Endocrine Abstracts

JOINT1466

Introduction: Growth deceleration in children may indicate an underlying condition. One such condition is Empty Sella Syndrome (ESS), a radiological finding caused by the herniation of the subarachnoid space into the sella turcica, leading to pituitary gland flattening. While rare in children and often an incidental MRI finding, ESS can be associated with hormonal deficiencies, particularly growth hormone deficiency (GHD), which contributes to growth impairment.

Case Report: A 6-year-old boy was referred to the endocrinology clinic for growth monitoring due to concerns about his growth velocity. Over the past year, his height declined from -1.83 standard deviations (SD) to -2.40 SD, with a growth velocity of 3 cm/year—below the expected rate for his age. His medical history included left cryptorchidism, surgically corrected in infancy, with no family history of endocrine disorders. On physical examination, his height was 105 cm (-2.40 SD, <3rd percentile), below the mid-parental height with a Z-score of -2.22. His weight was 23 kg (BMI = 18.6, 95th percentile). He was Tanner stage I, with no other systemic abnormalities or developmental delays. Laboratory tests revealed low IGF-1 (19.3 ng/ml; normal: 36.6–156) and low IGFBP-3 (1.24 μg/ml; normal: 1.94–5.19), with normal thyroid, ACTH, cortisol, and prolactin levels, ruling out other pituitary axis dysfunctions. Bone age assessment showed a 2-year delay, and karyotype analysis confirmed a normal male karyotype. A clonidine stimulation test demonstrated a poor GH peak of 1.77 ng/ml, confirming growth hormone deficiency. MRI revealed an extension of the subarachnoid space into the pituitary fossa, compressing and flattening the pituitary gland—a characteristic finding of ESS. The patient was diagnosed with growth hormone deficiency secondary to Empty Sella Syndrome and was started on recombinant human growth hormone at a dose of 0.025 mg/kg/day (0.55 mg/day). After three months of treatment, he demonstrated significant improvement, with a height increase of 5.5 cm, reaching 110.5 cm. His Z-score improved by approximately 1 SD (from -2.49 to -1.59), reflecting notable catch-up growth. He will continue to be closely monitored for growth response and other potential pituitary hormone deficiencies.

Conclusions: ESS is rare in children, presenting as either an incidental finding or with hormonal deficiencies. Growth hormone deficiency, the most common pituitary disorder in ESS, affects 12% to 60% of patients. Early diagnosis and timely growth hormone therapy are essential for optimizing growth outcomes in affected children.