EMPTY SELLA SYNDROME III. Medical management/ Surgical management

Aquino, Felix SP
Sarmiento, Noel

III. Medical management/ Surgical management

Primary as well as secondary empty sella syndrome is usually benign conditions not requiring any treatment. According to Melmed(2008), there is no specific treatment if pituitary function is normal. Unless the disorder results in other medical problems, treatment is symptomatic and supportive. In some cases, surgery may be needed. (Pituitary Network Association, 2009).However, surgery is not indicated for the great majority of patients with an empty sella syndrome. The initial complaints of some of these patients are related to hypertension or obesity and require only medical treatment (Answer.com, 2010).

Treatment of symptomatic empty sella syndrome would typically involve replacement therapy for any deficient hormones. However, the endocrinopathy associated with the primary empty sella syndrome is usually slight and rarely requires replacement therapy. For instance, hypothyroidism would require treatment with synthetic thyroid hormone, hypoadrenalism could be treated with steroids (cortisol), and hypogonadism might require sex hormone replacement therapy. (Agarwal et al, 2001) Medications, such as bromocriptine, which lower prolactin levels, may be prescribed if the prolactin levels are high and interfering with function of the ovaries or testes. It is also effective in correcting the problem.(Health Scout, 2009). According to Elias (2005), such a conservative approach is supported by a study which followed 12 women (mean age, 43 years) with a primary empty sella syndrome. In none of them did the disease progress, either clinically or radiographically .Treatment of endocrinological dysfunction can be especially difficult because of the complicated way in which the many hormones of the body interact with and affect each other. In addition, all treatments for empty sella syndrome would be symptomatic treatments; there is no method to restore the pituitary gland to its normal size.

According to Elias (2005), patient with a visual field deficit caused by the empty sella syndrome was ophthalmologically stable as well. However, overt diabetes mellitus developed in 15 percent of the cohort, and hypertension developed in twice that number. Together these diseases caused noncompressive, ischemic visual deficits in 2 of the 12 patients (15 percent). Furthermore, Elias (2005) cited that study of have shown that as many as seven-eighths of empty sella syndrome patients suffer from coexisting glaucoma, which is readily treatable. Clearly, conservative treatment requires close medical and ophthalmologic follow-up.

Cases associated with benign intracranial hypertension (pseudotumorcerebri) are usually self-limited and best treated by nonsurgical methods.(Elias, 2005). An occasional patient may require lumboperitoneal CSF shunting to control papilledema or debilitating headache. Optic nerve fenestration has largely replaced subtemporal decompression for relief of optic nerve pressure. Patients with communicating hydrocephalus and elevated CSF pressure should undergo shunting without delay. When hydrocephalus is secondary to a tumor or Chiari malformation, the underlying pathologic process is usually treated first. (Elias, 2005)

CSF rhinorrhea via an empty sella requires surgical intervention since spontaneous obliteration is unlikely. Like other nontraumatic CSF leakage, this type of rhinorrhea seldom stops spontaneously. The first step in diagnosis is to determine whether a high-pressure or normal-pressure mechanism underlies the rhinorrhea. When the pressure is elevated by a tumor, diagnosis and surgical therapy is primarily directed at tumor diagnosis and removal. (Elias, 2005)However, it is not enough to correct the cause; the leak must be repaired as well. When the leakage is due to hydrocephalus, shunting procedures may lead to the development of tension pneumocephalus. After appropriate preoperative localization of the fistulous tract (fluorescein) endoscopic or transphenoidal closure is the treatment of choice (Balaganapathy,2009).Transphenoidal repair using fat and fascia, followed by a shunting procedure, may serve to avoid this problem. Occasionally, transcranial exploration is necessary if the site of leakage cannot be identified on preoperative studies.

When the intracranial pressure is normal, several treatment options are available. Continuous external lumbar subarachnoid drainage of CSF has been successfully employed to treat both postoperative and posttraumatic CSF fistulae. Continuous drainage is not without risk, and overdrainage may result in pneumocephalus. Several methods have been proposed to minimize this problem, including flow-regulated drainage(Elias, 2005)

In Empty Sella Syndrome, visual disturbance is caused by tractions of the chiasms or the optic nerves toward the sellar floor by the adhesion scar or pituitary stalk, for which chiasmapexy is usually indicated. Chiasmapexy is preferably performed via the transsphenoidal rather than the transcranial approach. During chiasmapexy, traction on the optic system is relieved dy elevating the sellar floor extradurally withy various materials, the most common being autologous tissues, including fat, muscle, fascia, bone and cartilage. Fat and muscle maybe absorbed over the long term after surgery. Synthetic materials have also been used, such as silicone balloons and silicone tubing(Kubo, 2005)

