[Basic Knowledge and Current Evidence on Cerebrospinal Shunt Devices].

Revision surgeries after cerebrospinal shunt placement are sometimes unavoidable mainly because of the mechanical limitations of current shunt devices despite their technological improvements. This article discusses evidence on existing shunt devices, basic knowledge on their structure and function, and possible future issues.

Development of a Lumbar Extradural Arachnoid Cyst Associated with a Lumbar Catheter of Lumboperitoneal Shunt: A Case Report.

A 78-year-old man, who had undergone lumboperitoneal shunt (LPS) placement for idiopathic normal-pressure hydrocephalus eight years prior, presented with intermittent claudication, lower back pain, and radicular pain on the inside of the right thigh. Magnetic resonance imaging (MRI) revealed an extradural arachnoid cyst (EDAC) above the lumbar catheter of the LPS. The EDAC compressed the spinal dural sac and cauda equina toward the anterior side at level L3/4, triggering his clinical manifestations. The LPS was removed and simultaneously converted into a ventriculoperitoneal shunt (VPS), which immediately improved the neurological deficits. Postoperative MRI showed shrinkage of the cyst and restoration of the compressed cauda equina. Spinal EDAC is a rare entity resulting from arachnoid membrane herniation due to a small defect in the dura mater. This is the first report showing that symptomatic EDAC can be accompanied by the lumbar catheter of the LPS and that a mere conversion from LPS to VPS or ventriculoatrial shunt might be sufficient to shrink LPS-related EDAC without invasive lumbar surgeries.

Receptor Tyrosine Kinases: Principles and Functions in Glioma Invasion.

Protein tyrosine kinases are enzymes that are capable of adding a phosphate group to specific tyrosines on target proteins. A receptor tyrosine kinase (RTK) is a tyrosine kinase located at the cellular membrane and is activated by binding of a ligand via its extracellular domain. Protein phosphorylation by kinases is an important mechanism for communicating signals within a cell and regulating cellular activity; furthermore, this mechanism functions as an "on" or "off" switch in many cellular functions. Ninety unique tyrosine kinase genes, including 58 RTKs, were identified in the human genome; the products of these genes regulate cellular proliferation, survival, differentiation, function, and motility. Tyrosine kinases play a critical role in the development and progression of many types of cancer, in addition to their roles as key regulators of normal cellular processes. Recent studies have revealed that RTKs such as epidermal growth factor receptor (EGFR), platelet-derived growth factor receptor (PDGFR), c-Met, Tie, Axl, discoidin domain receptor 1 (DDR1), and erythropoietin-producing human hepatocellular carcinoma (Eph) play a major role in glioma invasion. Herein, we summarize recent advances in understanding the role of RTKs in glioma pathobiology, especially the invasive phenotype, and present the perspective that RTKs are a potential target of glioma therapy.

Contribution of Intrasellar Pressure Elevation to Headache Manifestation in Pituitary Adenoma Evaluated With Intraoperative Pressure Measurement.

BACKGROUND: Headache frequently occurs in patients with pituitary adenoma and is reported in large as well as small adenomas. However, the exact mechanism of headache derived from pituitary adenoma remains unknown. OBJECTIVE: To evaluate the contribution of intrasellar pressure (ISP) to headache manifestation by using intraoperative ISP measurement. METHODS: The records of 108 patients who had first-time transsphenoidal surgery for pituitary adenoma were reviewed retrospectively. Measurement of intraoperative ISP was undergone using intracranial pressure monitoring sensors and compared with radiological assessment. RESULTS: Among 30 patients with headache, 29 (96.7%) presented with significant headache (Headache Impact Score-6, 50 or greater). Intraoperative ISP measurement was conducted successfully in all cases, and revealed higher ISP in patients with headache (35.6 ± 9.2 mm Hg) than in those without headache (15.8 ± 5.2 mm Hg). The ISP reduction after sella floor decompression was greater in patients with headache than that in patients without headache. In patients with headache, the frequency of invasion into the cavernous sinus or sphenoid sinus was significantly lower, and the diameter of the foramen at the diaphragm sellae was narrower. In addition, intratumoral cyst or hematoma was more common in patients with headache. Postoperatively, headache was either diminished or improved in 28 patients (93.3%). CONCLUSION: Headache in patients with pituitary adenomas associated with ISP elevation, results from compromised dural integrity at the sella and intratumoral hemorrhage. The increased stretch force of the sella dura may be a notable etiology of headache in patients with pituitary adenoma.

