diagnosis and management of empty sella syndrome

1. Dr. Shahnawaz Alam
Guided by: Dr. Vikas Chandra Jha
HOD, Dept. of Neurosurgery
Moderated by: Dr. Saraj kumar Singh
Faculty, Dept. of Neurosurgery
EMPTY SELLA SYNDROME

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

3. Definition
• An empty sella is defined as a
sella, which, regardless of its size,
is completely or partially filled
with CSF.
• The pituitary gland is compressed
and lies posteriorly and inferiorly
in the sella.
• Empty sella syndrome can be
primary or secondary.
• PES syndrome- absence of pituitary surgery or irradiation for pituitary disease and
SES is that which occurs following these procedures.
• ESS resulted from herniation of the subarachnoid space through the diaphragm
sella displacing the normal pituitary gland.
• First coined by Bush in 1951.

4. * Guinto et al. 2019; www.journals.elsevier.com/world-neurosurgery

5. PATHOGENESIS
• An incomplete sellar diaphragm is an essential pre-requisite.
• Inherent weakness of the diaphragm sella and/or Increase in the intracranial
pressure.
1. Congenital Factors: Deficiency of the diaphragma sellae.
2. Suprasellar Factors
3. Intrasellar Factors
• Physiological involution
• Pathological involution: Pituitary apoplexy/ Rupture of an intrasellar or parasellar cyst
4. Systemic Factors

7. CLASSIFICATION
 Empty sella is defined as partial or total.
• Partial: less than 50% of the sella is filled with CSF with the gland thickness being >
2 mm.
• Total: More than 50% of sella filled with CSF with the gland thickness being < 2
mm.

8. Primary empty sella syndrome
• Range from the occasional discovery of a clinically asymptomatic arachnoid pouch
within the sella turcica to severe intracranial hypertension and rhinorrhoea.
• PES may be seen anatomically and radiologically without producing any symptoms or
signs.
• 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).

9. Association
• Female sex ( female:male ratio = 5:1)
• Obesity and HTN
• 8-35 % in general population
• 30-40 years of age
• Present in 70-80 % patients with IIH

10. Clinical presentation
• Severity depends on the extent to which the hypothalamus, hypophysis and optic
structure are involved.
• Systemic, neurological and endocrine Symptoms.
• About 30% hypertensive.
• Headache, which occurs in 50−70%- MC neurological symptoms.
• Visual disturbances up to 34%.
• Memory disturbances, imbalance, dizziness, convulsions and CSF rhinorrhoea.

11. Endocrine abnormalities
 Endocrine symptoms reported in 10−15% of patients.
 30% = abnormal pituitary function tests.
 Isolated GH deficiency is being the commonest; reduced response of GH to
insulin induced hypoglycaemia.
 Mild elevation of prolactin (PRL) and reductionof ADH : stalk effect
 These patients show a normal PRL rise with TRH stimulation (whereas
patients with prolactinomas do not)
 Hyperprolactinaemia is not as high as in a prolactin secreting adenoma, unless
the partially empty sella is associated with a prolactinoma. The prolactin level
is usually below 100−125 ng/ml.

15. Secondary Empty Sella Syndrome
• Occurs after surgical and/or radiation therapy of a pituitary tumour.
• May be caused by pituitary adenomas undergoing spontaneous necrosis (ischemia or
hemorrhage).
• Other causes: infective/ autoimmune/ traumatic/ drugs.
• Regression of an inflammatory lesions of a pituitary gland such as lymphocytic and
granulomatous hypophysitis.
• The predominant clinical finding in these patients is visual abnormality, occurring
due to arachnoidal adhesions and traction on the optic apparatus.
• The Endocrine symptoms due to hypersecretion or hyposecretion are more common
in secondary empty sella than in the primary variety.
• This is mainly due to the tumour itself and not due to the empty sella. CSF
rhinorrhoea can occur due to shrinkage of the tumour.

16. MRI sagittal of a patient with secondary empty sella showing
evidence of transsphenoidal surgery with empty sella
MRI of a patient having a pineal
tumour (white arrow) and empty sella

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

18. Diagnosis and Management
*Chiloiro et al. 2017; European Journal of Endocrinology

20. Treatment
• Empty sella syndrome is usually a benign condition. The majority of cases do not require
treatment, except symptomatic for headache.
• Hypertension must be treated and weight reduction advised in obese patients.
• Hyperprolactinemia may be treated e.g. with bromocriptine, if it interferes with gonadal
function.
• Indications for surgery:
 Visual disturbances
 CSF rhinorrhoea
• When surgery is indicated, the type of surgery depends on clinical presentation and
radiologicalfindings.

21. • CSF rhinorrhoea requires prompt surgical treatment, as the fistula rarely closes
spontaneously.
• When associated with hydrocephalus or BIH, an initial CSF diversion procedure, like
VP- shunt or lumboperitoneal shunt should be done and, if the leak persists, definitive
surgery must be undertaken.
• Simple shunting for hydrocephalus runs the risk of producing tension pneumocephalus
from air drawn in through the former leak site.
• When the fistula is from the sella into the sphenoid, the transsphenoidal route is ideal.
The sellar dura should be opened and the sella packed with fat covered over by fascia.

22.  Different surgical procedures according to the mode of presentation and the radiological
findings:
• Extradural transsphenoidal packing of the sella using fat and fascia with fibrin glue.
(transsellar fistulous tract after intrathecal contrast)
• Subfrontal craniotomy with intradural repair of the anterior cranial fossa with fascia.
• Thecoperitoneal/ VP- shunt.
• Reconstruction of the sellar floor using either a fragment of the bone from the anterior
sphenoid wall or the bony septum.
• In cases with previous transsphenoidal surgery (secondary empty sella), the reconstruction
of the sellar floor using a bone graft from iliac crest or acrylic material.

23. * Guinto et al. 2019; www.journals.elsevier.com/world-neurosurgery

24. • When visual symptoms are present in SES the cause should clearly be identified.
• On MR, if there is a clear demonstration of descent of the optic apparatus into the sella and
there is kinking of the optic nerves or chiasm, chiasmapexy is indicated.
• Chiasmapexy (propping up the chiasm) usually by transsphenoidal approach and packing
the sella with fat, muscle or cartilage.

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).
• 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

26. • Patients diagnosed to have an empty sella should be followed up regularly and any
progression of symptoms should appropriately be treated.
• Sudden and rapid deterioration, especially in vision, may occur and the patient should
be warned of this.
• Patients with no abnormalities at baseline are unlikely to develop neurological/
ophthalmological symptoms or endocrine abnormalities in the follow-up.
• Moreover, the radiological degree of PES also tends to remain constant over time.
• However, because of the theoretical risk of progression, a re-evaluation after 24−36
months (if there are no clinical indications before) of the endocrine/ neurological/
ophthalmological/ radiological picture is reasonable.
Follow-up

27. References:
• Youmans and Winn neurological surgery 7th edition
• Ramamurthi & Tandon's textbook of neurosurgery 3rdediton edition
THANK YOU