Diabetes insipidus secondary to sellar/parasellar lesions.

Diabetes insipidus (DI) is a well-recognised transient or permanent complication following transsphenoidal surgery for pituitary adenomas or other sellar/parasellar lesions. However, data regarding the prevalence of pre-operative DI in sellar/parasellar lesions other than pituitary adenomas are scarce. We systematically reviewed the existing data for defining the prevalence of DI before any treatment in adult patients with sellar/parasellar lesions, excluding pituitary adenomas and metastatic lesions. In total, 646 patients with sellar/parasellar lesions presenting with DI at diagnosis were identified. The most common pathologies of sellar/parasellar lesions presenting with DI at diagnosis were lymphocytic hypophysitis (26.5%), craniopharyngiomas (23.4%), Langerhans's cell histiocytosis (18.9%) and Rathke's cleft cyst (12.7%), accounting for the vast majority (more than 80%) of these lesions. Overall, DI at diagnosis was found in 23.4% of all patients with sellar/parasellar lesions, albeit with a wide range from 10.6% to 76.7%, depending on the nature of the pathology. The highest prevalence of DI was found in less commonly encountered lesions namely germ-cell tumours (76.7%), abscesses (55.4%) and neurosarcoidosis (54.5%), each accounting for less than 3% of all sellar/parasellar lesions. Most DI cases (68.8%) were associated with anterior pituitary hormonal deficiencies, in contrast to pituitary adenomas that rarely present with DI. The enlargement and enhancement of the pituitary stalk were the most common findings on magnetic resonance imaging besides the loss of the high signal of the posterior pituitary on T1-weighted images. Resolution of DI spontaneously or following systemic and surgical management occurred in 22.4% of cases. Post-operative DI, not evident before surgery, was found in 27.8% of non-adenomatous sellar/parasellar lesions, and was transient in 11.6% of them. Besides distinctive imaging features and symptoms, early recognition of DI in such lesions is important because it directs the diagnosis towards a non-adenomatous sellar/parasellar tumour and the early initiation of appropriate treatment.

Assessment of post-operative healing following endoscopic, transnasal, transsphenoidal pituitary surgery without formal sellar grafting.

INTRODUCTION: Multiple options exist for sellar reconstruction after endoscopic transnasal, transsphenoidal surgery (TSS) including free mucosa, fat, bone and synthetic materials. The objective of this study was to assess healing and mucosalization of the sellar face following TSS without formal sellar grafting or reconstruction. METHODS: Single institution retrospective chart review was conducted for patients undergoing TSS without intraoperative CSF leaks between January 2014 and March 2017 at Rush University Medical Center. No formal sellar reconstruction was performed for the entire patient group. Follow-up endoscopic data and clinical notes were coded for time to mucosalization of the sella as well as degree of abnormal mucosal healing, epistaxis, crusting and scarring. RESULTS: 83 patients were included in this study. Mean time to mucosalization was 119 days (range, 17 to 402 days). Incidence of abnormal mucosal healing, epistaxis, crusting and scarring increased from the first to the second postoperative visit but trended down by the third visit. Nasal crusting was the most common finding, followed by abnormal mucosal healing. Chi square analysis showed smoking to be associated with prolonged time to full mucosalization of the sella. Two patients (2.4%) had post-operative CSF leaks requiring lumbar drain placement. CONCLUSION: Adequate sellar healing is achievable in all cases without formal grafting or reconstruction after TSS. Great care must be exercised given the small inherent risk of unmasking a subclinical intraoperative CSF leak. Patients should be followed closely endoscopically during the first four months after TSS to minimize the impact of crusting.

Morphometric analysis of sella turcica in growing patients: an observational study on shape and dimensions in different sagittal craniofacial patterns.

