Empty Turkish saddle syndrome: causes, symptoms, diagnosis, treatment

Quite often, the empty sella turcica syndrome is asymptomatic. In the presence of symptoms, the clinical picture is extremely diverse. The main manifestation of the empty sella turcica syndrome (ESS) is hypothalamic-pituitary dysfunction of varying degrees. Headaches in the forehead, cerebrospinal fluid leakage from the nose when coughing and sneezing, and changes in the visual fields are possible. Neurometabolic-endocrine syndromes are very widely represented: cerebral obesity with oligo- or amenorrhea, persistent galactorrhea-amenorrhea syndrome, myxedema, false pseudohypoparathyroidism, acromegaly, diabetes insipidus, panhypopituitarism, partial hypopituitarism, subclinical disorders of the secretion level of tropic hormones. Clinical symptoms are extremely dynamic, with one neuroendocrine syndrome changing to another, and spontaneous remissions. Emotional-personal, motivational and vegetative disorders are quite significant, but no specific features are observed.

In 80% of cases, skull radiographs show an increase in the size of the sella turcica, thinning of its back, and the shape of the sella turcica is most often cylindrical. Hydrocephalic skull shape and hypertensive phenomena are often encountered. However, empty sella turcica syndrome can occur against the background of a normal radiographic picture.

In the clinical picture, symptoms of benign intracranial hypertension - "pseudotumor of the brain" - may come to the forefront, which are characterized by headaches of a hypertensive nature, edema of the optic discs, increased intracranial pressure with a normal composition of the cerebrospinal fluid. Diagnosis is possible after pneumoencephalography or targeted computed tomography. It is more often observed in women who have had many pregnancies, after long-term use of oral contraceptives, against the background of hypertension, after hormone replacement therapy.

Causes of the empty sella syndrome. There are primary and secondary empty sella syndromes. Among the primary syndrome etiologic factors, the most important are congenital insufficiency of the sella turcica diaphragm, transient hyperfunction and hyperplasia of the pituitary gland with subsequent involution (pregnancy, long-term use of oral contraceptives, hormone replacement therapy), increased cerebrospinal fluid pressure (Pickwickian syndrome, "pseudotumors of the brain", arterial hypertension). Congenital underdevelopment of the diaphragm can be aggravated by transient hyperfunction of the pituitary gland and its stalk, and fluctuations in cerebrospinal fluid pressure lead to the introduction of the arachnoid membrane with fluid into the sella cavity.

The causes of secondary empty sella syndrome are disruption of the sella diaphragm during neurosurgical intervention and the formation of free space in the sella turcica cavity as a result of removal of a space-occupying lesion.

Pathogenesis of the "empty" sella turcica syndrome. With a congenitally defective sella diaphragm and increased intracranial pressure, a bulging of the arachnoid membrane filled with cerebrospinal fluid into the sella turcica occurs as a result of various causes. The implanted "cerebrospinal fluid sac" pushes the pituitary gland to the wall of the sella turcica, disrupting the functions of the adenohypophysis mainly. In addition, there is compression of the pituitary stalk with disruption of hypothalamic control over the pituitary functions. The latter cause is considered the most significant for the formation of neuroendocrine syndromes. The picture of neuroendocrine disorders depends on the quality of the constitutional inferiority of the cerebral systems of neuroendocrine regulation.

Differential diagnosis. First of all, it is necessary to exclude a volumetric process in the sella turcica (tumor, cyst), hemorrhage into the tumor.

Treatment of the "empty" sella turcica syndrome. Surgical intervention with plastic surgery of the sella turcica diaphragm is used only when visual disorders increase. Pharmacotherapy consists of using agents aimed at reducing the pressure of the cerebrospinal fluid, improving the hemodynamics of the brain, and normalizing arterial pressure. For this purpose, vascular, dehydrating, and hypotensive agents are used. Other therapeutic measures depend on the quality of neuroendocrine disorders.