Background: Spinal Epidural Lipomatosis (SEL) is an excessive fat deposition in the spinal canal which can lead to compression of nervous structures. SEL is a rare but recognised side effect of exogenous steroid excess. There are only six previously reported cases of SEL associated with endogenous steroid excess in the available literature. We present a case of SEL, caused by Cushings disease, that presented in the immediate post-operative period.
Case: A 17-year old man presented with increasing stretch marks for 1 year, two stone weight gain within 6 months, lower limb weakness, poor mobility, pedal oedema and loss in height. He had a history of bronchial asthma, which was well controlled with beclomethasone inhaler. On examination, he displayed typical Cushingnoid features such as facial plethora, widespread striae covering torso and limbs and proximal myopathy. He was obese and hypertensive. Investigation showed that he had hypokalemia, low testosterone and low gonadotrophins levels. His 24 hour urinary free cortisol was 4411 nmol/l, serum cortisol (overnight dexamethasone suppression test) 696 nmol/l and ACTH 146 ng/l, confirming he had ACTH dependent Cushings disease. Initial MRI failed to reveal pituitary adenoma. He was then commenced on metyrapone and dexamethasone block and replacement therapy. Subsequent SPGR MRI confirmed 5 mm pituitary microadenoma. IPSS also confirmed that pituitary was the source of excess ACTH secretion. On day 1 post-transphenoidal surgery, he became paraplegic with increased tone, power 2/5 and reduced sensation from T2 level. MRI spine showed increased fat deposition in spinal canal from T2 to T9. A conservative approach for spinal epidural lipomatosis (SEL) was adopted. He was transferred to a rehabilitation facility and has gradually regained his mobility. This case illustrates a rare but serious complication of endogenous steroid excess and an additional cause of leg weakness in Cushings patients.
18 - 21 May 2019
European Society of Endocrinology