Pituitary Apoplexy and Fulminant Liver Failure

Pituitary apoplexy is a rare endocrine emergency due to acute infarction or hemorrhage of the pituitary gland. It most often involves an underlying adenoma. Precipitating factors include hypertension, major surgery, coagulopathy, or medications. Fulminant liver disease giving rise to a coagulopathy can lead to an increased risk of hemorrhage into the pituitary. A 20-year-old female was transferred from British Overseas Territory for evaluation of fulminant liver failure after presenting with worsening of longstanding abdominal pain and vomiting. She was taking ciprofloxacin and metronidazole for colitis and Tylenol for pain. She denied alcohol abuse, illicit drugs or herbal supplements. Her blood pressure was 107/72 mmHg and physical exam was notable for jaundice. Labs were significant for Alanine Aminotransferase (ALT) 10277 IU/L (n<35 IU/L), Aspartate aminotransferase (AST) 10886 IU/L (n<40 IU/L), total bilirubin 15 mg/dl (n<1.2 mg/dl), alkaline phosphatase 160 IU/L (n<115 IU/L) and INR 4.9 (n<1.3). She was given 100 mg of methylprednisolone. CT head without contrast (WO) completed prior to transfer showed no abnormal findings. The next day, she reported new onset severe headache and diplopia. Bilateral wrist asterixis and worsening lethargy was noted on exam. Ammonia was 278 umol/L (<n 42 umol/L). CT head WO showed a 0.6 x 0.9 x 1.0 cm hyperdense area in the posterior aspect of pituitary gland concerning for apoplexy. Additional labs showed cortisol 11 ug/dL (n<22 ug/dL), ACTH 2.9 pg/mL (n 7.2-63.3 pg/mL), TSH 0.04 uIU/mL (n 0.3-4.5 uIU/mL), free (F) T4 1.4 ng/dl (n 0.6-1.5 ng/dl), FT3 1.2 ng/dl (n 2.4-4.2 ng/dl), FSH 1.8 mIU/ml (n 3.5-12.5 mIU/ml), LH <0.1 mIU/mL (n 2.4-12.6 mIU/ml), prolactin 49 ng/mL (n 5-23 ng/mL), and IGF-1 141 ng/mL (n 109-372 ng/mL). She was managed conservatively with hydrocortisone for secondary adrenal insufficiency and hypopituitarism. With extensive work up, no underlying cause was found for her fulminant hepatic disease. She underwent a deceased donor liver transplant. Perioperatively, she was given 200 mg methylprednisolone which was subsequently tapered to 5 mg prednisone. Postoperative course was complicated by a new right parietal lobe hemorrhage. There was no change in pituitary hemorrhage size. As her FT4 trended down to 0.6 ng/dl, along with suppressed TSH, she was started on 50 mcg of levothyroxine. Upon discharge, she was advised to follow with an endocrinologist and have her hormones revaluated once the acute phase has resolved. Pituitary apoplexy as a consequence of coagulopathy caused by liver failure has not been described before. Although she was given methylprednisolone which can suppress ACTH for up to 48 hours, ACTH deficiency is the most critical endocrine dysfunction and is present in nearly 70% of cases of pituitary apoplexy. Therefore, urgent treatment of secondary adrenal insufficiency is warranted along with thyroid hormone replacement.