|Year : 2019 | Volume
| Issue : 1 | Page : 55-59
Empty Sella Syndrome: Management of 34 Cases
Keashav Mohan Jha1, Om Prakash Gupta2, Samrendra Kumar Singh3
1 Professor, Dept. of Neurosurgery, IGIMS, Patna, India
2 Assistant Professor, Dept. of Neurosurgery, IGIMS, Patna, India
3 Associate Professor, Dept. of Neurosurgery, IGIMS, Patna, India
|Date of Web Publication||20-Nov-2020|
Om Prakash Gupta
Dept. of Neuro Surgery, IGIMS, Patna
Source of Support: None, Conflict of Interest: None
Empty sella syndrome (ESS) is an anatomical/ radiological entity, in which the pituitary fossa is enlarged and is filled with Cereberospinal fluid (CSF) owing to arachnoids’ herniation, while pituitary gland is compressed.
This retrospective study was done to evaluate the clinical features, medical and surgical management and outcome in 34 cases with ESS presented to our department for a period of seven years (2011- 2017). ESS is divided into primary and secondary type, depending on the presence or absence of pituitary pathology. 21 Patients presented with primary ESS and 13 had secondary ESS. 4 patients were asymptomatic. 30 patients presented with headache, 15 patients with endocrinological dysfunction, 12 patients with visual manifestation and 8 patients with CSF rhinorrhea. 10 patients (29.4%) underwent surgical treatment and the remaining 24 patients (70.6%) underwent medical management. CSF rhinorrhea, visual disturbances and persistent raised ICP were the main indications of surgery. The type of surgery depended on clinical presentation and radiological finding. The outcomes in the surgical cases were favorable.
Keywords: Empty sella, Treatment of empty sella syndrome, Rhinorrhea, Radiology of sellar region, Endocrinopathy, Intradural and trans sphenoidal repair of anterior cranial base.
|How to cite this article:|
Jha KM, Gupta OP, Singh SK. Empty Sella Syndrome: Management of 34 Cases. J Indira Gandhi Inst Med Sci 2019;5:55-9
| Introduction:|| |
Empty sella syndrome (ESS) is an anatomical/ radiological entity, in which pituitary fossa is enlarged and is filled with cereberospinal fluid (CSF) owing to arachnoids’ herniation, while pituitary gland is compressed against posterior and inferior rim of the sella,. It is an incidental finding and occasionally results in an abnormal pituitary function. The entity is usually associated with structural changes of sella that can be depicted by studies such as Magnetic Resonance Imaging (MRI) and/or computed tomography.
Empty sella syndrome was first described by Sheehan and Summers in 1949. Busch in 1951 first applied the term “empty sella” to describe this anatomical variation in an autopsy study. It is reported in 5% of normal subjects,.
It is more common in women and increases in frequency with age,. Patients with ESS presents with a wide spectrum of clinical findings with neurological, visual and endocrine abnormalities even in the absence of pituitary disease. Some patients can present with CSF rhinorrhea. However, many patients with ESS are asymptomatic.
ESS is classified into primary ESS and secondary ESS depending on the presence or absence of pituitary pathology. In Primary ESS, subarachnoid space herniates into sella in the absence of primary pituitary pathology. Secondary ESS patients may be associated with spontaneous necrosis or hemorrhage of pituitary adenoma, infection, trauma, auto immune disease or previous pituitary surgery, radiotherapy or dopamine antagonist,,. Both groups of patients may present with spontaneous rhinorrhea. Surgical therapy is rarely required, except for cases presented with CSF rhinorrhea, progressive visual loss and raised ICP not controlled with medical treatment,,.
The aim of this study was to evaluate different diagnostic criteria of primary and secondary ESS and evaluate results of surgical management of this condition. We will compare our results to other contemporary surgical series.
| Methods:|| |
This retrospective study was carried out at the Department of Neurosurgery at Indira Gandhi Institute of Medical Sciences, Patna over a period of seven years (2011- 2017) after clearance from our institutional ethics committee. The study included 34 Patients who underwent treatment of ESS at our Department. Patients (61.76 %) had Primary ESS and 13 patients presented with secondary ESS (38.24%). Detailed history and clinical examination was done to rule out any endocrinological abnormality. All patients underwent detailed ophthalmological examination including visual acuity, visual field and fundus examination. All patients underwent routine lateral view of skull, CT scan of Brain and MRI. CT scan with intrathecal contrast was done in 8 patients who presented with CSF rhinorrhea. All patients underwent following endocrinological diagnostic procedure: FT3, FT4, TSH, PRL, FSH, LH and Cortisol level. Patients were divided into two groups based on history, clinical and radiological examination into Primary and secondary ESS.
