Extradural Remodeling in Empty Sella Syndrome: 3-Dimensional Operative Video.

Sellar arachnoidocele is a term used to define the herniation of the subarachnoid space to the sella.1 This is a rare radiologic finding that, in most cases, does not require treatment.2-5 When symptoms appear, the term empty sella syndrome is used. Two varieties exist: primary and secondary empty sella syndrome.2 The aim of this 3-dimensional operative video (Video 1) is to demonstrate the extradural microsurgical remodeling of the sellar fossa with autologous bone in 2 cases of primary empty sella syndrome. Both patients signed an informed consent for the procedures and agree with the use of their images for research purposes. In both cases, magnetic resonance imaging scans showed herniation of the subarachnoid space into the pituitary fossa and an anchor-like silhouette on coronal view. Patients evolved favorably, improving their visual deficit after the surgery, as can be observed in the postoperative visual field study. If surgery is indicated due to visual loss, the procedure is known as chiasmapexy. Recently, Guinto et al3 described a technique for chiasmapexy. Our team considers this procedure to be useful, technically simple, and low cost. Being autologous, rejection possibilities are almost null. This 3D video serves as a complement to illustrate the technique.

Does pituitary compression/empty sella syndrome contribute to MRI-negative Cushing's disease? A single-institution experience.

OBJECTIVE: Cushing's disease arises from functioning adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing's disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing's disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing's disease. METHODS: A single-institution database of Cushing's disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing's disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI. RESULTS: Of the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61-6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002). CONCLUSIONS: Empty sella syndrome is associated with an increased rate of MRI-negative Cushing's disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.

Empty Sella in the Making.

BACKGROUND: Pituitary apoplexy may occur when a large tumor compresses or outgrows its nutrient supply, resulting in ischemic necrosis and hemorrhage. Although once deemed a neurosurgical emergency, increasing evidence suggests that conservative management of pituitary apoplexy leads to favorable neuro-ophthalmologic and endocrinologic outcomes as well. Spontaneous remission after pituitary apoplexy has been described in functioning pituitary adenomas, but it is a rare occurrence in nonfunctioning tumors. CASE DESCRIPTION: We report a man that presented with pituitary apoplexy of a nonfunctioning pituitary macroadenoma that was managed conservatively and treated hormonally for hypopituitarism during a 2-year follow-up period, with serial neuroimaging demonstrating significant tumor volume reduction with almost complete resolution resulting in partial empty sella. In addition, a short literature review was performed pertaining to the management of pituitary apoplexy with emphasis on a more conservative approach. CONCLUSIONS: A subset of patients with pituitary apoplexy without altered consciousness and nonprogressive or mild ophthalmologic deficits may be managed conservatively; however, lifelong periodic assessment, preferably by a specialized multidisciplinary pituitary team, is essential until clinical outcomes become clear.

Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome.

BACKGROUND: Empty sella is an anatomic finding that is usually asymptomatic. However, when patients with empty sella finding present with visual deficits, surgical treatment may be necessary. The main goal of surgery is to elevate sellar content through a transsphenoidal approach. The aim of this study was to demonstrate a new technique for precise reconstruction of the sellar floor using a heterologous bone block to restore the anatomic elements of the sella turcica. METHODS: Three patients with primary empty sella who presented with visual field defects were prospectively included. Surgery was performed through a microsurgical transsphenoidal approach and involved elevating the sellar content by using a tricortical heterologous bone graft, the dimensions of which were obtained considering the exact dimensions of each patient's sella turcica. The graft was premodeled outside the surgical bed and carefully introduced into the sella turcica to achieve the required elevation (chiasmapexy). RESULTS: Vision of all 3 patients improved, and the improvement persisted throughout the follow-up period. 2 patients, this improvement was noted immediately after surgery, and in the third patient, this change in vision occurred 1 week later. Long-term imaging studies showed the persistence of the bone graft in the sella turcica and the exact elevation of the sellar content at the end of the follow-up period. There were no complications in the present series. CONCLUSIONS: The technique presented here is simple and reproducible and allows an almost exact and persistent elevation of the sellar content.