Directional Regulation of Extrasellar Extension by Sellar Dura Integrity and Intrasphenoidal Septation In Pituitary Adenomas.

OBJECTIVE: Pituitary macroadenomas extend into the extrasellar space, such as the sphenoid sinus, cavernous sinuses, and suprasellar region. However, factors that regulate the direction of their extensions into the surrounding anatomical structures remain unknown. METHODS: This retrospective study included 162 patients who were treated for pituitary adenomas that had maximum diameters greater than 20 mm. According to the direction of adenoma extension, patients were divided into 4 groups: group A, inferior into the sphenoid sinus; group B, lateral into the cavernous sinus; group C, suprasellar region with enlarged sella turcica; and group D, supraellar region without enlarged sella turcica. Several anatomical structures surrounding the sella turcica were evaluated statistically, and multivariate logistic regression analysis was performed if the structures met the determining factors of adenomas extensions. RESULTS: The maximum diameter of adenomas was significantly larger in groups A and D. The maximum diameter of the diaphragmatic foramen was largest in group C (19.3 mm) and was significantly narrower in groups B (12.7 mm) and D (12.5 mm). Intrasphenoid septation, attached on the midline of the sella turcica, was observed most frequently in group D (78.6%) and was not detectable in group A (0%). Extension into the cavernous sinus, classified as dural discontinuity, was highly prevalent in group B (80.0%) and was uncommon in group C (12.3%). Erosion of the posterior clinoid process was most apparent in group B (92.0%). CONCLUSIONS: The integrity of the sella dura and the intrasphenoid septation can regulate adenoma extension by encouraging their growth towards paths of least resistance.

Outcome of diabetes insipidus in patients with Rathke's cleft cysts.

OBJECTIVES: It is well-known that Rathke's cleft cysts (RCCs) infrequently cause headache, endocrinological dysfunction, and visual disturbance, and in rare cases, cause diabetes insipidus (DI). Although surgical evacuation of the cyst content can result in high rates of symptomatic improvement, not only the treatment efficacy but also the pathophysiology of DI with RCC are undetermined. The aim of this study is to elucidate the underlying mechanisms and outcomes of DI associated with RCCs. PATIENTS AND METHODS: We retrospectively studied 109 patients with RCCs treated at Kanazawa University Hospital between 2000 and 2016. Their age, sex, symptoms, endocrinological status, DI, visual disturbance, neuroradiological findings, pathological appearances, and pre-/post-operative hormone levels and status of anti-diuretic hormone replacements were assessed. RESULTS: Among 109 cases of RCCs, five cases (4.6%, 2 males and 3 females) manifested with DI as initial presentation were included. These five cases could be divided into two types: the acute type and the chronic type, based on the onset and duration of symptoms. Three acute onset cases presented with not only strong thirst but also sudden headaches without pituitary dysfunction, whereas the two chronic onset cases presented with chronic headaches and hypopituitarism. Pathological examination in the acute type revealed inflammatory cell infiltration into only the posterior lobe of the pituitary and disruption of the cyst wall adjacent to the posterior lobe, which might suggest RCC rupture. In contrast, the chronic type showed inflammatory cell infiltration into both the anterior and posterior lobes of the pituitary and thickened fibrosis beneath the cyst wall. Postoperatively, two cases of the acute type could be controlled with a smaller amount of 1-deamino-8-D-arginine vasopressin (DDAVP) than that required preoperatively, whereas no change was observed in the cases of the chronic type. CONCLUSION: The cases of DI onset caused by RCCs could be divided into the acute type and the chronic type. In the chronic type, surgical treatment could not affect the status of DI. However, in acute type, urgent surgical intervention partially relieved DI.