The aim of this study was to evaluate the differences in sella dimensions and shape between growing patients with Class I, Class II, and Class III skeletal malocclusions, evaluated through morphometric analysis. Seventy-eight subjects aged between 9 and 13 years were selected and assigned to either the Class I, Class II, or Class III groups according to the measured ANB angle (the angle between the Nasion, skeletal A-point and skeletal B-point). Six landmarks were digitised to outline the shape of the sella turcica. Linear measurements of the sella length and depth were also performed. Procrustes superimposition, principal component analysis, and canonical variate analysis were used to evaluate the differences in sella shape between the three groups. A one-way MANOVA and Tukey's or Games-Howell tests were used to evaluate the presence of differences in sella dimensions between the three groups, gender, and age. The canonical variate analysis revealed a statistically significant difference in sella shape between the Class I and the Class II groups, mostly explained by the CV1 axis and related to the posterior clinoidal process and the floor of the sella. No differences were found regarding linear measurements, except between subjects with different age. These differences in sella shape, that are present in the earlier developmental stages, could be used as a predictor of facial growth, but further studies are needed.

Degrees of Diaphragma Sellae Descent during Transsphenoidal Pituitary Adenoma Resection: Predictive Factors and Effect on Outcome.

This study is aimed to classify degrees of diaphragma sellae (DS) descent into sella turcica according to the surgical field block caused by the descent and to construct predictive imaging criteria for the degree of descent, and in addition, to determine whether there is any correlation between the degree of DS descent and the operative outcome (in the form of cerebrospinal fluid leak and/or presence of residual tumor). Totally, 72 patients were enrolled in our study. Their clinical and radiological data as well as the high definition videos of operations were retrospectively reviewed. The degree of DS descent during the operation was classified into five degrees according to surgical field block caused by the descent. We investigated the correlation between these five degrees and the clinical findings, radiological findings as well as the surgical outcomes. We found that the most important determining factors of DS descent degree were the volume and the height of the tumor portion above diaphragma opening. On the other hand, the total tumor volume, the maximum tumor height and the morphological pattern according to Wilson's system (modified from Hardy) had no statistically significant correlation with DS degree of descent. Presence of residual tumor on postoperative magnetic resonance images was significantly correlated with Wilson's classification and with supradiaphragmatic tumor height. On the other hand, cerebrospinal fluid leak showed no statistically significant difference between variable degrees of DS descent. Volumetric data of the tumor portion above the diaphragma opening are more important than morphological data for prediction of surgical field block caused by descended DS. While DS prolapse significantly increases the difficulty of the operative procedure, residual tumor presence is mainly dependent on morphological classification, especially cavernous sinus invasion.

Glimpse into Pathophysiology of Sellar Arachnoid Cysts.

BACKGROUND: Sellar arachnoid cysts are a rare occurrence but may impinge on vital parasellar anatomy and thus are often symptomatic. The etiology of sellar arachnoid cysts is contentious, fueled by heterogeneity in cyst wall structure and contents between cases. The "ball-valve" mechanism is 1 of 2 predominant theories describing their formation, which contends that an aperture in the diaphragm allows cerebrospinal fluid to enter the cyst, propelled by pulsatile flow, but its egress is obscured by the pituitary during the ebb of the pressure wave. CASE DESCRIPTION: Here we present a case of a 51-year-old female with a symptomatic sellar arachnoid cyst. She underwent an endoscopic transsphenoidal fenestration which alleviated her symptoms. CONCLUSIONS: Intraoperative video evidence during arachnoid cyst fenestration supports the "ball-valve" theory of sellar arachnoid cyst development.

DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review.

Primary empty sella (PES) is characterized by the herniation of the subarachnoid space within the sella, which is often associated with variable degrees of flattening of the pituitary gland in patients without previous pituitary pathologies. PES pathogenetic mechanisms are not well known but seem to be due to a sellar diaphragm incompetence, associated to the occurrence of upper sellar or pituitary factors, as intracranial hypertension and change of pituitary volume. As PES represents in a majority of cases, a neuroradiological findings without any clinical implication, the occurrence of endocrine, neurological and opthalmological symptoms, due to the above describes anatomical alteration, which delineates from the so called PES syndrome. Headache, irregular menses, overweight/obesity and visual disturbances compose the typical picture of PES syndrome and can be the manifestation of an intracranial hypertension, often associated with PES. Although hyperprolactinemia and growth hormone deficit represent the most common endocrine abnormalities, PES syndrome is characterized by heterogeneity both in clinical manifestation and hormonal alterations and can sometime reach severe extremes, as occurrence of papilledema, cerebrospinal fluid rhinorrhea and worsening of visual acuity. Consequently, a multidisciplinary approach, with the integration of endocrine, neurologic and ophthalmologic expertise, is strongly advocated and recommended for a properly diagnosis, management, treatment and follow-up of PES syndrome and all of the related abnormalities.

