Syndrome of an "empty" Turkish saddle
- Symptoms of the Syndrome of an "empty" Turkish saddle
- Reasons of the Syndrome of an "empty" Turkish saddle
- Treatment of the Syndrome of an "empty" Turkish saddle
The phrase "empty Turkish saddle" was included into medical practice in 1951. After anatomic work it was offered by C. Busch which studied autopsiyny material of 788 dead from the diseases which are not connected with hypophysis pathology. In 40 cases (34 women) the combination of almost total absence of a diaphragm of the Turkish saddle with распластываниес a hypophysis in the form of a thin layer of fabric at its bottom was found. At the same time the saddle was empty. Similar pathology was described earlier by other authors, however Bush was the first who connected partially empty Turkish saddle with insufficiency of a diaphragm. Its observations were podpverzhdena and later researches. In literature this phrase designates various nosological forms which general sign is expansion of a subarachnoid space to the intrasellyarny area. The Turkish saddle at the same time is, as a rule, increased.
Symptoms of the Syndrome of an "empty" Turkish saddle:
The syndrome of an empty Turkish saddle often proceeds asymptomatically and accidentally comes to light at radiological inspection. "The empty Turkish saddle" is found preymushchestvenno at women (80%), more often later there are 40 years which were multigiving birth. About 75% of patients have obesity. Clinical signs are various. The headache happens at 70% of patients that is an occasion of an initial X-ray analysis of a skull which in 39% of cases shows the changed Turkish saddle and leads to further more detailed inspection. The headache widely varies on localization and degree - from easy, periodic, to intolerable, almost constant.
Decrease in visual acuity, generalized narrowing of its peripheral fields, bitemporal hemianopsia are possible. The papilledema is observed seldom, however its description in literature meet. The rhinorrhea is the rare complication connected with a rupture of a bottom of the Turkish saddle under the influence of a pulsation of cerebrospinal fluid. The arising communication between a suprasellyarny subarachnoid space and a sphenoidal sine increases risk of development of meningitis. Emergence of a rhinorrhea demands surgical intervention, for example a tamponade of the Turkish saddle a muscle.
Endocrine disturbances at a syndrome of an empty Turkish saddle are shown in change of tropny functions of a hypophysis. Researches with use of sensitive radio immune methods and stimulation tests revealed high percent of disturbance of secretion of hormones (subclinical forms). So, To. Brismer and соавт. found out that at 8 of 13 patients the response of secretion of somatotropic hormone to stimulation is lowered by an insulin hypoglycemia, and at an axis research a hypophysis bark of adrenal glands secretion of cortisol after intravenous administration at 2 of 16 patients of AKTG changed inadequately; reaction on метирапон was normal at all inspected. Unlike этх data of Feglia and соавт. (1973) observed inadequate emission of corticotropin on various incentives (a hypoglycemia, a lysine-vazopresin) at all inspected patients. Studied also TTG GT reserves by means of TRG and RG respectively. Tests showed a number of changes. The nature of these disturbances is not clear yet.
There are works describing hypersecretion of tropny hormones in combination with a syndrome of an empty Turkish saddle more and more. Information on the patient with an acromegalia and the increased Somatotropinum level was the first of them. J.N. Dominique and соавт. reported about PTS at 10% of patients with an acromegalia. Usually these patients have also hypophysis adenoma. Primary syndrome of an empty Turkish saddle develops as a result of a necrosis and an involition of adenomas, and the adenomatous remains continue to gipesekretirovat somatotropic hormone.
Most often at a syndrome of "an empty Turkish saddle" increase in prolactin is noted. It is reported about its growth at 12-17% of patients. As well as in cases with STG-hypersecretion, the giperprolaktinemiya and PTS are often connected with existence of adenomas. The analysis of observations shows that at 73% of patients with PTS and a giperprolaktinemiya on operation adenomas are found. The description of primary "empty Turkish saddle" and at patients with AKTG hypersecretion Imtsya. More often it is cases of a disease of Itsenko-Cushing with hypophysis microadenoma. However it is known of the patient with a disease of Addison at whom long stimulation of kortikotrof because of insufficiency of adrenal glands brought to AKTG-sekretiruyushchey to adenoma and PTS. The description of 2 patients with a syndrome of an empty Turkish saddle and hypersecretion of AKTG at the normal level of cortisol is of interest. Authors make the assumption of products of AKTG-peptide with low biological activity and the subsequent heart attack of giperplazirovanny kortikotrof with formation of PTS. A number of authors give examples of the isolated insufficiency of AKTG and PTS, a combination of an empty Turkish saddle and a carcinoma of adrenal glands.
Thus, disturbances of endocrine function at a syndrome of PTS are extremely various. Meet as hyper, - and hyposecretion of tropny hormones. Disturbances lie in the range from the subclinical forms revealed by means of stimulation tests to the expressed panhypopituitarism. Variability of changes of endocrine function corresponds to the width of etiological factors and a pathogeny of education первичног a syndrome of an empty Turkish saddle.
Reasons of the Syndrome of an "empty" Turkish saddle:
The etiology and pathogeny are up to the end not found out. The empty Turkish saddle developing after beam or surgical treatment is secondary, and arisen without preliminary intervention in areas of a hypophysis - primary. Clinical manifestations of a secondary empty Turkish saddle are caused by a basic disease and complications of the applied therapy. This head is devoted to a problem of primary Turkish saddle. Believe that insufficiency of its diaphragm, i.e. the reinforced protrusion of a firm meninx forming a roof of the Turkish saddle and closing an exit from it is necessary for development of "an empty Turkish saddle". The diaphragm separates a saddle cavity from a subarachnoid space, excluding only an opening, through there passes the hypophysis leg. The diaphragm attachment, its thickness and character of an opening in it are subject to considerable anatomic variations.
The line of its fastening to a back of a saddle and its hillock, perhaps, is lowered, the general surface is evenly thinned, and the opening is expanded because of almost full reduction of the diaphragm which remained in the form of thin (2 mm) of a rim on the periphery. Its insufficiency arising at the same time leads to distribution of a subarachnoid space to intrasellyarny area and to emergence of ability of a likvorny pulsation to influence directly a hypophysis that can lead to reduction of its volume.
All options of inborn pathology of a structure of a diaphragm cause its absolute or relative insufficiency, as is necessary premises of development of a syndrome of an empty Turkish saddle. Other factors only contribute to the following changes: 1) to increase in pressure in a suprasellyarny subarachnoid space which through a defective diaphragm strengthens impact on a hypophysis (at intracranial hypertensia, an idiopathic hypertensia, hydrocephaly, intracranial tumors); 2) to reduction of the sizes of a hypophysis and reduction of volume ratios between it and the Turkish saddle, at disturbance of blood supply and a heart attack of a hypophysis or adenoma (at a diabetes mellitus, head injuries, meningitis, a sinus thrombosis) as a result of physiological involution of a hypophysis (at pregnancy - during this period the volume of a hypophysis can double, and at the multigiving birth women it becomes still the big sizes as after the delivery is not returned to initial volume after approach of a menopause when the hypophysis size уменьшаетя, - similar involution can be observed at patients with primary hypofunction of perefirichesky closed glands at which there is an increase in secretion of tropny hormones and a gipeplaziya of a hypophysis, and the beginning of replacement therapy leads to involution of a hypophysis and development of a syndrome of an empty Turkish saddle; the similar mechanism опиван and after reception of oral contraceptives); 3) to one of rare options of development of an empty Turkish saddle of a syndrome - to a rupture of the vnutrisellyarny tank containing liquid. Thus, the syndrome of an empty Turkish saddle is a polyetiological syndrome which main reason the defective diaphragm of the Turkish saddle is.
Treatment of the Syndrome of an "empty" Turkish saddle:
Special therapy concerning a syndrome of an empty Turkish saddle it is not carried out. Though the combination to PTS does not influence tumor treatment planning, it is important to neurosurgeon to know about his existence as the risk of development of postoperative meningitis increases in these cases.
Prevention of PTS includes the prevention of injuries, inflammatory diseases including pre-natal, and also thromboses and tumors of a brain and hypophysis.
The forecast depends on character and the course of associated diseases of a brain and a hypophysis.