Delayed Occurrence of Diabetes Insipidus After Transsphenoidal Surgery with Radiologic Evaluation of the Pituitary Stalk on Magnetic Resonance Imaging.

BACKGROUND: Diabetes insipidus (DI) is a major complication of transsphenoidal surgery (TSS). DI usually occurs within a couple of days after TSS. Delayed occurrence of postoperative DI is rarely observed and its developing mechanisms remain unknown. METHODS: Six patients were identified as having postoperative delayed DI, which was defined as DI that first occurred 2 or more weeks after TSS. They consisted of 1 male and 5 females, and their mean age was 38.3 years (range, 10-76 years). Five patients were histologically diagnosed with Rathke cleft cyst (RCC), and one had RCC coexisting with prolactin-secreting adenoma. Sequential T1-weighted magnetic resonance imaging was evaluated for hyperintensity (HI) in the pituitary stalk and the posterior lobe, indicating the location of antidiuretic hormone. RESULTS: No patients had any DI before TSS. Delayed DI occurred 2 weeks to 3 months after TSS and persisted for 2 weeks to 5 months. T1-weighted magnetic resonance imaging showed that the HI in the posterior lobe became faint but did not disappear after DI occurrence, and their intensities increased with recovery from DI. In contrast, the HI in the pituitary stalk was found faintly preoperatively and turned clear postoperatively and decreased with recovery from DI. The morphologic patterns were dependent on DI duration. CONCLUSIONS: In the delayed occurrence of DI, it was suggested that preoperative antidiuretic hormone transport was mildly congested yet not completely blocked when DI manifested postoperatively. Gradual spreading of inflammation to the infundibulum after RCC removal was considered as 1 possible mechanism of this delayed DI development.

Contribution of sellar dura integrity to symptom manifestation in pituitary adenomas with intratumoral hemorrhage.

PURPOSE: Although hemorrhage within pituitary adenomas frequently exacerbates the symptoms, there are many grades of severity. Moreover, the contributing factors for symptom severity are still controversial. METHODS: This retrospective study included 82 patients who underwent transsphenoidal surgery for pituitary adenomas with intratumoral hemorrhage. The grades of preoperative symptoms were classified into group A, asymptomatic or minor symptoms; group B, moderate symptoms sufficient for complain; and group C, severe symptoms disturbing daily life. RESULTS: The hemorrhage volume within an adenoma was significantly higher in group C (92.6%) than in groups A (48.6%) and B (58.7%). Both headache and diplopia were dominant in group C, occurring in 72.2% and 27.8% of the patients, respectively. In group C, there was no significant difference in frequency between adenoma extensions into the sphenoid sinus (0%) and involvement of the cavernous sinus of Knosp grade 4 (0%), and extensions into the suprasellar region were not common (38.9%). The most distinctive feature was that "no extrasellar extension" was found only in group C (41.2%), and "multidirectional extension" was not detected in this group (0%). Multiple regression analysis revealed that the most powerful determining factors were the high frequencies of intratumoral hemorrhage and lack of extrasellar and multidirectional extensions. CONCLUSION: Rapid volume expansion of a hematoma and lack of extension or unidirectional extension might lead to significant compression of the sellar and surrounding structures. Of note, the integrity of the sellar dura might contribute to the acute onset of symptom manifestations caused by hemorrhage in pituitary adenomas.

Preoperative Evaluation of the Interface Between Tuberculum Sellae Meningioma and the Optic Nerves on Fast Imaging with Steady-State Acquisition for Extended Endoscopic Endonasal Transsphenoidal Surgery.