Invagination of the Sphenoid Sinus Mucosa after Endoscopic Endonasal Transsphenoidal Approach and Its Significance.

OBJECTIVE: To describe the clinical features of invagination of the sphenoid sinus mucosa (ISM) and compare them with other similar cases using a visual analog scale (VAS) to assess the various nasal symptoms and to discuss its clinical significance and means of prevention. STUDY DESIGN: Retrospective chart review at a tertiary referral center. METHODS: Between 2010 and 2015, 8 patients who had undergone EETSA surgery displayed postoperative ISM. The comparison group consisted of 147 patients who underwent the same surgical procedures and were diagnosed with the same diseases. Pre- or postoperative paranasal sinus computed tomography (PNS CT) and VAS were performed and subsequently analyzed. RESULTS: The clinical features of ISM of the sphenoid sinus showed sellar floor invagination and regenerated inverted ingrowing sphenoid mucosa on endoscopic imaging. PNS CT also showed a bony defect and invaginated air densities at the sellar turcica. Pre- and postoperative VAS scores revealed that the ISM group had much less of an improvement in headaches after surgery than that of the comparison group (p = 0.049). CONCLUSION: ISM may occur because of a change in pressure, sphenoid mucosal status, or arachnoid membrane status. Moreover, ISM is related to improvements in headaches. Therefore, EETSA patients should avoid activities that cause rapid pressure changes during the healing process. In addition, sellar reconstruction that is resistant to physical pressure changes should be mandated despite the absence of an intraoperative CSF leak.

Sellar Chondroma with Endocrine Dysfunction that Resolved after Surgery: Case Report.

Chondromas originating from the sella turcica are rare, and the most common initial symptoms are headache and visual disturbance. We describe a case of sellar chondroma with endocrine impairment as an initial manifestation that completely resolved after surgery. A 40-year-old Japanese woman with amenorrhea and galactorrhea for the last 2 years was referred to our department of neurosurgery for the evaluation of high prolactin levels and a tumor in the sella turcica. A biochemical assessment indicated endocrine dysfunction. Magnetic resonance imaging and computed tomography indicated a tumor in the sella turcica. The patient's presentation favored the preoperative diagnosis of pituitary adenoma or Rathke's cleft cyst. However, because calcification was detected, other types of tumors, such as craniopharyngioma, meningioma, and chordoma, were also considered. Endoscopic transsphenoidal surgery was performed, and the possibility of a bony tumor was recognized. Finally, the tumor was completely removed, and the histopathological findings confirmed chondroma. The postoperative course was uneventful, and endocrine function improved. Five years after surgery, the patient is doing well without pituitary insufficiency, pituitary hormone medications, or signs of tumor recurrence. In cases of sellar chondroma, endocrine dysfunction sometimes precedes other symptoms, such as headache and visual disturbance. When examining a patient with an intrasellar tumor harboring calcification, clinicians must consider the possibility of sellar chondroma. Furthermore, to the best of our knowledge, this case is the first of sellar chondroma treated with endoscopic surgery to be reported.

Sellar meningiomas: an endocrinologic perspective.

To review the clinical manifestations and outcomes of those with sellar meningiomas treated surgically at Mayo Clinic between 1975 and 2003. This is a retrospective chart and pathology review of 17 patients with a diagnosis of purely or largely intrasellar meningiomas treated surgically at our institution. Data in regards to presentation, endocrine hormonal status, surgical approach, pathology findings, outcome and adjunctive treatment were abstracted from the medical records. The majority of patients present with visual disturbances. All 17 tumors were WHO grade I. Surgical cure was achieved in 53 % after initial surgery. Postsurgical hypopituitarism occurred with high frequency. A substantial proportion of patients required subsequent surgical intervention or adjunctive treatment with external beam radiation. Sellar meningiomas are technically challenging and carry a high risk for visual disturbance and pituitary hormonal abnormalities. Many patients experience persistent disease requiring further intervention. These patients require long-term follow-up for evaluation of recurrence or development of new pituitary hormonal insufficiencies.

