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Laboring Patient with Hereditary Angioedema and Recent Lumbar Surgery
Abstract Number: S-32
Abstract Type: Case Report/Case Series
We present a case of a patient with Hereditary Angioedema in labor with h/o previous C-Section and recent lumbar spine surgery requesting VBAC.
32 years female,G2P1, at 42 weeks with a history significant for HAE, G6PD deficiency, anxiety and prior C-Section presented in early labor for desired VBAC. Patient was diagnosed with HAE type 1 at age of 12 with symptoms of bowel angioedema. She had a strong family history of HAE type 1(her two sisters are affected and one of them required multiple intubations during her pregnancy). For the last 20 years, the patient had flare ups 1-2 times a year, mainly abdominal pain from bowel angioedema. She was hospitalized 3 times for laryngeal edema, was never intubated and treated with IV C1 concentrate. Patient underwent back surgery for lumbar disc problems. Patient claimed that the neurosurgeon forbade regional/neuraxial anesthesia in the future.
Upon consultation with Anesthesia at 37 weeks, MRI of lumbar spine were requested. Neurosurgical record was not available. MRI of lumbar spine revealed mild degenerative disc disease at L4-L5 level w/o spinal or foraminal stenosis, and 12 mm benign hemangioma(incidental) at left posterior L3 vertebral body. Patient agreed for neuraxial anesthesia, once the risks versus benefits were explained to her.
On admission, the patient was hysterically crying in pain and very anxious. A CSE was successfully placed at L5-S1 level uneventfully. IV fluids were minimized to KVO and patient was allowed to have clear liquids. First stage of labor lasted 5 hours and overall was uneventful. There was a short period of flat tracing of the baby’s heart rate and a decision was made to proceed with C-Section. In the interim, patient became fully dilated and she delivered a healthy girl. C1 esterase inhibitor 500 units was given IV prophylactically as per the recommendation from the immunologist. Postpartum period was uneventful.
Acute therapy for HAE includes C1 esterase inhibitor (human), kallikrein inhibitors, and bradykinin inhibitors. The patient in this case had C1 esterase inhibitor, which is dosed at 20 units/kg. Prophylactic regimens include 17-alpha-alkylated androgens such as danazol, oxandrolone and stanozolol, all of which are thought to increase endogenous C1 esterase inhibitor levels in the serum via hepatic synthesis.
Based on our case experience, we advocate for:
1. Early multidisciplinary team involvement(Pharmacy, OB, Anesthesia, Blood Bank, Allergy-Immunology)
2. Have C1 Esterase inhibitor readily available and in-service on it's administration.
3. Early placement of epidural anesthesia to provide effective analgesia.
4. Restrictive fluid management to minimize interstitial edema
5. Early airway management if symptoms of swelling evident. Stand by jet ventilation and tracheostomy kit available.