Endonasal Chiasmapexy Using Autologous Cartilage/Bone for Empty Sella Syndrome After Cabergoline Therapy for Prolactinoma.

BACKGROUND: Visual field deterioration caused by secondary empty sella after cabergoline therapy for prolactinoma is a rare event. Chiasmapexy is performed to treat empty sella syndrome. Although various materials have been used for the elevation of the optic chasm, the most appropriate material remains to be established. Here, we describe the efficiency of chiasmapexy for empty sella syndrome following dopamine agonist treatment and the utility of septal cartilage and sphenoidal sinus bone as materials for chiasmapexy. CASE DESCRIPTION: A 35-year-old male with a history of cabergoline therapy for prolactinoma presented with visual deterioration. His magnetic resonance imaging revealed optic chiasm herniation into the empty sella. Endoscopic endonasal transsphenoidal chiasmapexy was performed using septal cartilage and sphenoidal sinus bone as materials for elevating the chiasm. Visual function improved immediately after operation. CONCLUSIONS: Chiasmapexy is an effective surgical method for treating visual deterioration caused by empty sella after cabergoline treatment. Endoscopic endonasal chiasmapexy with septal cartilage and sphenoidal sinus bone is a considerable option because it is minimally invasive and involves decreased risk of infection.

Evaluation and treatment of pulsatile tinnitus associated with sigmoid sinus wall anomalies.

OBJECTIVE: Describe clinical and radiographic features of sigmoid sinus wall anomalies (SSWA) associated with pulsatile tinnitus (PT) and determine factors predictive of response to surgery. METHODS: Preoperative diagnostic imaging and treatment response were reviewed after surgical repair of 40 ears among 38 consecutive patients presenting with PT associated with SSWA who underwent transtemporal sinus wall reconstruction. RESULTS: Twenty-three ears had isolated sigmoid sinus dehiscence, and 17 had diverticulum. The rates of transverse sinus stenosis (TSS) and empty sella, 66% and 32% respectively, were significantly higher than in historical controls (P = 0.02 and 0.001). Thirty-six out of 40 subjects (90%) had complete resolution of their PT following surgery, including all those with a diverticulum. For subjects with dehiscence alone without diverticulum, a favorable response to surgery was strongly associated with the presence of TSS (P = 0.01) and empty sella (P = 0.02). CONCLUSION: Sigmoid sinus diverticulum and dehiscence are a clinically important cause of PT. Women of childbearing age with an elevated body mass index (BMI) are commonly affected, and there is a high rate of associated TSS and empty sella. Transtemporal sinus wall reconstruction has a high rate of success in appropriately selected patients. Patients with isolated sinus wall dehiscence without diverticulum, TSS, or empty sella are less likely to respond to transtemporal sinus wall reconstruction. These data imply a multifactorial cause of PT in at least some patients with SSWA. LEVEL OF EVIDENCE: 4 Laryngoscope, 128:S1-S13, 2018.

Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques.

PURPOSE: Chiasmapexy is a poorly described surgical procedure adopted to correct the downward displacement of suprasellar visual system (SVS) into an empty sella (ES) causing visual worsening. The aim of our study is to define the indications for extradural and intradural chiasmapexy. METHODS: A systematic literature review has been performed on MEDLINE database (US National Library of Medicine), including only articles that depicted cases of surgically treated patients affected by ES and progressive delayed visual worsening. Moreover, we have reported three cases of secondary ES syndrome (SESS) with visual worsening treated in our Department with transsphenoidal (TS) microsurgical intradural approach. Finally, we have compared the results of extradural and intradural chiasmapexy described in literature. RESULTS: The etiology of visual impairment is different in primary and secondary ESS. In primary ESS (PESS) the only predisposing factor is a dehiscence of diaphragma sellae, and the anatomical distortion caused by displacement of optic chiasm or traction of pituitary stalk and infundibulum may determine a direct injury of neural fibers and ischemic damage of SVS. In PESS the mechanical elevation of SVS performed through extradural approach is sufficient to resolve the main pathologic mechanism. In SESS, arachnoidal adhesions play an important role in addition to downward herniation of SVS. Consequently, the surgical technique should provide elevation of SVS combined to intradural release of scar tissue and arachnoidal adhesions. In treatment of SESS, the intradural approaches result to be more effective, guaranteeing the best visual outcomes with the lowest complications rates. CONCLUSIONS: The intradural chiasmapexy is indicated in treatment of SESS, instead the extradural approaches are suggested for surgical management of PESS.

