ESPE Abstracts

Introduction: Empty Sella (ES) is a radiological finding which is due to herniation of subarachnoid space into sella turcica. It’s partial, when <50% space is filled with cerebrospinal fluid (CSF) and complete, when CSF fills >50% with pituitary gland flattened to <=2mm. Actual occurrence is debatable and often noted incidentally during magnetic resonance imaging (MRI). However, it can cause pituitary hormone (PH) dysfunctions, most commonly growth hormone (GH) deficiency. We are reporting a case of partially empty sella with GH and cortisol deficiencies.

Case: A 15year old boy presented with delayed puberty and absence of secondary sexual characteristics (G1P1TV 2mL & 3mL). He had short stature with height (153.2 cm) below parental target, on the 2nd centile (target 25th to 98th) and weight (58.05kg) on 50th centile. Mother felt he had low stamina during sports and took longer to recover from illnesses. However, he did not have morning tiredness. His birth and past medical history were insignificant. Parents were nonconsanguineous and both were late developers. Mum needed oestrogen for pubertal induction. His baseline tests were normal including thyroid function, coeliac, full blood count, IGF-1. He had 2.66 years bone age delay and normal male karyotype. Hence, he had a primed insulin tolerance test (ITT). It detected a poor GH peak of 1.7mcg/l and cortisol peak of 293nmol/l (acceptable minimum 6.7mcg/l & 430nmol/l respectively). ACTH was within range (16.3ng/l, 0-46ng/l). After diagnosing GH and secondary cortisol insufficiency, he was started on hydrocortisone (HC) replacement. His T2 weighted MRI pituitary revealed partially empty sella with asymmetrical pituitary (pituitary gland measuring 4mm cranio-caudally on right and 2mm on left). He responded well to hormone replacement. Eventually he progressed to puberty on his own (G2P2TV 5mL & 6mL).

Discussion and learning points: ES is a rare entity in the paediatric population and probably less reported. Primary ES can be idiopathic, developmental, due to ICP etc. Although it is five times more common in adult females, in childhood more reported cases are males. Secondary ES is commonly due to injury to the gland from surgery, tumour, or trauma. Presentation varies from incidental radiological findings to symptoms of hormone deficiencies. PH deficiency can be in up to 60% cases of ES with GH being most affected. Treatment consists of hormone replacement, close monitoring of evolving deficiencies and treatment of the secondary causes if present.