INTRODUCTION: Endoscopic endonasal transsphenoidal surgery (EETS) is increasingly applied to treat tuberculum sellae meningiomas. However, if the tumor adheres firmly to the optic nerve, dissection of the interface between both structures should be prudent to preserve visual function. The purpose of this study was to investigate whether tumor adhesion to the optic nerve can be predicted preoperatively by fast imaging with steady-state acquisition (FIESTA). METHODS: Twenty-two patients with tuberculum sellae meningioma treated with EETS were retrospectively identified. Clinical characteristics, radiologic studies, intraoperative findings, and outcomes were reviewed from their clinical charts. RESULTS: Patients' symptoms included visual function impairment in 18 patients and headaches in 4 patients. Symptoms were resolved in 19 patients after operation. Preoperative radiologic evaluation was performed in 44 sides (22 patients) of the interface between tumors and the optic nerves and showed absence of peritumoral hyperintensity on FIESTA in 7 sides in 7 patients. In 5 of the 7 sides, tumor dissection was complicated by firm adhesion to the optic nerves. Among these cases, visual functions were unchanged in 1 patient after complete removal of the adhesion but substantially improved in 3 patients after partial resection. In the remaining 37 sides with preoperative peritumoral hyperintensity, no adhesion was found between both structures intraoperatively. CONCLUSIONS: Absence of peritumoral hyperintensity between tuberculum sellae meningioma and the optic nerve on FIESTA may indicate firm adhesion at the interface, severely complicating complete removal. Preoperative recognition of this adhesion is important for safe tumor removal and preservation of visual functions.

Significant Improvement in Chronic Persistent Headaches Caused by Small Rathke Cleft Cysts After Transsphenoidal Surgery.

PURPOSE: Rathke cleft cysts (RCC) usually are asymptomatic and can be observed via the use of conservative methods. Some patients with RCCs, however, have severe headaches even if they are small enough to be confined to the sella, and these small RCCs seldom have been discussed. This study presents an investigation into clinical characteristics of small RCCs associated with severe headaches, demonstrating efficacy and safety of endoscopic transsphenoidal surgery (ETSS) to relieve headaches. METHODS: In this study, 13 patients with small RCCs (maximum diameter <10 mm) who presented with headaches and were treated by ETSS at our institute from 2009 to 2014 were recruited. These RCCs were treated Headache Impact Test-6 (HIT-6) score was calculated both pre- and postoperatively to evaluate headache severity. RESULTS: All patients complained of severe headaches, which disturbed their daily life. Most headaches were nonpulsating and localized in the frontal area. Characteristically, 6 patients (46%) experienced severe headaches with sudden onset that continued chronically. HIT-6 score was 64 on average, meaning headaches affected daily life severely. After surgical decompression of the cyst, headache in all of the patients improved dramatically and HIT-6 score decreased significantly to 37, suggesting that headaches were diminished. No newly developed deficiencies of the anterior pituitary lobe function were detected. Postoperative occurrence of diabetes insipidus was found in 2 patients, both of which were transient. No recurring cysts were found. CONCLUSIONS: Severe headaches can develop from small RCCs. In the present study, ETSS was performed on such patients effectively and safely to relieve their headaches.

Descent of the anterior communicating artery after removal of pituitary macroadenoma using transsphenoidal surgery.

BACKGROUND: After removal of pituitary macroadenoma, the anterior communicating artery (AComA) descends toward the original position. However, the process and contributing factors of this descent are not elucidated. METHODS: This retrospective study included 102 patients who underwent transsphenoidal surgery (TSS) for macroadenomas with maximum diameters of >2 cm. Sequential T2-weighted magnetic resonance images were used to assess the AComA flow void and its distance from the planum sphenoidale before and after TSS. The AComA position in relation to the adenoma was divided into four groups as follows: anterior, anterosuperior, superior, and posterior. The descent was compared to the presence of intratumoral hemorrhage or adenoma extension into the sphenoid sinus. RESULTS: One week after TSS, the AComA descent was more pronounced than when originally in the superior position (6.5 ± 3.7 mm vs 4.4 ± 3.5 mm, P < 0.0001). The postoperative descents of the AComA were well correlated with those of residual adenomas only when in the superior position (P = 0.030). The AComA descent was more significant at 1 week (4.4 ± 3.5 mm) than at 1 week to 3 months (0.7 ± 1.0 mm) in all the groups. Both intratumoral hemorrhage and sphenoid sinus extension of adenoma did not affect the AComA descent in each group. CONCLUSION: AComA descent was most influenced when it was superior to the macroadenoma and progressed mostly within 1 week after TSS, probably initiating during TSS. The position of the AComA in relation to a macroadenoma should be considered preoperatively to avoid vascular injury.