Visual field disturbances usually are mild and should therefore only be followed carefully. Surgery should be reserved for cases with progressive deterioration and radiological evidence of traction or angulation of the optic nerves and chiasm (Balaganapathy, 2009). The primary empty sella is generally asymptomatic and incidentally detected, it requires no specific treatment. However, when it is accompanied by endocrine dysfunction, replacement therapy of the appropriate target gland hormones would be required. Presence of CSF rhinorrhoea may require surgical correction.This is especially true for the secondary empty sella syndrome. In secondary empty sella, adhesions are formed between the diaphragmasellae and the chiasma. Retraction of these adhesions due to reduction in tumour size may pull the chiasma into the empty sella resulting in visual field defects. (Agarwal et al, 2001)This needs to be corrected by chiasmopexy. Chiasmapexy has been advocated by a few, but better results have been obtained using an extradural approach early after the onset of symptoms. By placing fat and fascia between the sellar floor and the sellardura, one avoids injuring the intradural structures, which may include the downwardly displaced median eminence as well as the often enlarged intercavernous veins

Several methods have been proposed to achieve transsphenoidal arachnoid mobilization and chiasmapexy in symptomatic empty sella. These procedures are often difficult to perform and have not always had satisfactory long-term outcomes because of the difficulty of achieving adequate and long-lasting sellar filling over time. The volume of fat or muscle packing decreases over time as a result of scar retraction. The same problem may occur with intrasellar balloon placement because of deflation of the balloon. This technique was used in four patients with satisfactory and long-lasting clinical results. It presents several advantages over previous methods, it can be tailored to each patient. Silastic is an inert substance, and therefore scarring, with consequent shrinkage, does not occur and because the coil is very elastic, it presents few risks of inflammatory complications or of excessive compression of sellar, parasellar, and suprasellar structures. Furthermore, this technique does not require a supplementary skin incision to harvest autologous tissues. A skilled neurosurgeon can perform the procedure in a few minutes with more ease and less expense than other techniques. (Gianluigi, 2002)

Alternatively, Elias (2005) cited that a lamina terminalisotomy which in theory vents the CSF pulsations and, in at least one case, resulted in successful treatment of a bitemporalhemianopsia. Lysis of adhesions and scar tissue surrounding chiasmal structures is to be avoided because little improvement can be expected and worsening has been reported.

The transsphenoidal approach is traditionally used in the surgical treatment of intrasellar or intra-suprasellar infradiaphragmatic lesions for which there is no intentional disruption of the integrity of the subarachnoid space.In such cases, sellar floor reconstruction is needed mainly when intraoperative CSF leaks occur. Different techniques of sellar repair using various materials have been successfully applied, either microscopically or endoscopically. (Cavallo, 2007)



References:

Agarwal J. et al..(2001). Empty Sella Syndrome.Journal, Indian Academy of Clinical Medicine Vol. 2, No. 3

Answer.com.(2010).Empty sella syndrome. Retrieved on February 16, 2010 at http://www.answers.com/topic/empty-sella-syndrome

Balaganapathy, M. (2009) Empty sella syndrome. Retrieved on February 14, 2010 at http://www.thamburaj.com/empty_sella.htm

Cavallo, L. (2007). Skull base reconstruction in the extended endoscopic
transsphenoidal approach for suprasellar lesions. Retrieved on February 17, 2010 at http://thejns.org/doi/abs/10.3171/JNS-07/10/0713

Elias, M.(2005).Empty sella syndrome. Retrieved on February 14, 2010 at http://pituitaryadenomas.com/emptysella.htm

Gianluigi, Z. (2002), Transsphenoidal Treatment of Empty Sella by Means of a Silastic Coil: Technical Note, Neurosurgery Volume 51 - Issue 5 - pp 1299-1303, Retrieved on February 17, 2010 at http://journals.lww.com/neurosurgery /Abstract/2002/11000/ Transsphenoidal_Treatment_of_Empty_Sella_by_Means.32.aspx

Health Scout, (2009). Empty Sella Syndrome. Retrieved on February 17, 2010 at http://www.healthscout.com/ency/1/000349.html

Kubo, S. et al (2005).“Endonasal Endoscopic Transsphenoidal Chiasmapexy With Silicone Plates for Empty Sella Syndrome”, Neurologia medico-chirurgica, Vol. 45, pp.428-432.

Melmed S,(2008), Empty Sella Syndrome. Williams Textbook of Endocrinology. Philadelphia, PA: Saunders Elsevier, Retrieved on February 17, 2010 at http://health.nytimes.com/health/guides/disease/empty-sella-syndrome/overview.html

Pituitary Network Association.(2009).Pituitary disorders. Retrieved on February 16, 2010 athttp://www.pituitary.org/disorders/empty_sella_syndrome.aspx