New Technique for Chiasmapexy Using Iliac Crest Bone Graft: 2 Cases of Visual Impairment Caused by Empty Sella Syndrome.

BACKGROUND: Chiasmapexy is used to treat empty sella syndrome, and various materials are used for the elevation of the optic chiasm. However, the use of artificial substances may have the risk of graft infection, and fat and muscle may be absorbed over the long term after surgery. In addition, bone and cartilage may be unavailable in adequate amounts. Here, we describe a new technique for chiasmapexy using an iliac crest bone graft. CASE DESCRIPTION: The first patient was a 71-year-old woman who had undergone transsphenoidal surgery twice for the treatment of pituitary adenoma and Rathke cleft cyst. The optic chiasm collapsed after the second surgery and her visual field worsened gradually. We performed chiasmapexy using fat, fascia, and a septal mucosal flap, but the optic chiasm did not remain in the normal position because of graft shrinkage. Finally, we used an iliac crest bone graft, which resulted in good visual function. The second patient was a 58-year-old man who was incidentally diagnosed with empty sella syndrome. The patient's bitemporal hemianopia gradually progressed. As in the first case, we used an iliac crest bone graft, which halted the deterioration of visual function after chiasmapexy. CONCLUSIONS: The advantages of iliac bone are that it is less likely to absorb and become infected than synthetic materials. This method may be suitable for reoperative cases, especially those wherein the septal cartilage has been removed in a previous surgery. This method will halt visual deterioration and may be one of the considerable options for chiasmapexy operations.

Surgical Outcome of Growth Hormone-Secreting Pituitary Adenoma with Empty Sella Using a New Classification.

OBJECTIVE: To investigate outcomes and identified risk factors affecting cure and intraoperative cerebrospinal fluid leak after transsphenoidal surgery using a new classification for growth hormone-secreting pituitary adenoma associated with empty sella. METHODS: In this retrospective cohort study, 51 patients enrolled from January 2010 to June 2016 were categorized into 3 groups using a new classification scheme: grade A, whole tumor occupation area beneath a horizontal line drawn along the lowest level of empty sella; grade B, tumor occupation area that crossed the horizontal line on 1 side; and grade C, tumor occupation area that crossed the horizontal line on 2 sides. Clinical data were collected and analyzed. RESULTS: Cure rate for grade A patients (88.24%; 15/17) was significantly higher than cure rate for grade B (55.00%; 11/20) and grade C (50.00%; 7/14) patients. Occurrence of cerebrospinal fluid leaks in grade C patients (35.71%; 5/14) was higher than in grade A patients (5.88%; 1/17). Logistic regression analysis indicated that risk factors affecting cure included large maximum tumor diameter (P = 0.009, odds ratio [OR] = 1.222), high preoperative fasting growth hormone level (P = 0.031, OR = 1.088), and high classification (P = 0.017, OR = 4.485). Risk factor affecting intraoperative cerebrospinal fluid leak was high classification (P = 0.039, OR = 3.580). CONCLUSIONS: Transsphenoidal surgery is the current optimal treatment strategy. Empty sella increases the difficulty of surgery with a higher incidence of complications. The new classification scheme was better for predicting the surgical outcome for this disease.

Clinical characteristics of acromegalic patients with empty sella and their outcomes following transsphenoidal surgery.

