Mechanism of development (pathogenesis)
Normally, the diaphragma sellae separates the pituitary gland from the subarachnoid space of the brain. In empty sella syndrome, this barrier is disrupted.
The pathogenetic chain is as follows:
- Presence of a congenital or acquired defect of the diaphragma sellae.
- Free penetration and constant pressure of cerebrospinal fluid (CSF) on the pituitary tissues.
- Progressive deformation of the organ.
- Impaired transport of liberins (releasing hormones) from the hypothalamus.
- Gradual atrophy and replacement of glandular tissue with connective tissue (sclerosis).
On radiographs, this appears as the pituitary gland visually failing to fill the entire sella turcica, forming an "empty" space filled with CSF.
Primary and secondary forms
The syndrome can develop as an independent rare anomaly or be a consequence of other severe pathologies of the hypothalamo-pituitary system. Secondary empty sella syndrome can develop against the background of the following conditions:
- Sheehan's syndrome — acute postpartum infarction or necrosis of part of the anterior pituitary gland, caused by impaired blood supply due to severe obstetric hemorrhage.
- Pituitary cachexia (Simmonds' syndrome) — a condition developing with necrosis of at least 90% of adenohypophyseal cells (due to sepsis, traumatic brain injuries, tumor removal).
- Pituitary adenoma infarction — tissue necrosis of an existing tumor followed by their replacement with dense connective tissue.
- Radiation therapy of the pituitary region, leading to organ destruction.
Clinical manifestations and role in endocrinopathies
The clinical presentation of empty sella syndrome largely overlaps with the symptoms of tropic hormone deficiency. As the pituitary tissue undergoes sclerosis, hypopituitarism develops.
This pathology is one of the rare causes of generalized hypogonadism. Compression of the pituitary gland and impaired transport of neurotransmitters and gonadotropin-releasing hormone lead to disruption of the "hypothalamus-pituitary-ovary" axis. As a result, women develop secondary ovarian insufficiency.
Empty sella syndrome is a non-neoplastic form of pituitary amenorrhea. Unlike neoplastic forms (such as prolactinomas or ACTH- and GH-secreting adenomas causing Cushing's disease or acromegaly), symptoms of hormone deficiency rather than hormone hypersecretion come to the forefront here.
Differential diagnosis of causes of hypopituitarism
In addition to empty sella syndrome, adenohypophyseal hypofunction and similar symptoms can occur due to other pathologies causing tissue necrosis or compression:
- Non-functioning chromophobe adenomas (oncocytomas): small cells form rosettes, and symptoms are associated with mechanical compression of surrounding brain structures.
- Suprasellar hypothalamic tumors: for example, craniopharyngiomas (tumors originating from Rathke's pouch). In adults, they also cause hypogonadism (amenorrhea, infertility, gonadal atrophy), "white Addison's disease" (adrenal insufficiency without hyperpigmentation), and hypothyroidism.
- Chronic wasting diseases (tuberculosis, chronic myeloid leukemia) and psychogenic factors (recurrent stress), which also inhibit the "hypothalamus-pituitary-ovary" system.
To remember the essence of the syndrome: "CSF pushed through the membrane — the pituitary flattened along the floor."
Class and exam questions
What is visible on an X-ray in empty sella syndrome?
Radiological imaging reveals that the pituitary tissue is deformed and does not occupy the entire volume of the sella turcica, unlike in a healthy person. The remaining space is filled with cerebrospinal fluid.
What reproductive system disorders does this syndrome lead to?
The pathology causes impaired liberin transport and decreased gonadotropin production. This leads to dysfunction of the hypothalamic-pituitary-ovarian axis, secondary ovarian insufficiency, hypogonadism, and non-oncological amenorrhea.
What diseases can trigger the secondary development of the syndrome?
The secondary form often develops after a pituitary adenoma infarction, radiation therapy of the organ, as well as against the background of postpartum pituitary necrosis (Sheehan's syndrome) or Simmonds' syndrome.
What else to study on this topic
- Pathogenesis and autoimmune mechanisms of Sheehan's syndrome (postpartum pituitary necrosis)
- Pituitary cachexia (Simmonds' syndrome) and manifestations of panhypopituitarism
- Suprasellar hypothalamic tumors: craniopharyngioma and pituitary dwarfism
- Neoplastic forms of amenorrhea: hyperprolactinemia, acromegaly, and Cushing's disease
- Impact of chronic wasting diseases (malabsorption syndrome, osteomyelitis) on hypogonadism
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