A short illustrated review of sellar region schwannomas.

INTRODUCTION: In this short illustrated review, we present the case of a frontal lobe schwannoma without cranial nerve or sellar attachment and a sellar schwannoma which mimicked a pituitary adenoma. LITERATURE REVIEW: We review the rare occurrence of sellar region schwannomas, their clinical presentation, operative management, outcomes, and the possible pathogenesis. ANALYSIS: The absence of Schwann cells in the central nervous system and the absence of neighboring cranial nerves in these two cases raise issues of the pathogenesis of these rare but curious lesions.

Primary sellar lymphoma: intravascular large B-cell lymphoma diagnosed as a double cancer and improved with chemotherapy, and literature review of primary parasellar lymphoma.

Lymphoma is one of the causative factors of hypothalamus-pituitary dysfunction, and intravascular large B-cell lymphoma (IVLBCL) is a subtype of primary extranodal neoplasm. A 69-year-old woman visited our hospital because of general fatigue. We diagnosed her with presumable non-functional primary pituitary adenoma and subsequent dysfunction. Eight months after, the patient revisited our hospital because of dyspnea. Though we conducted systemic investigations including chest and abdomen enhanced computer tomography, transbronchial lung biopsy, and bone marrow biopsy, the diagnosis was not confirmed. Inadvertently, a breast cancer was found, and the surgical specimen proved that the patient had double cancer-adenocarcinoma and IVLBCL. Rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone regimen was initiated, and complete remission was achieved. Notably, the sellar mass returned to normal size with improved function. We reviewed 32 patients with primary parasellar lymphoma. In affected sites, both sellar and pituitary stalk (6.7%), both hypothalamus and pituitary stalk (6.7%), only sellar (63.3%), only pituitary stalk (6.7%), only hypothalamus (13.3%), and only clivus (3.3%) were observed. In hypothalamus-pituitary dysfunction, both anterior and posterior dysfunction (20.7%), only anterior dysfunction (58.6%), only posterior dysfunction (3.4%), and no dysfunction (17.2%) were observed. It seemed that hypothalamic lesion is related to both anterior and posterior dysfunction, while sellar lesion is related to mainly anterior dysfunction. In cranial nerve dysfunction, 2nd nerve dysfunction (45.2%) and 6th nerve dysfunction (35.5%) were frequently observed. It seemed that sellar lesion is related to both 2nd and 6th nerve dysfunction, while hypothalamic lesion is related to mainly 2nd nerve dysfunction.

Sellar angiolipomas: two case reports and a review of the literature.

Angiolipomas are mesenchymal hamartomas composed of abnormal blood vessels and mature adipose tissue, most commonly found in the subcutaneous tissue of the extremities. Intracranial location is extremely rare, and only five cases have been described in the sellar region. We report on two patients that were initially diagnosed with pituitary adenomas that were postoperatively verified as angiolipomas. Sellar angiolipomas should be considered in the differential diagnosis of pituitary lesions due to the potential catastrophic bleeding during surgery. Preoperative diagnosis is very difficult; however, some MRI characteristics can help make an accurate diagnosis. Adequate MRI sequences should be used in the evaluation of pituitary lesions, as they can help optimize the microsurgical management.

Intrasellar pressure and tumor volume in pituitary tumor: relation study / Relação entre pressão intra-selar e volume de tumor de hipófise

OBJECTIVE: To determine if there was a relationship between intrassellar pressure (ISP) and pituitary tumor volume. METHOD: Between August 2002 and May 2004, 60 patients aged between 13 and 75 years old (39 males), having a pituitary adenoma were submitted to an endoscope transseptal approach. During the surgery and before tumor resection, 2 mm of the sellaÆs floor were removed and a 1.5 mm dural opening made to place a transducer into the pituitary adenoma. The transducer was connected to a pressure monitor. RESULTS: The intrasellar pressure, ranged from 2-51 mmHg and was measured based on the classification of Hardy-Vezina. The most elevated was in the type II macro adenomas with 32.6 mmHg, sharply superior to the value of a normal intracranial pressure. CONCLUSION: These values showed that the macroadenomas confined to the sella, without destruction of the floor and integrity of the diaphragm, type II of Hardy-Vezina, presented a value of ISP much higher than intra-extrasellar macroadenomaÆs.