PURPOSE: To analyze the clinical characteristics of acromegalic patients with empty sella (ES, herniation of the subarachnoid space within the sella turcica) and the impact of ES on transsphenoidal surgery in such patients. METHODS: Seventy-eight patients, newly diagnosed with acromegaly who underwent transsphenoidal surgery were included. ES was defined as the pituitary gland and adenoma occupying less than 50% of the sella turcica on midsagittal magnetic resonance (MR) imaging. RESULTS: Twelve patients (15.4%), predominantly female (10 women, p = .047), had ES in preoperative MR imaging. ES patients had smaller mean tumor diameter (6.3 mm) than non-ES patients (11.2 mm, p = .001). In preoperative MR imaging, occult adenoma was found in three (25%) ES and three (4.5%) non-ES patients (p = .044). Intraoperative cerebrospinal fluid (CSF) leakage was more frequent in the ES patients than in the non-ES patients (58.3 vs. 25.8%, p = .024). This led to an increased rate of sellar floor reconstruction using abdominal fat and/or postoperative lumber drainage in the ES patients (ES: 41.7 vs. non-ES: 16.7%, p = .063). Endocrinological remission after surgery was more frequent in the non-ES patients (72.7%) than in the ES patients (58.3%) (p = .248). CONCLUSIONS: Co-existence of acromegaly with ES is not rare, and is associated with occult adenoma, intra/postoperative CSF leakage, and a worse endocrinological outcome after transsphenoidal surgery; although, the underlying mechanism remains unclear.

Endonasal endoscopic transsphenoidal chiasmapexy using a clival cranial base cranioplasty for visual loss from massive empty sella following macroprolactinoma treatment with bromocriptine: case report.

Visual deterioration after dopamine-agonist treatment of prolactinomas associated with empty sella syndrome and secondary optic apparatus traction is a rare event. Chiasmapexy has been described as a viable treatment option, although few cases exist in the literature. Here, a novel endonasal endoscopic approach to chiasmapexy is described and its efficacy is demonstrated in a case report. A 55-year-old female patient with a history of a giant prolactinoma and 14 years of treatment using dopaminergic agonist therapy presented to our institution with a 1-month history of visual changes. Neuroophthalmological examination confirmed severe bitemporal field defects, and MRI revealed a large empty sella with downward optic chiasmal herniation. Endoscopic endonasal chiasmapexy was performed by elevating the chiasm with lumbar drainage and filling the clival and sellar defect with an extradural liquid (HydroSet; a cranioplasty bone cement), and a piece of AlloDerm was used to cover and cushion the chiasm. Postoperative imaging demonstrated successful anatomical elevation of the optic apparatus, and the patient showed functional improvement in the visual field at 3 months postoperatively. Although rare, massive empty sellar and chiasmal descent from macroadenoma treatment can result in progressive visual loss. Here, a novel technique of endonasal endoscopic extradural cranioplasty aided by lumbar drainage is reported, which appears to be an effective technique for stabilizing and possibly reversing anatomical and visual deterioration.

Growth hormone secreting pituitary microadenomas and empty sella - An under-recognized association?

OBJECTIVE: To describe an association of growth hormone (GH) secreting pituitary microadenomas and empty sella (ES), which has been described in case reports - the underlying mechanisms are unclear. METHODS: We retrospectively analyzed patients operated for GH-producing pituitary adenomas between February 2004 and February 2009. Magnetic resonance imaging (MRI), computed tomography (CT) imaging, and pituitary function testing were performed. All cases underwent transsphenoidal surgery (TSS). Mean follow up was 38 months (range 12-80 months). RESULTS: Out of 152 patients with acromegaly due to GH-producing pituitary adenomas (female:male=73:79; age range 17-63 years), 69 patients had microadenomas (45.4%; 38 females, 31 males). We found 14 cases (14/69, 20.3%), all microadenomas, with presurgical evidence of ES - 10 females (71%) and 4 males (29%) (female:male=2.5:1). When compared with 103 patients with GH-negative microadenomas treated in the same time period (ES in 4 of 103; 3.9%), ES was highly significantly associated with GH production by the microadenoma (p=0.001). In acromegalics with empty sella, no cases of ectopic adenoma were found. Postoperatively, GH and IGF-1 levels fell in all patients, and 7 cases had random GH and IGF-1 levels consistent with cure. CONCLUSION: The combination of GH-producing microadenomas and empty, enlarged sella is not rare. In this setting, preoperative CT scans are very useful and the transsphenoidal approach is efficient and safe. The mechanism underlying the association of GH-producing microadenomas and empty sella remains unclear and requires further studies.