Prediction of postoperative diabetes insipidus using morphological hyperintensity patterns in the pituitary stalk on magnetic resonance imaging after transsphenoidal surgery for sellar tumors.

PURPOSE: Diabetes insipidus (DI) remains a complication of transsphenoidal surgery (TSS) for sellar and parasellar tumors. Antidiuretic hormone (ADH) appears as hyper intensity (HI) in the pituitary stalk and the posterior lobe of the pituitary gland on T1-weighted magnetic resonance (MR) imaging. Its disappearance from the posterior lobe occurs with DI, indicating a lack of ADH. The appearance of HI in the pituitary stalk indicates disturbances in ADH transport. METHODS: This retrospective study included 172 patients undergoing TSS for sellar tumors at our institute from 2006 to 2014. Sequential T1-weighted MR images without enhancement were evaluated for HI in the pituitary stalk and the posterior lobe to assess the localization of ADH before and at intervals after TSS. DI was assessed pre- and postoperatively. HI in the pituitary stalk showed the following morphology: (1) ovoid in the distal end of the pituitary stalk (group A), (2) linear in the distal part of the pituitary stalk (group B), (3) linear in the whole pituitary stalk (group C). RESULTS: Preoperative DI occurred in 6 patients (3.5 %) with no HI observed in the posterior lobe. Postoperative DI was transient in 82 patients (47.7 %), and permanent in 11 (6.4 %). One week after surgery, HI was absent in the posterior lobe in 74 patients (43.0 %), and present in the pituitary stalk in 99 patients (57.6 %); both were significantly correlated with postoperative DI (p < 0.001). The absence of HI in the posterior lobe (A, 48.9 %; B, 68.3 %; C, 92.3 %), persistence of DI (A, 3.7 days; B, 45.9 days; C, 20.5 months), and duration until HI recovery in the posterior lobe (A, 3.6 months; B, 6.8 months; C, 22.9 months) were greatest in group C, followed by group B, and then group A. Fourteen group A patients did not have postoperative DI despite having HI in the pituitary stalk and the posterior lobe. Four group C patients developed permanent DI with persistence HI in the pituitary stalk. CONCLUSION: HI in the pituitary stalk and its absence in the posterior lobe indicated postoperative DI, which was transient if HI was detected in the pituitary stalk. DI duration could be predicted by the length of HI in the pituitary stalk, which corresponded to the degree of ADH transport obstruction.

Xanthomatous hypophysitis associated with autoimmune disease in an elderly patient: A rare case report.

BACKGROUND: Xanthomatous hypophysitis (XH) is an extremely rare form of primary hypophysitis characterized by infiltration of the pituitary gland by mixed types of inflammatory cells, including foamy cells, plasma cells, and small mature lymphocytes. XH manifests as varying degrees of hypopituitarism. Although several previous reports have denied a possible contribution of autoimmune mechanism, the exact pathogenesis of XH remains unclear. CASE DESCRIPTION: We describe the case of a 72-year-old woman with a history of rheumatoid arthritis and Sjögren's syndrome who presented with panhypopituitarism and diabetes insipidus. At the time of her visit, she also experienced relapsed rheumatoid arthritis and Sjögren's syndrome, manifesting as arthralgia. Magnetic resonance imaging (MRI) showed a multicystic mass in the sellar and suprasellar regions. In the course of steroid replacement therapy for hypocortisolism, the patient's arthralgia diminished, and MRI revealed shrinkage of the mass. XH was diagnosed histologically following a transsphenoidal endoscopic biopsy, and it was the oldest case of XH. CONCLUSION: To the best of our knowledge, this patient is the oldest of reported patients diagnosed with XH. Steroid therapy may be effective to XH temporarily. XH should be considered when diagnosing pituitary cystic lesions in elderly patients with autoimmune disease.

Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma.

INTRODUCTION: Symptomatic Rathke cleft cysts (RCCs) are rarely detected in neuroradiological screening and are less commonly found in children than in adults. However, when RCCs are observed in children, it is important to carefully distinguish a RCC from a cystic craniopharyngioma (CP) even if surgically treated or conservatively followed up. METHODS: We conducted a retrospective review of clinical data from 11 patients with symptomatic RCCs whose ages were under 18 years and compared the data with data from 15 age- and sex-matched patients with cystic CP who were treated at our institute. RESULTS: The mean age of the patients with RCCs was 12.2 years (range, 6-18). There were six males and five females. As initial symptoms, nine patients presented with headache, while two each had impaired visual function, diabetes insipidus, and activity loss. The 14 patients with CP suffered from impaired visual function. Magnetic resonance imaging (MRI) mainly showed hyperintensity on T1-weighted images (WIs) and hypointensity on T2-WI in patients with RCC. However, patients with CP had characteristic hyperintensity on T2-WI. The average maximum diameter of the RCCs was 19.0 mm on average (range, 8-33 mm). The RCCs were thus significantly smaller than CPs (34.9 mm; range, 21-54 mm). The RCCs were usually oval or dumbbell-shaped and regular in appearance, while the larger CPs were lobular and irregular. A preoperative endocrinological evaluation revealed insufficiencies in four axes in five patients with RCC. Postoperative endocrinological status improved in three patients, remained unchanged in three, and worsened in one. The gonadotropin axis was damaged in a majority (nine) of the patients with CP preoperatively. Postoperative evaluation revealed deficits in five axes in 14 patients with CP, which is a significantly different trend than observed in patients with RCC. Eight patients underwent surgical procedures (transsphenoidal surgery (TSS) in four, craniotomy in four). Two of these patients experienced a recurrence of the cysts. One of these patients subsequently underwent two craniotomies followed by radiation and other underwent TSS. Among the three conservatively treated patients, two experienced a transient worsening of their symptoms along with cyst enlargement. However, none of the three conservatively treated patients required an operation. CONCLUSIONS: When RCCs become symptomatic in children, the most common symptom they lead to is headache. The cysts are commonly small, regular, and oval in shape. Hypointensity of cyst contents on MRI is a characteristic of RCCs, which distinguishes them from CPs. Surgical intervention can be effective and lead to the relief of symptoms without a high rate of complications. However, there seems to be a relatively high recurrence rate following surgery. Thus, if the patient's symptoms remain minor, the surgical treatment option should be used only when prudent, as the patient's symptoms may improve over time.

Midline dural filum of the sellar floor: Its relationship to the septum attachment to the sellar floor and the ossification in the sphenoid sinus.

