Background: Pituitary apoplexy is uncommon in childhood and adolescence. Typical clinical features are acute confusion, headache, vomiting and visual disturbance. It is caused by haemorrhage into the pituitary gland. Its association with cerebral infarction is rare. We report an unusual case associated with a cerebral infarction secondary to internal carotid artery compression.
Case: 16 year old male was referred to the Stroke Team with acute onset confusion, visual disturbance, slurred speech and right-sided weakness. He was vomiting and complained of worsening headache. Examination revealed right-sided increased tone, reduced power and bi-temporal hemianopia. He was confused and unable to follow commands. Initial CT head revealed 3.5×2 cm sellar/suprasellar mass prompting urgent endocrine profile. This revealed Prolactin 87 089 mIU/l [100410 mIU/l], Cortisol 494 nmol/l [130580 nmol/l], TSH 1.0 mIU/l [0.35.5 mIU/l], T4 5.5 pmol/l [925 pmol/l], IGF-1 19.1 [15.666.9 nmol/l]. Serum Sodium 127 mmol/l and Osmolality 271 mosm/kg suggested inappropriate ADH secretion. The working diagnosis was macroprolactinoma with TSH deficiency. In view of neurological signs and symptoms, he underwent CT angiography. This revealed tumour mass effect causing luminal occlusion of both internal carotid arteries. MRI confirmed pituitary apoplexy with haemorrhagic fluid levels and ischaemic changes in left fronto-parietal region (Middle Cerebral Artery (MCA) distribution). Following treatment with intravenous Hydrocortisone and oral Cabergoline, there was significant improvement in focal neurology signs and vision. He underwent endoscopic trans-sphenoidal debulking of tumour within 48 hours. Post-operative imaging confirmed significantly debulked sellar/suprasellar mass with a maturing left MCA infarct. Histology confirmed pituitary adenoma with strong immunopositivity for prolactin with increased proliferation, Ki-67 index 7%. Ongoing treatment includes replacement Hydrocortisone, Levothyroxine and Growth Hormone (post-op IGF-1 16 [1667 nmol/l]). Cabergoline 500 mcg twice weekly has controlled his prolactin with level now down to 294 mIU/l. Further clinical assessment revealed delayed puberty (G3 PH2 TV5 ml) consistent with longstanding hyperprolactinaemia effect. Post-operative visual fields were normal. Neuro-rehabilitation assessments highlighted significant new cognitive difficulties. He completed a prolonged hospital stay for neuro-rehabilitation and has ongoing support in the community.
Conclusion: Cerebral infarction following pituitary apoplexy and internal carotid artery occlusion is rare. Assessment and knowledge of clinical, radiological and biochemical features of pituitary apoplexy is important in patients presenting with acute neurology.
27 - 29 Nov 2019
British Society for Paediatric Endocrinology and Diabetes