OBJETIVO: Determinar se existia uma relação entre a pressão intraselar (ISP) e o volume de tumor de hipófise. MÉTODO: Entre agosto de 2002 e maio de 2004, 60 pacientes com idades variando entre 13 e 75 anos (39 homens), portadores de adenoma hipofisários foram operados por via transesfenoidal. Durante o ato cirúrgico e antes da resseção do tumor, uma osteotomia de 2 mm foi realizada no assoalho selar e uma abertura de 1,5 mm na duramater para a introdução de um transdutor dentro do tumor. O transdutor foi conectado a um monitor de pressão e esta foi medida por 2 minutos. RESULTADOS: A pressão intra-selar variou entre 2-51 mmHg e a correlação entre tamanho do tumor e ISP foi baseada na classificação de Hardy-Vezina. A média da ISP mais elevada foi encontrada nos macroadenomas tipo II com 32,6 mmHg, nitidamente superior a pressão intra selar normal. CONCLUSÃO: Estes valores mostram que os macroadenomas confinados a sela sem destruição do assoalho selar e com integridade do diafragma, classificados como tipo II de Hardy-Vezina, apresentam uma ISP muito superior aos outros adenomas.

Recurrent lymphocytic hypophysitis and bilateral intracavernous carotid artery occlusion. an observation and review of the literature.

OBJECTIVES: Bilateral carotid artery occlusion associated with lymphocytic hypophysitis is exceedingly rare. We describe this association and review the literature. METHODS: The authors describe a 38-year-old woman with a history of severe headaches. Magnetic resonance (MR) imaging showed an intrasellar mass with invasion of both cavernous sinuses. Lymphocytic hypophysitis was diagnosed by transphenoidal biopsy. In the course of the disease, she developed symptoms of cerebral ischemia attributable to bilateral occlusion of her internal carotid arteries in both cavernous sinuses. She underwent bilateral superficial temporal artery-middle cerebral artery bypass surgery. RESULTS: The patient experienced progressive neurological recovery after surgery. A literature search revealed no other cases describing this unique association. CONCLUSIONS: Bilateral carotid artery occlusion may develop in the course of lymphocytic hypophysitis with cavernous sinus involvement. If indicated, cerebral revascularization should be performed to reverse cerebral ischemia.

Xanthogranuloma of the sellar region.

The incidence of diagnosed xanthogranuloma of the sellar region is very low [1, 2, 5, 6]. We report about two cases 1) in a 57-year-old female and 2) in a 5-year-old boy. In both cases radiographic findings revealed an inhomogeneous, contrast enhancing sellar lesion. Histopathology showed the typical features of a xanthogranuloma of the sellar region with cholesterol clefts, lympho-plasmacellular infiltrates, marked hemosiderin deposits, multinucleated foreign body giant cells around cholesterol clefts, accumulation of macrophages and only small epithelial cell clusters [6]. As xanthogranuloma of the sellar region are rarely diagnosed we want to draw attention to this rather unusual diagnosis.

Intrasellar pressure and tumor volume in pituitary tumor: relation study.

OBJECTIVE: To determine if there was a relationship between intrassellar pressure (ISP) and pituitary tumor volume. METHOD: Between August 2002 and May 2004, 60 patients aged between 13 and 75 years old (39 males), having a pituitary adenoma were submitted to an endoscope transseptal approach. During the surgery and before tumor resection, 2 mm of the sellas floor were removed and a 1.5 mm dural opening made to place a transducer into the pituitary adenoma. The transducer was connected to a pressure monitor. RESULTS: The intrasellar pressure, ranged from 2-51 mmHg and was measured based on the classification of Hardy-Vezina. The most elevated was in the type II macro adenomas with 32.6 mmHg, sharply superior to the value of a normal intracranial pressure. CONCLUSION: These values showed that the macroadenomas confined to the sella, without destruction of the floor and integrity of the diaphragm, type II of Hardy-Vezina, presented a value of ISP much higher than intra-extrasellar macroadenoma's.

A case of a GH-producing pituitary adenoma associated with a unilateral headache with autonomic signs.