OBJECTIVES: It is important to identify and maintain a midline orientation during endoscopic transsphenoidal surgery (ETSS) for sellar lesions to prevent critical injury to the internal carotid artery. Therefore, the preoperative neuroradiological assessment of the bony structures in the sphenoid sinus, including the septum attachment to the sellar floor and its surrounding structures, is essential. It has been reported that the midline filum of the sellar dura can function as a useful intraoperative orientation guide during ETSS. However, the relationship between the midline dural filum and the intrasinus bony structures, such as the sellar floor, the intrasinus septation and the ossification, remains unclear and the mechanisms underlying development of the midline dural filum have also not yet been explored. METHODS: This retrospective study included 160 patients undergoing ETSS to assess both the midline dural filum and the intrasinus bony structures, using video recording reviews. The intrasinus septum and the ossification in the sphenoid sinus were evaluated on the computed tomography images of the bone window. RESULTS: A midline dural filum was identified in 66 (41.3%) of 160 patients. Attachment of the septum to the sellar midline was found in 61 (39.4%) of 155 patients, after excluding 5 patients with the conchal type of sphenoid sinus, 55 (90.2%) of 61 patients with a septum on the midline and only 6 (6.4%) of the remaining 94 patients without a septum on the midline had a midline dural filum. The relationship between a midline dural filum and a septum on midline was statistically significant (p<0.001), regardless of the number of intrasphenoidal septa. In terms of the types of sphenoid sinus, the midline dural filum was predominantly detected in patients where ossification extended over the midline filum. In patients with the sellar type of sphenoid sinus, 49 (36.0%) of 136 had a midline dural filum, meanwhile, 16 (84.2%) of 19 patients with the pre-sellar type (p=0.039) and all 5 patients (100%) with the choncal type harbored a midline dural filum (p<0.001). CONCLUSION: This study clearly verified the importance of the midline dural filum in a large series and evaluated the obvious relationship between the midline dural filum and the bony structures on the sellar floor. Our results strongly suggest that, during developing of the midline dural filum, the sellar dura becomes tethered to the bony elements attached to the sellar surface, such as the septum on the midline and the ossification in both the pre-sellar and the conchal type of sphenoid sinus.

Simultaneous ventriculoperitoneal shunt removal and endoscopic third ventriculostomy for three patients previously treated for intracranial germ cell tumors more than 20 years ago.

BACKGROUND: Obstructive hydrocephalus is the most common complication of intracranial germ cell tumors (IGCTs). Although ventriculoperitoneal shunts (VPSs) were previously used for treating this condition, they have recently been replaced by endoscopic third ventriculostomy (ETV), as the latter can avoid various complications caused by a VPS and can preserve physiological cerebrospinal fluid (CSF) dynamics. Therefore, if a VPS malfunctioned in patients who had previously been treated for IGCTs, it seems reasonable to consider removing the problematic VPS, followed by ETV, as long as magnetic resonance imaging reveals third ventricle floor ballooning and the cause of obstruction in the ventricular system, which are indicators for ETV. CASE PRESENTATION AND DISCUSSION: Here, we present the cases of three patients who underwent simultaneous removal of malfunctioning VPSs and ETV, more than 20 years (range 20-27 years) after initial treatment for IGCT and relevant obstructive hydrocephalus. Despite a relatively long shunt dependency, their obstructive hydrocephalus was well-managed by ETV, probably because CSF absorptive capacity remained intact after IGCT ablation by radio-chemotherapy. CONCLUSION: Patients with previously treated IGCTs can thus be released from the need for a VPS, regardless of long-term shunt dependency.

Significant improvement of intractable headache after transsphenoidal surgery in patients with pituitary adenomas; preoperative neuroradiological evaluation and intraoperative intrasellar pressure measurement.

OBJECT: Headache is the most common symptom of both primary and metastatic brain tumor, and is generally considered the primary symptom in patients with large pituitary adenomas. However, patients with small pituitary adenomas rarely complain of intractable headache, and neurosurgeons are unsure whether such small adenomas actually contribute to headache. If conventional medical treatments for headache prove ineffective, surgical removal of the adenoma can be considered as an alternative management strategy. METHODS: We conducted a retrospective review of 180 patients who underwent transsphenoidal surgery (TSS) for pituitary adenomas at Kanazawa University Hospital between 2006 and 2014. Patients with acute phase intratumoral hemorrhage were excluded. We identified nine patients with intractable headache as the chief complaint associated with small pituitary adenoma (diameters 15.8 ± 2.6 mm, 11-20 mm), non-functioning in eight, and prolactin-secreting in one. The preoperative neuroradiological studies and headache characteristics were assessed retrospectively, and the intrasellar pressure evaluation was performed during TSS in the last seven patients. RESULTS: All nine patients had complete or substantial resolution of their formerly intractable headache after TSS. Headaches consisted of ocular pain ipsilateral to the adenoma localization within the sella in four cases and bifrontal headache in five. Magnetic resonance imaging of these patients revealed small diaphragmatic foramen, which were so narrow that only the pituitary stalk could pass. Computed tomography scans showed ossification beneath the sellar floor in the sphenoid sinus, presellar type in six cases, and choncal type in three. The adenomas included cysts in seven cases. There was no cavernous sinus invasion. Intrasellar pressure measurements averaged 41.5 ± 8.5 mmHg, range 34-59, significantly higher than in control patients without headache (n = 12), namely 22.2 ± 10.6 mmHg (16-30). CONCLUSION: In this study, the authors demonstrated the validity of TSS in the treatment of intractable headache associated with pituitary adenoma. The presence of ocular pain, especially ipsilateral to the adenoma, integrity of the diaphragm sella, and ossification in the sphenoid sinus, cyst or hemorrhage and the absence of cavernous sinus invasion were the indications for TSS for patients complaining of intractable headache and having pituitary adenomas.

