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Object Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD). Methods Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height > 2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point. Results Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p < 0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27). Conclusions Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.

Object. The primary empty sella syndrome (ESS) represents a heterogeneous clinical picture characterized by endocrine disturbances and signs of intracranial hypertension. An increase in intracranial pressure (ICP) is proposed to be one of the involved pathogenetic factors. Methods. The series included 142 patients who were observed during a period of 20 years. All patients underwent an ICP and cerebrospinal fluid (CSF) dynamics evaluation through the use of a lumbar constant-rate infusion test. Impairment of ICP and CSF dynamics was observed in 109 patients (76.8%). In 35 of the 36 patients affected by severe intracranial hypertension without rhinorrhea, improvement in adverse neurological symptoms was achieved after implanting a CSF shunt. Visual function, already seriously compromised before surgery, remained severely altered in one patient. In the group of 34 patients affected by rhinorrhea, CSF leakage was controlled using different surgical treatments: CSF shunt placement in 16 cases, surgical repair of the sellar floor in three, and both procedures in the remaining 13. Two patients refused any surgical treatment. Conclusions. The role of increased ICP in the pathogenesis and perpetuation of primary ESS has been confirmed. Adverse neurological signs and a CSF leak are correlated with an actual increase in ICP and are relieved after CSF shunt insertion. Cerebrospinal fluid rhinorrhea is more common than generally thought. Its resolution can be achieved using a careful diagnostic protocol and sometimes may require different surgical procedures.

Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD).Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point.Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27).Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.

Object. Closure of the sella turcica after transsphenoidal surgery is mainly accomplished with autologous muscle fascia and fat or muscle; this requires a second surgical incision. The authors review the results of using resorbable vicryl patches, gelatin foam, and fibrin glue for sellar reconstruction.Methods. A review was conducted of 376 consecutive patients who underwent surgery for pituitary adenomas, cysts, or subdiaphragmatic craniopharyngiomas in the sella turcica that the senior author (R.W.S.) had performed or directly supervised over the last 10 years. The sellar reconstruction was performed with a commercially available, synthetic absorbable patch composed of polyglactin 910/poly-p-dioxanone, gelatin foam, and fibrin glue. The patch is essentially resorbed in 2 to 3 months and replaced by fibrous collagen tissue. There were 117 small, 112 medium-sized, and 147 large lesions. The overall nonendocrine postoperative morbidity rate was 2.8%, and included visual deterioration, meningitis, secondary epistaxis, nasal septum complication, and cerebrospinal fluid (CSF) leakage. Two patients with macroadenomas needed reoperation for persistent CSF leakage, which comprised 0.5% of the whole series or 0.8% of the 259 patients with medium-sized or large lesions. There was no mortality and no morbidity related to the implanted material, and in particular no delayed empty sella syndrome.Conclusions. Closure of the sella turcica with a synthetic absorbable vicryl patch, gelatin foam, and fibrin glue after transsphenoidal surgery is safe and very effective in preventing postoperative CSF fistulas. The use of this technique obviates the need for a second surgical incision and shortens the operating time. Because of the progressive resorption of the substitute material, the interpretation of postoperative magnetic resonance studies was not significantly hindered.

Object. The empty sella syndrome (ESS) is well documented in adults, and although the same phenomenon of herniation of the arachnoid space into the enlarged sella turcica has been noted in children, it is not widely known that children suffer from this syndrome. Therefore, the aims of this paper are to increase neurosurgeons' awareness of the existence of this phenomenon in children and to add to the scant body of literature on the subject.Methods. The authors treated 12 children, ranging in age between 2 and 8 years, in whom neuroradiological studies demonstrated an enlarged sella turcica filled with cerebrospinal fluid and herniation of suprasellar and arachnoid spaces. The causes of ESS in these children were high intracranial pressure, neglected or improperly treated hydrocephalus, and suprasellar arachnoid cyst. Primary ESS was found as well. Most of the children presented with headache, abnormal body weight (the majority being underweight), and short stature. The results of hormone assays were normal in all children.Conclusions. If undiagnosed and untreated, ESS in children may lead to serious consequences, including impairment of pituitary and hypothalamic function and damage to the optic chiasm. It is important to raise awareness in the neurosurgical community about the existence of ESS in children so that it can be diagnosed and treated at an early stage. A classification system for the diaphragma sellae is recapitulated.

Delayed chiasmal syndromes after emptying of a Rathke's cleft cyst have not been reported previously. When these deficits occur following the treatment of parasellar lesions they are usually associated with the descent of a scarred optic system into an empty sella, and vision often improves promptly when the optic system is elevated. Two months after transsphenoidal surgery with emptying of a large intrasellar cyst, a 22-year-old man developed recurrent bitemporal visual field deficits over a 3-day period. Sagittal magnetic resonance images demonstrated an enhancing band of tissue extending anteriorly from the normally placed chiasm down to the anterior portion of the sella turcica. At craniotomy the enhancing tissue was found to be scar extending from the anterior border of the chiasm to the diaphragma sellae. The anterior portion of the diaphragm was resected as widely as possible without dissecting the scar itself from the chiasm. A membrane consistent with the wall of a Rathke's cleft cyst was found attached to the resected tissue. The patient's vision was improved 2 days after surgery. This case illustrates that traction by scar extending from the chiasm to the diaphragm, even when the chiasm is in its normal anatomical location, may cause progressive visual loss; and that untethering of the chiasm by resecting the diaphragm while leaving the scar intact can result in improved vision.

