PREGNANCY MANAGEMENT AND DELIVERY OF A PATIENT WITH HEREDITARY ANGIOEDEMA, A CASE REPORT: P-0015 | POSTER | CESAREAN SECTION DILEMMA
Publication Date: 2017/10/14,
Abstract:
Background: Hereditary angioedema (HAE) is a rare genetic disease caused by mutations on the C1 esterase inhibitor gene. The incidence in the general population is estimated between 1 in 10,000- 1 in 150,000. The onset of HAE is usually during adolescence and it presents with episodic edema of the upper and lower extremities, the face, the vulva, upper respiratory airways and the gastrointestinal tract. Laryngeal oedema is life-threatening and accounts for the majority of deaths in patients with the condition.
Case report: We present the case of a 23-year-old primigravida, diagnosed with HAE at the age of 21. Her symptoms outside pregnancy included frequent attacks of peripheral edema, epigastric pain and occasional respiratory symptoms that responded to C1 esterase inhibitor (C1-INH) infusion. Her family history was of HAE in her mother. As pregnancy progressed, the attacks remainded the same as before pregnacy approximately every 3-5 days, and just few times C1 esterase inhibitor (C1-INH) infusion was given during the pregnancy. Serial growth scans showed appropriate fetal growth but at the last scan was detected polyhydramnion. The patient was admitted at the University Clinic of Obstetrics and Gynecology at 39 weeks of gestation for observation and planning the vaginal delivery. She was delivered by cesarean section under spinal anesthesia at 40+1 gw because of functional dystocia. A predelivery prophylactic dose of 4200 IU of C1-INH (Ruconest) was administered to cover for any HAE exacerbation and additional C1-INH concentrate was also made available in the operating room. A male baby was delivered weighing 3480 g with Apgar score 8/9. After the C-section a dose of 1200 IU of C1-INH (Berinert) was administered. The surgical and postoperative period was uneventful and patient was discharged from the clinic after 4 days.
Conclusion: HAE is a rare but potentially life threatening condition. In these cases a safe obstetric approach would be to administer a predelivery prophylactic infusion of C1-INH concentrate. During labour, endotracheal intubation can trigger severe acute laryngeal edema and every effort should be made for regional anesthesia whenever feasible. During pregnancy women with HAE should be monitored closely by different specialists (perinatologist anesthesiologist, pediatrician) who can work together with the HAE specialist (immunologist/dermatologist), thereby providing a multidisciplinary approach.
...A predelivery prophylactic dose...