Septal cartilage plug technique in spontaneous cerebrospinal fluid rhinorrhea postoperatively diagnosed with partial empty sella syndrome.

The otolaryngologist should consider empty sella syndrome for diagnostic guidance when evaluating patients with nontraumatic spontaneous cerebrospinal fluid (CSF) rhinorrhea. The radiographic finding of empty sella is frequently reported in patients with benign intracranial hypertension (BIH). Patients who have a spontaneous CSF leak in the absence of florid symptoms of BIH may have a disrupted pattern of CSF flow, and because they are actively leaking CSF before surgical repair, they may be at risk for developing elevated intracranial pressure and BIH after the CSF leaks have been successfully closed. We describe a patient with CSF rhinorrhea who developed headache, papilledema, and visual disturbance after surgical repair of the CSF leak. The leak was repaired by the placement of a septal cartilage plug with a free mucosal suture graft. This technique provides fundamental biomechanical stability, reduces the complexity of the multilayer packing method, and promotes an effective seal.

Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease.

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.

Ectopic pituitary adenoma associated with an empty sella: a case report and review of the literature.

A case of ectopic pituitary adenoma in the sphenoid sinus associated with an empty sella is reported. The coexistence of an ectopic pituitary adenoma and an empty sella is quite rare. The diagnosis was made with an intraoperative finding of the intact dura mater of the sellar floor. In the present case, the hypointense line that coated the pituitary gland was clearly demonstrated on 3-tesla T2-weighted magnetic resonance imaging. The hypointense line is considered to be the pituitary capsule and was critical in diagnosing this rare entity.

Primary amenorrhea due to empty sella: an underestimated entity.

We report a 16-year-old girl who had stunted growth and primary amenorrhea presenting with headache and vomiting. MRI of brain showed posterior fossa dermoid cyst with hydrocephalus and empty sella. Growth hormone (GH), Leutinizing hormone (LH) and Follicular stimulating hormone (FSH) were grossly reduced on endocrinological work-up. She underwent suboccipital midline craniectomy and complete excision of dermoid cyst .She had no additional deficit after the surgery. She had menarche six months after the surgery and developed adequate secondary sexual characters, however her height remains subnormal at follow-up after 2 years as compared to age matched controls. We briefly review the radiological features, pathogenesis and outcome of this underestimated entity.

Coexistence of ectopic pituitary adenoma and empty sella in a patient with acromegaly : a case report and review of literature.

Ectopic pituitary adenoma with an empty sella is extremely rare. We report an unusual patient with an ectopic growth hormone-secreting pituitary adenoma in the sphenoid sinus with an empty sella. The association is related to a development disorder of the anterior pituitary tissues. Tumor in the sphenoid sinus was completely removed by endoscopic endonasal transsphenoidal approach. During the follow-up, the patient met the criteria for endocrinological cure.

Persistence of primary empty sella syndrome despite obesity surgery: report of two unusual cases.

Primary empty sella syndrome (ESS) shares with idiopathic intracranial hypertension (IIH) high intracranial pressure (ICP) and association with obesity. Bariatric surgery is effective in IIH treatment; no data, however, exist about ESS. We report two overweight ESS patients in whom bariatric surgery resolved obesity but failed in curing ESS, requiring cerebrospinal fluid (CSF) shunt placement.