opted for clinical and biological supervision of the associated pathology. The delivery of the baby was through a scheduled C-section accompanied by prophylactic administration of C1-INH concentrate (Berinert). The evolution was favorable both for the mother and the newborn. Discussions These particular cases require multidisciplinary management. In this case, there was a complex team formed of obstetrician, anesthesiologist, general surgeon, hematologist, and allergologist and neonatologist, who elaborated a therapeutic plan. Although all the women with angioedema from the patient's family gave birth vaginally without any complications, this mode of delivery was excluded due to the unpredictability of the disease, the associated thrombophilia, and due to the risk to the fetus (that could possibly inherit the disease). Delivering through C-section was also a difficult decision to make taking into consideration the surgical record of the patient's mother, who developed intraoperative severe angioedema.