A 66-year-old man suffered from a drug-resistant, left-sided headache with autonomic signs, triggered by the supine position. The acromegalic facies initially suggested a possible increase in basal plasma levels of GH, but routine haematological controls excluded abnormal values of GH. Cerebral and facial CT scan and MRI did not detect any alterations in the nasal sinuses, except for a mucous cyst. Surgical ablation of the cyst did not alleviate the pain. Further endocrinological the pain. Further endocrinological tests demonstrated an increase of IGF-1 (somatomedin C), and another MRI scan of the sellar region confirmed the presence of a pituitary macroadenoma on the left paramedian side. After an initial improvement of the symptomatology due to trans-sphenoidal ablation of a benign GH-producing macroadenoma, the headache worsened again. Pain was well correlated with the increased plasma levels of IGF-1. The patient died suddenly for myocardial infarct.

Outcome determinants of pterional surgery for tuberculum sellae meningiomas.

BACKGROUND: Current literature on tuberculum sellae meningiomas is very heterogenous due to wide variation in nomenclature, diagnostic and operative techniques. The aim of this study is specifically to analyze the results of pterional craniotomy for tuberculum sellae meningiomas. A homogenous cohort of 42 consecutively operated tuberculum sellae meningioma cases are reviewed with special emphasis on the effects of pterional microsurgery on visual outcome. METHODS: This is a retrospective clinical analysis. 42 consecutive patients operated upon during the period of 15 years in a single institution using standard imaging protocols and pterional microsurgery are presented and effect of various variables on visual outcome analysed. FINDINGS: 81% of the patients presented with visual symptoms. The mean duration of symptoms was 12 months. Tumour volumes ranged from 7.5 to 210 mm(3). A right sided pterional microsurgery was used in all patients. Complete resection rate was 81%. Vision improved in 58%, worsened in 14%. Non-visual morbidity was 7.1% and mortality was 2.4%. The follow up period of patients ranged from 3 to 192 months (median: 30 months). The mean was 37.5 months (SD = +/-36.7 months) and a recurrence rate of 2.4% was observed. CONCLUSIONS: A standard pterional craniotomy using microsurgical technique provides the necessary exposure enabling total removal while keeping the complications to a minimum. Upon analysis of our findings we found that patient age of more than 60, duration of visual symptoms longer than 1 year, severe visual symptomatology, predominantly vertical growth, presence of significant peri-tumoural oedema, absence of an intact arachnoid plane and subtotal removal were correlated with a dismal visual outcome.

Extreme elevation of intrasellar pressure in patients with pituitary tumor apoplexy: relation to pituitary function.

The dominant mechanism for hypopituitarism and hyperprolactinemia commonly observed in patients with pituitary macroadenomas was postulated to be increased intrasellar pressure (ISP) caused by the slow and gradual expansion of adenomas within the sella turcica. Hemorrhagic infarction of adenomas (pituitary tumor apoplexy) is associated with a rapid, rather than gradual, increase in intrasellar contents. The impacts of the sudden increase in intrasellar contents on ISP and pituitary function are unknown. ISP and pituitary function were determined in 13 patients with pituitary tumor apoplexy who had surgical decompression within 1 wk of symptoms' onset. ISP measurements were remarkably high (median, 47 mm Hg), whereas serum prolactin (PRL) concentrations were generally low (median, 3.5 microg/liter). There was an inverse correlation (r = -0.76; P < 0.01) between ISP measurements and serum PRL concentrations. Postoperatively, partial recovery or maintenance of pituitary function was noted in seven of 13 patients. These seven patients had higher (P = 0.013) serum PRL levels (9.3 +/- 7.4 microg/liter) and lower (P < 0.001) ISP measurements (35.9 +/- 7.3 mm Hg) than the respective values in the remaining six with persistent postoperative hypopituitarism (1.6 +/- 0.6 microg/liter and 55.9 +/- 2.4 mm Hg, respectively). The low serum PRL levels in patients with tumor apoplexy suggested that ischemic necrosis of the anterior pituitary resulting from sudden and extreme elevation of ISP was commonly observed in this setting. A normal or elevated serum PRL level in patients with non-PRL-secreting macroadenomas indicates the presence of viable pituitary cells and the high likelihood of postoperative recovery of pituitary function.