68. Hemorrhagic shock due to ruptured hepatic adenoma secondary to oral norethindrone acetate in a patient with type 3 von Willebrand disease: a case report

Background Patients with von Willebrand's disease (vWD) often require menstrual suppression in order to avoid episodes of heavy bleeding. While multiple hormone-based options are appropriate for these patients, specific side effects and clinical considerations based on the type and severity of vWD need further study. We discuss refinement of treatment options for menorrhagia for an adolescent with type 3 vWD who presented with a complication likely related to chronic progestin exposure. Case Patient C.D. was diagnosed with type 3 vWD in infancy and presented at 11 years-old for management of menorrhagia with a goal of amenorrhea. She was initially started on oral norethindrone acetate alone 3 times daily, which was continued for years to control breakthrough bleeding while receiving injectable progestins followed by the etonorgestrel-releasing implant. At 15 years-old, C.D. presented to the emergency room after 1 day of severe abdominal pain. She was found to be hypotensive with peritoneal signs on exam. CT demonstrated multiple hepatic masses, the largest being 9 cm with active contrast extravasation suggesting a bleeding adenoma with associated hemoperitoneum. She underwent an emergent right hepatic lobe embolization by interventional radiology. She recovered in the pediatric ICU with stabilization of her bleeding. The etiology of her hepatic adenomas was suspected to be chronic progestin use. After department discussions with hematology and gynecology, the oral norethindrone acetate was discontinued during her hospital course and she experienced withdrawal bleeding. On hospital day 13, the patient's contraceptive implant was removed and a levonorgestrel-releasing IUD was placed, which expelled 2 days later in the setting of heavy vaginal bleeding. C.D. received depot-medroxyprogesterone and was discharged home on daily recombinant VWF. At 1-month follow-up, the patient was amenorrheic and had stable hepatic masses on repeat imaging. The patient was maintained on injectable progestins with plans to trial a progestin IUD in the future. Comments Hepatic adenoma is a known complication of estrogen-containing medications and a rare side effect from peripheral conversion of oral progestins. A few case reports have shown regression of hepatic adenomas after cessation of progestin therapy. Furthermore, menses suppression for patients with severe vWD is critical to avoid menorrhagia. Patients with type 3 vWD particularly have limited pharmacologic options for preventing heavy menses. Through this case report, we discuss the management options for menstrual suppression and control of menorrhagia in patients with severe vWD with consideration of potential morbid side effects, and explore areas of further research of treatment regimens. Patients with von Willebrand's disease (vWD) often require menstrual suppression in order to avoid episodes of heavy bleeding. While multiple hormone-based options are appropriate for these patients, specific side effects and clinical considerations based on the type and severity of vWD need further study. We discuss refinement of treatment options for menorrhagia for an adolescent with type 3 vWD who presented with a complication likely related to chronic progestin exposure. Patient C.D. was diagnosed with type 3 vWD in infancy and presented at 11 years-old for management of menorrhagia with a goal of amenorrhea. She was initially started on oral norethindrone acetate alone 3 times daily, which was continued for years to control breakthrough bleeding while receiving injectable progestins followed by the etonorgestrel-releasing implant. At 15 years-old, C.D. presented to the emergency room after 1 day of severe abdominal pain. She was found to be hypotensive with peritoneal signs on exam. CT demonstrated multiple hepatic masses, the largest being 9 cm with active contrast extravasation suggesting a bleeding adenoma with associated hemoperitoneum. She underwent an emergent right hepatic lobe embolization by interventional radiology. She recovered in the pediatric ICU with stabilization of her bleeding. The etiology of her hepatic adenomas was suspected to be chronic progestin use. After department discussions with hematology and gynecology, the oral norethindrone acetate was discontinued during her hospital course and she experienced withdrawal bleeding. On hospital day 13, the patient's contraceptive implant was removed and a levonorgestrel-releasing IUD was placed, which expelled 2 days later in the setting of heavy vaginal bleeding. C.D. received depot-medroxyprogesterone and was discharged home on daily recombinant VWF. At 1-month follow-up, the patient was amenorrheic and had stable hepatic masses on repeat imaging. The patient was maintained on injectable progestins with plans to trial a progestin IUD in the future. Hepatic adenoma is a known complication of estrogen-containing medications and a rare side effect from peripheral conversion of oral progestins. A few case reports have shown regression of hepatic adenomas after cessation of progestin therapy. Furthermore, menses suppression for patients with severe vWD is critical to avoid menorrhagia. Patients with type 3 vWD particularly have limited pharmacologic options for preventing heavy menses. Through this case report, we discuss the management options for menstrual suppression and control of menorrhagia in patients with severe vWD with consideration of potential morbid side effects, and explore areas of further research of treatment regimens.