Background: Pituitary apoplexy is a clinical syndrome caused by haemorrhage of the pituitary gland, typically characterised by acute confusion, headache, vomiting and visual disturbance. It is regarded as a medical emergency. It is rare in childhood and adolescence, occurring in association with pituitary tumours. We report an unusual case of pituitary apoplexy associated with a cerebral infarction secondary to internal carotid artery compression.
Case: 16 year old male presented to Emergency Department with acute onset confusion, visual disturbance, slurred speech and right-sided weakness. There was a three day history of vomiting, and two days of worsening headache. He was unable to follow commands (GCS 11:E4V2M5). Examination revealed right-sided increased tone, reduced power and bi-temporal hemianopia.
The patient was initially managed according to "stroke" guidelines. CT head revealed 3.5x2cm sellar/suprasellar mass. Urgent endocrine profile revealed Prolactin (x20 dilution) 87089mIU/L [100-410mIU/L], Cortisol 494nmol/L [130-580nmol/L], TSH 1.0mIU/L [0.3-5.5mIU/L], T4 5.5pmol/L [9-25pmol/L]. Serum Sodium 127mmol/L and Osmolality 271mosmo/kg supported inappropriate ADH secretion. These results were consistent with diagnosis of macroprolactinoma with TSH deficiency.
CT angiography within 24 hours showed luminal occlusion of both internal carotid arteries secondary to tumour mass effect. MRI confirmed pituitary apoplexy with haemorrhagic fluid levels. Ischaemic changes were also seen in left fronto-parietal, middle cerebral artery (MCA) region.
Treatment was commenced with 100mg IV Hydrocortisone STAT on admission and subsequently 250micrograms oral Cabergoline. Within 24 hours there was improvement in focal neurology and vision. Endoscopic trans-sphenoidal debulking of tumour was performed within 48 hours. Post-operative imaging confirmed significantly debulked sellar/suprasellar mass with a maturing left MCA infarct. Histology showed pituitary adenoma with strong immunopositivity for prolactin with increased proliferation, Ki-67 index 7%.
Maintenance Hydrocortisone, Levothyroxine and Cabergoline 250 micrograms twice weekly were commenced. Repeat Prolactin day 5 was 3491mIU/L. Cabergoline was increased to 500micrograms twice weekly and within 2 weeks Prolactin reduced to 645mIU/L. Further clinical assessment revealed delayed puberty (G3 PH2 TV5ml) consistent with longstanding hyperprolactinaemia effect. Post-operative visual fields were normal.
Neuro-rehabilitation assessments revealed significant cognitive difficulties. Prior to this event the patient was functioning at a good cognitive level according to school reports. Ongoing neuro-rehabilitation is integral to his ongoing care.
Outcome: Clinical surveillance to review evolving pituitary hormone replacement, definitive tumour management and neurological outcome are in progress.
Conclusion: Cerebral infarction following pituitary apoplexy and internal carotid artery occlusion is rare. Cranial MRI scan (to confirm pituitary apoplexy) and CT angiography helped understand the pathophysiology.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology