Empty sella syndrome

Empty sella syndrome

1. EMPTY SELLA SYNDROME DR. SURESH REGISTRAR NINAS

2. Normal dimension of sella Dimension (mm) Min Max Avg Depth 4 12 4.1 Length 5 16 10.6

3. Definition The Empty Sella Syndrome (ESS), resulted from herniation of the subarachnoid space through the diaphragm sella displacing the normal pituitary gland Sella is enlarged and the pituitary gland is compressed and reshaped

4. Misnomer It is not just empty. Intra- sellar arachnoidocele

5. TYPES Primary empty sella syndrome Secondary empty sella syndrome

6. CLASSIFICATION Empty sella is defined as PARTIAL OR TOTAL . Partial: less than 50 % of the sella is filled with CSF Total: More than 50% of sella filled with CSF with the gland thickness being < 2 mm.

7. Primary empty sella syndrome Herniation of the arachnoid membrane into the sella turcica which can act as a mass, probably as a result of repeated CSF pulsation. The sella can become enlarged and the pituitary gland may become compressed against the floor . Occurs in the absence of prior treatment of a pituitary tumor (medical, surgical or XRT).

8. Pathophysiology Inherent weakness of the diaphragm sella and/or Increase in the intracranial pressure

9. Hypothesis A number of hypotheses ; Pituitary infarction, pituitary apoplexy, and rupture of an intrasellar cyst. Transient or constant elevation in intracranial pressure and who has incompetent diaphragma . Recently , shrinkage of the pituitary gland by antipituitary antibodies was advocated as another possible cause of primary ESS

10. CSF pulsations : Remodeling of the bony sellar floor. The bony erosion : communication of the intrasellar subarachnoid space with the sphenoid sinus : CSF rhinorrhoea .

11. Association Frequent association: female sex ( female:male ratio = 5:1), obesity* and HTN

12. Clinical presentation Primary empty sella syndrome is an incidental radiological diagnosis and usually asymptomatic Severity depends on the extent to which the hypothalamus , hypophysis and optic structure are involved

13. Middle-aged obese females Headache Vision CSF rhinorrhea Endocrine: occasionally Amenorrhea- galactorrhea syndrome

14. Endocrine abnormalities Clinically evident endocrine disturbances are rare with primary ESS: 30 % = abnormal pituitary function tests. Isolated GH deficiency is being the commonest Mild elevation of prolactin (PRL) and reduction of ADH : stalk effect These patients show a normal PRL rise with TRH stimulation (whereas patients with prolactinomas do not )

15. INVESTIGATION

16. MRI

17. Treatment Surgical treatment is usually not indicated In this setting, it is necessary to determine if there is increased ICP, and if so, if there is an identifiable cause. Simple shunting for hydrocephalus runs the risk of producing tension pneumocephalus from air drawn in through the former leak site.

18. I ndications for surgery. Visual disturbances CSF rhinorrhea When surgery is indicated, the type of surgery depends on clinical presentation and radiological findings. The surgical outcome of cases with ESS is favorable

19. This may necessitate transsphenoidal repair with simultaneous external lumbar drainage, to be converted to a permanent shunt shortly thereafter. Subfrontal craniotomy with intradural repair of the anterior cranial fossa with fascia . Hyperprolactinemia may be treated e.g. with bromocriptine , if it interferes with gonadal function.

20. SECONDARY EMPTY SELLA SYNDROME

21. Secondary Empty Sella (SES) may be caused by pituitary adenomas undergoing spontaneous necrosis (ischemia or hemorrhage ). Other causes known to cause SES are infective, autoimmune, traumatic, radiotherapy , drugs, and surgery (SLTS). Regression of an inflammatory lesions of a pituitary gland such as lymphocytic and granulomatous hypophysitis

22. Association 1 . F ollowing trauma 2. After successful transsphenoidal removal or XRT for a pituitary tumor 3. A ny cause of increased intracranial pressure, including : Idiopathic intracranial hypertension ( pseudotumor cerebri ), Chiari malformation Often presents with visual deterioration due to herniation of the optic chiasm into the empty sella .

23. There may be hypopituitarism from the underlying cause.

24. Visual deterioration may be treated with chiasmapexy (propping up the chiasm ) usually by transsphenoidal a pproach and packing the sella with fat, muscle or cartilage. May be done endoscopically . Appears to be better for improving visual field deficits than loss of visual acuity.

25. Surgical benefit Patients with preoperative complaint of headache respond well to surgery, with complete resolution in 85.3% of cases (12 cases out of 14). On contrast only 60% of the patients with preoperative visual field defect improved (6 cases out of 10). No patients with preoperative poor visual acuity ( 4 cases) have improved after surgery. Wael Fouad : 2011 Fouad W. 2011

26. Other sellar pathology in comparision SN Pathology Sella enlargement Clinoid Erosion 1 Pituitary adenoma + - 2 Craniopharyngioma +/- + 3 ESS + - 4 Tuberculum meningioma - +