Different surgical procedures were done, based on mode of presentations and radiological findings. 10 patents (29.4%) underwent surgical treatment and another 24 patients (70.6%) underwent medical management. All patients have been reviewed in the outpatient Department. The last clinical follow up and MRI examination were done in a period ranging from 6 months to 60 months with Mean follow up period was 34 months.
| Results:|| |
Patients were divided into two groups based on history, clinical and radiological examination into Primary and secondary ESS. In this study, 21 cases were in the Primary ESS group and 13 in the secondary ESS group. In both groups majority of patients were female. In primary ESS group there were 17 females (80.9%) and in secondary ESS group 9 females (69.2%). Two patients among primary ESS and two from secondary ESS were asymptomatic. Headache was commonest symptom in the both groups, 90.5% in primary ESS and 84.6% in secondary ESS.
In Primary ESS group, 4 patients were diagnosed incidentally. 6 patients presented with visual manifestations. Field defect was present in 4 patients. Four patients had diminished visual acuity and another two had bilateral papilloedema with enlarged blind spot. Two patients could count fingers at six feet and another two patients had visual acuity less than 6/60. Visual field defect was present in the form of constricted field in 2 patients, binasal hemianopia in one case and multiple scotomas in one case. 7 patients had CSF rhinorrhea. 6 patients had endocrinological abnormality. Hypopituitrism was present in two patients, Hyperprolactinemia in two patients, diminished growth hormone in one patient and another one had diabetes insipidus.
Out of 13 cases of secondary ESS, six patients had previous pituitary surgery and another two had surgery for supra sellar and sellar meningioma. One patient had severe head injury with its sequel and another patient had CVA involving anterior cerebral artery territory. Rest four patients had hypopituitrism associated with post partum hemorrhage. 11 patients presented with headache and features of raised ICP. In this group CSF rhinorrhea was present in only one patient. Six patients had diminished visual acuity, two patients had visual field defect and another two patients had secondary optic atrophy. Visual acuity was restricted to counting fingers at few feet in four cases and two cases had visual acuity less than 6/60. Visual field defect was present in the form of constricted field defect in one case and binasal field defect in another patient. Six patients had no visual symptoms Endocrinological dysfunction was present in 8 patients. All four patients with history of post partum hemorrhage had pan hypopituitrism. Three males presented with features of hypothyroidism. Diminished cortisol level along with hypothyroidism was found in one operated case of prolactinoma and another patient with ACA territory infarction (CVA).
Plain X-ray showed enlarged sella turcica in 18 cases. CT scan with intrathecal contrast showed trans sellar fistulous tract in 4 cases, and CSF leak through ciribriform plate in another two cases. In two cases the exact site of leak could not identified. MRI showed posterior displacement of pituitary gland in 16 cases (47.1%). Pituitary gland was seen at the floor of the sella in three cases (8.8%). In rest of 15 cases (44.1%), pituitary tissue could not be identified.
Four cases of CSF rhinorrhea with primary ESS were initially treated with subfrontal craniotomy and intradural repair of anterior cranial fossa. As this procedure failed in two case, both underwent thecoperitoneal shunt with successful results. Six patients with CSF rhinorrhea, including one with secondary ESS, underwent trans sphenoidal packing of the sella using fat and muscle and sellar floor reconstruction with successful outcome. One patient with features of raised ICP and visual symptoms improved with Thecoperitoneal shunt. Another patient with large arachnoidal cyst and hydrocephalus and ESS underwent initially endoscopic fenestration and third ventriculostomy. This patient improved initially, but the cyst recurred with symptom. This patient improved with bilateral shunt surgery. Two patients with progressive visual symptoms underwent transphenoidal packing of the sella using fat and muscle and sellar floor reconstruction. Visual acuity and visual field of one patient improved. Another patient did not show improvement, however, visual acuity was stabilized. Postoperative MRI studies after trans sphenoidal extradural packing revealed that the sellar contents remained satisfactorily elevated both on sagittal and coronal planes with upward lifting of suprasellar structures.
