X013

An Unusual Presentation of Partial Empty Sella Syndrome and Arnold Chiari Type 1 Malformation

Zeeshan Yasin, Emily Banks, Sagen Zac-Varghese, Samer Alsabbagh, Babak Langroudi, Lister Hospital Stevenage, East and North Hertfordshire NHS Trust

A 25 year old Caucasian female presented with a one month history of headache following head trauma. She described frontal headaches that radiated to her occiput, which worsened on standing and in the early morning. There was associated nausea and vomiting, but no photophobia, fever or neck pain. There was no reported vertigo, limb weakness or numbness. She reported no past history of lumbar puncture or epidural.

On examination: BP 115/89, heart rate; 80. No postural hypotension. BMI 37.

Cranial nerves normal, no cerebellar signs. Normal power and sensations in legs. No signs of meningism. Rest of systemic examination unremarkable.

Bloods revealed normal full blood count, inflammatory marker and renal functions.

CT Brain revealed a partial empty sella and slit shaped ventricles with evidence of intracranial hypertension. Further, MRI Brain showed a partial empty sella and cerebellar herniation of less than 5 mm. MRV Brain showed no venous sinus thrombosis seen.

We checked her pituitary profile and found a very severe cortisol deficiency with 9AM cortisol level of 30. The rest of pituitary profile was normal. The patient was commenced on oral Hydrocortisone replacement. 

This is a challenging case as we were unable to do lumbar puncture to confirm intracranial hypertension due to the presence of cerebellar ectopia.

This is likely intracranial hypertension which may have resulted in partial empty sella syndrome.