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The primary empty sella syndrome

M Farber, R N Turksoy, J Rogers

    Obstetrics and Gynecology
    |January 1, 1977
    PubMed
    Summary

    A rare form of empty sella syndrome in an 18-year-old female presented with primary amenorrhea and hormonal deficiencies. This unique case highlights the complex endocrine and anatomical manifestations of the condition.

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    Area of Science:

    • Endocrinology
    • Neuroscience
    • Radiology

    Background:

    • Primary empty sella syndrome is a condition where the sella turcica is partially or completely filled with cerebrospinal fluid.
    • It is often associated with hormonal imbalances and visual disturbances.

    Observation:

    • A unique case of primary empty sella syndrome was identified in a short, 18-year-old, prepubescent female.
    • Pneumoencephalography revealed air within the sella turcica, an extreme manifestation of the syndrome.

    Findings:

    • The patient presented with primary amenorrhea, indicating a lack of menstruation.
    • Selective deficiencies in gonadotropins and growth hormone were observed.
    • Hormonal levels did not respond to stimulation tests: luteinizing hormone releasing hormone (LHRH) for gonadotropins and insulin-induced hypoglycemia for growth hormone.

    Implications:

    • This case underscores the diverse and extreme presentations of primary empty sella syndrome.
    • It highlights the importance of comprehensive endocrine evaluation in patients with anatomical abnormalities of the sella turcica.
    • Understanding such unique cases contributes to the diagnostic and therapeutic strategies for pituitary and hypothalamic disorders.

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