Patients with preoperative complaint of headache responded well to surgery/ treatment with complete resolution in 93.3%. In contrast, only 66.6 % of patients with visual field defect improved (4 out of 6). In this series, only three patients out of ten with preoperative diminished visual acuity improved. This included one case, where the primary indication for surgery was deteriorating visual acuity.
No patient in this series developed new deficits or had progression of an existing preoperative deficit. There was no postoperative mortality, and surgery did not provoke any permanent neurological aggravation of any of the cases. T
| Discussion:|| |
The term empty sella is a misnomer, as sella is not completely empty. Pituitary is always present both anatomically and functionally. It may occasionally result in an abnormal pituitary function. The entity is usually associated with structural changes of sella that can be depicted by radiological studies. It is often discovered incidentally, when imaging was done for some unrelated conditions,,,. In our study, ESS was found when CT scan/ MRI was done for head injury, headache, CVA, work up for infertility. In one case it was detected when metastatic work up was done for Ca prostate.
It can occur in either sex and at any age. However, it is more common in women and increases in frequency with age (8). It has been reported in children (14, 15, 16). Hypertension, obesity and migraine can be associated with this condition,.
A number of hypotheses have been offered to explain the cause of ESS. Entities such as pituitary infarction, pituitary apoplexy, and rupture of intrasellar cyst may play some role in development of ESS. The condition arises in a patient who has had transient or chronic elevation in the intracranial pressure and when there is also an incompetent diphragma sellae. Recently, shrinkage of Pituitary glands by antipituitary antibodies was advanced as another possible cause of Primary ESS.
Secondary ESS develops secondary to Pituitary pathology. This may be associated with an iatrogenic event such as surgery, radiation or both. A non iatrogenic event such as infarction and infection of pituitary is also associated with secondary ESS,. In our study, we have one case with anterior cerebral artery territory infarction and other with severe head injury.
Post surgical empty sella is related to removal of pituitary tumor either through transcranial or trans sphenoidal route and leading to incompetent diaphragma sella at the end of procedure. This, subsequently, leads to herniation of third ventricle and optic apparatus to empty sella,.
CSF rhinorrhea is striking but fortunately uncommon clinical complication that may develop in patients with ESS. This may be etiologically related to benign intracranial hypertension, which is frequently associated with ESS. Site of CSF leak is usually through eroded sellar floor into sphenoid sinus, but it can happen through cirbriform plate. The site can be distinguished by CT scan after intrathecal contrast injection,,,. In this study 8 cases presented with CSF rhinorrhea. Site of CSF leak was through the sellar floor in 4 cases, through ciribriform plate in two cases and in two cases, sites could not be identified.
In our study, 19 patients with primary ESS (90.5%) presented with headache. Six patients had visual manifestations including decreased visual acuity (19.1%) and visual field loss (19.1%) and bilateral papilloedema with enlarged blind spot (9.5%). Similar Visual field and fundus abnormalities were reported from literature.,, In our study six patients (28.6%) with primary ESS had Endocrine abnormalities. Hypopituitrism was present in two patients. Hyperprolactinemia in two patients, diminished growth hormone in one patient and last one presented with diabetes insipidus. Endocrinopathy in primary ESS had been reported in literature from 30- 50%,. Children with empty sella most commonly have GH deficiency, although other pituitary dysfunctions may occur.
In our study, Secondary empty sella syndrome occurred in 8 cases due to surgical cause. Six patients underwent pituitary surgery and another two suprasellar meningioma surgery. Four patients had hypopituitrism as result of severe post partum hemorrhage (Sheehan syndrome). Other two patients presented as squeal of severe head injury and ACA territory infarction. 11 patients (84.6%) presented with headache and features of raised ICP. CSF rhinorrhea was present in only one patient.
In this group, visual abnormality was pre dominant clinical finding occurring due to arachnoidal adhesions and traction on optic apparatus. In this group seven patients had visual symptoms, while another six had no visual symptoms. Six patients had diminished visual acuity, two patients had visual field defect and another two patients had secondary optic atrophy. Visual acuity was restricted to counting fingers at few feet in four cases and two cases had visual acuity less than 6/60. Visual field defect was present in the form of constricted field defect in one case and binasal field defect in another patient.