✓ A 61-year-old woman under treatment with oral medroxyprogesterone acetate following surgery for breast cancer developed diplopia and papilledema due to raised intracranial pressure (ICP). Radiological studies disclosed an empty sella turcica, a dural arteriovenous malformation (AVM), and superior sagittal sinus thrombosis. The sinus thrombosis was considered to have been a side effect of the medroxyprogesterone acetate medication. The association between a primary empty sella and raised ICP is briefly discussed, along with the cause-and-effect relationship between a dural AVM and venous sinus thrombosis.

✓ Selected cases are presented demonstrating evaluation of sellar and juxtasellar pathology with metrizamide cisternography. The compatibility of metrizamide with the current generation of computerized tomography scanners has provided complementary data to metrizamide-enhanced hypocycloidal tomographic studies of the sellar and juxtasellar areas. The ease and low morbidity of metrizamide cisternography relative to pneumography is outlined.

✓ An empty sella was demonstrated on air study in five patients with the benign intracranial hypertension (BIH) syndrome. All patients had a protracted course and very high cerebrospinal fluid pressure; two required a shunt procedure. No patient had any endocrine symptoms or visual field defects but an air study was done to exclude a mass lesion in the sella region. Among the last 50 patients seen with the BIH syndrome, there were five cases of an associated empty sella (10%). In these cases, the empty sella is a probable consequence of the long-standing intracranial hypertension associated with a congenital deficiency of the diaphragma sellae.

✓ An enlarged sella turcica was discovered in a 40-year-old man who had bitemporal headaches. A pneumoencephalograph revealed a third ventricular cyst, dilated lateral ventricles, and an empty sella. The colloid cyst was lined by foregut epithelium, probably originating in the respiratory tract, and dense connective tissue. This case is the first instance of an empty sella associated with a colloid cyst of the third ventricle. It is proposed that enlargement of the mass in the third ventricle caused increased pulsation pressure of the cerebrospinal fluid, and that in the presence of an incompetent diaphragma sellae the subarachnoid space expanded into the sella. The origin of third ventricle cysts is reconsidered. It is concluded that suprasellar colloid cysts may arise from endoderm, ectoderm, neuroepithelium, or a combination of these epithelia.

✓ Seventeen cases of coexisting secreting pituitary adenomas and partially empty sellas are presented. The location of the cisternal invagination into the sella was not helpful in predicting the location of the tumor. In patients with endocrine indication of a secreting pituitary neoplasm, the finding of a partially empty sella should not contradict the diagnosis.

✓ A neuroradiological technique for demonstrating the empty sella syndrome using a low dose of intrathecally injected Amipaque (metrizamide) combined with computerized tomography scanning is described. Two case reports illustrating the potential value of the technique are presented. We have performed 55 cisternograms and have demonstrated the empty sella syndrome in six patients. The procedure is easier to perform and the morbidity is less than in pneumoencephalography.

✓ Metrizamide computerized tomographic cisternography was performed with a small dose and low concentration of intrathecal metrizamide using a newer generation of computerized tomography scanner for the evaluation of sellar and suprasellar abnormalities. The examination was performed with thin sections in axial, direct coronal, and (when feasible) direct sagittal projections in a high-resolution technique. The relationship of the lesions with carotid arteries, optic chiasms, and hypothalamic structures was accurately defined.

✓ The association of an empty sella with rhinorrhea is an unusual finding. Of 29 cases of empty sella observed by the authors, eight underwent surgery for rhinorrhea. None of the patients showed clinical evidence of increased intracranial pressure or hydrocephalus. Seven were cases of primary empty sella, the eighth was an acromegalic patient, who had received radiotherapy 4 years before. Except for this patient, the others showed no clinical signs of a previous tumor. In the surgical treatment of these patients, the authors used both the transfrontal and transsphenoidal approaches. In spite of generally accepted good results following surgical closure of this type of fistula, four patients needed more than one operation. The fistula closed in only three of them; in the last patient rhinorrhea persisted after three operations.

✓ The authors report a case of cerebrospinal fluid (CSF) rhinorrhea in a patient with the empty sella syndrome in whom continuous intracranial pressure (ICP) monitoring revealed intermittent peaks of raised ICP. Following a shunt procedure, ICP reverted to normal and the CSF rhinorrhea ceased for 10 weeks. The sella turcica was packed with muscle after the CSF rhinorrhea recurred.

✓ A patient with a pituitary adenoma secreting adrenocorticotropin hormone manifested panhypopituitarism after an episode of pituitary apoplexy. The previously elevated urinary levels of 17-ketogenic steroids dropped sharply, and plasma cortisol became undetectable. The apoplexy also resulted in a partially empty sella on which the dorsum sellae collapsed. Recurrent Cushing's disease developed and was cured by transsphenoidal resection of a microadenoma.

p ITUITARY tumors often encroach upon and compress the nearby optic nerves and chiasm causing a variety of visual deficits. Chamlin, et aL, 1 reported visual field defects in 86%, optic atrophy in 50%, and impaired visual acuity in 32% of these patients with neoplasms of the pituitary. Since these patients may become totally blind if not treated, the primary objective of treatment is to relieve the pressure on the visual pathways by operative decompression, irradiation, separately or in combination. The success of treatment is measured by the degree of improvement in visual function that can be objectively determined and recorded. Deterioration in visual function after treatment is usually assumed to indicate recurrence of tumor. However, on rare occasions, no tumor is found when the enlarged sella is explored surgically. This condition has been called the "empty sella syndrome, ''~ and is a rare complication. We are reporting three cases of this syndrome encountered at the University of California San Francisco Medical Center.

✓ A case of empty sella syndrome due to a benign intrasellar cyst leads the author to a review of variations in this syndrome. Association with intrasellar diverticuli of the subarachnoid space, deficiencies of the diaphragma sellae, and small pituitary glands are identified. Its relationship with certain headaches in women and to unexplained nontraumatic cerebrospinal fluid rhinorrhea are discussed.