Endocrinological dysfunction was present in 8 patients. All four patients with history of post partum hemorrhage had pan hypopituitrism. Three males presented with features of hypothyroidism. Diminished cortisol level along with hypothyroidism was found in one operated case of prolactinoma and another patient with ACA territory infarction (CVA).
In pre CT scan era lateral radiograph was most common investigation to diagnose sellar or suprasellar lesion. The confirmation of empty sella in pre CT era was done by pneumoencehalography or Metrizamide cisternography,,.
C T Scan of ESS will show that sellar fossa is occupied by fluid of CSF density instead of normal Pituitary gland,,.
MRI is currently definitive diagnostic and imaging of choice in ESS. Multiplanar MRI can demonstrate compressed pituitary gland inside sella. Typical central position of infundibulum is a useful sign in ESS and its presence rules out a Cystic lesion in supra sellar region,. MRI can readily depict herniation of optic nerve, optic chiasm, and anterior third ventricle in to sella,. Increased intra cranial pressure and traction over optic chiasm caused by post surgical adhesions can lead to visual field defect in ESS.
| Treatment of ESS:|| |
The uncomplicated Empty sella requires no treatment. When it is accompanied by endocrine dysfunction replacement therapy of the appropriate target gland hormone may be required,.
The operative management is indicated for the CSF rhinorrhea, the presence of progressive visual symptoms due to intra sellar visual pathway herniation and associated features of raised intracranial pressure,,. When spontaneous CSF leak is present, surgical correction of fistula is indicated. The trans sphenoidal approach is usually selected. Six patients with CSF rhinorrhea, including one with secondary ESS, underwent trans sphenoidal packing of the sella using fat and muscle and sellar floor reconstruction with successful outcome. In our initial period, four cases of CSF rhinorrhea with primary ESS were initially treated with subfrontal craniotomy with intradural repair of anterior cranial fossa. This procedure failed in two cases, both underwent thecoperitoneal shunt with successful results. We have found that simple thecoperitoneal shunt or conventional shunt is useful in selected cases.
Trans sphenoidal Chiasmapexy is indicated in the rare instances, when the chiasma herniates inside the sella and herniation causes progressive visual abnormalities. The procedure consists of inserting the amount of fat sufficient to push the optic structure into a natural suprasellar position. The rational of this procedure is to decrease the stretching of visual pathway and their vascular supply. We have done this procedure in two cases, One of these patient showed improvement in visual acuity and another showed stability of visual acuity.
New techniques of chiasmapexy, which involves placement of inflatable balloon or silastic coil in the extradural space in the inferior intrasellar region had been tried and had had proved successful,]. We don’t have facilities or the experience for these techniques.
| Summary:|| |
Empty sella syndrome is (ESS) an important diagnosis to consider in patient presenting with non characteristic neurological, endocrine or visual impairment with or without CSF rhinorrhea. In this complex and evolving syndrome raised ICP, CSF dynamics alterations together with and incompetent sellar diaphragm plays a clear pathogenetic role.
A large proportion of spontaneous CSF rhinorrhea is caused by ESS. Radiographic findings suggestive of empty sella including enlarged sella turcica, down ward herniation of optic chiasm and/or compression of pituitary gland with traction of infundibulum should prompt clinician to pursue a full pituitary hormone analysis and visual acuity and field testing. Endocrine abnormalities are amenable to medical treatment. CSF rhinorrhea and worsening visual problems may indicate surgical intervention. Clinician must be aware of the presenting symptoms of ESS, as many symptoms are readily reversible if appropriate treatment is started promptly.
|Figure 1: Sagittal T1 weighted MR image of sellar region demonstrates a CSF filled cistern which had herniated into sella turcica with posterior displacement and flattening of Pituitary gland.|
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|Figure 2: Coronal MRI in 30 year old lady who developed Secondry ESS following Seehan Syndrome. Pituitary gland is flattened against sellar floor and stretched pituitary stalk.|
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|Figure 3: Axial MRI demonstrating the intrasellar filling of CSF with position of Pituitary stalk seen as white dot.|
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[Figure 1], [Figure 2], [Figure 3]