Massive Hemorrhage from the Posterior Ethmoidal Artery during Transsphenoidal Surgery: Report of 2 Cases.

Nasal bleeding is a major complication that can occur during and after transsphenoidal surgery (TSS) for intra- and suprasellar tumors. In most cases, the cause of this bleeding can be attributed to a branch of the maxillary artery called the sphenopalatine artery, injury to which can lead to life-threatening situations. Upon exposure of the suprasellar region and planum sphenoidale during surgery, it is also important to avoid damaging the posterior ethmoidal artery (PEA), a branch of the ophthalmic artery. While recent advancement in endoscopic techniques enables the performance of extended TSS, the chances of PEA injury seem to be increasing. In the current report, we present two cases that showed massive PEA bleeding during regular (not extended) TSS. The total blood loss was 2280 ml and 2150 ml, and endoscopic views disturbed by the massive hemorrhages remarkably delayed accurate stanching of the responsive artery. Therefore, anatomical recognition of the PEA is required to avoid fatal hemorrhaging during even regular TSS, especially for the beginners of this surgery.

Pure Lymphocytic Infundibuloneurohypophysitis Caused by the Rupture of Rathke's Cleft Cyst: Report of 2 Cases and Review of the Literature.

The major symptoms that are caused by Rathke's cleft cysts (RCCs) are visual disturbances, headaches, and endocrine insufficiencies. Among these symptoms, the endocrine insufficiencies are thought to result from the spreading of inflammation that is induced by the cyst contents onto the pituitary gland or the compression of the gland and the pituitary stalk by RCCs. Here, we present 2 rare cases with lymphocytic infundibulohypophysitis with the sudden onset of headaches and subsequent diabetes insipidus (DI). Magnetic resonance imaging revealed remarkable swelling of the pituitary gland with a small mass that was located between the anterior and the posterior lobe of the pituitary gland. Transsphenoidal surgery was performed to remove the mass, and pathological examinations of the cyst wall demonstrated that the epithelial tissue of the RCC and the posterior lobe were affected by massive lymphocytic infiltration. The clinical courses and pathological results of these patients strongly suggested that the rupture of the RCC onto the posterior lobe caused the lymphocytic hypophysitis. Postoperatively, the DI could be controlled with a smaller amount of anti-diuretic hormone replacement compared to that required preoperatively.

Symptomatic foramen of Magendie arachnoid cyst in an elderly patient.

BACKGROUND: Arachnoid cysts (ACs) are congenital anomalies of the central nervous system and arise in virtually all locations where the arachnoid membrane exists. Posterior fossa ACs are considered to develop in the posterior aspect of the rhombencephalic roof plate and do not communicate freely with the fourth ventricle or perimedullary subarachnoid space. Although posterior fossa ACs have been reported a number of times, ACs arising from the foramen of Magendie are very rare. CASE DESCRIPTION: We report here on a 76-year-old female who presented with progressive gait disturbance. Magnetic resonance imaging of the head showed a large AC in the foramen of Magendie that was compressing the inferior vermis and medial aspects of the cerebellar hemisphere without causing hydrocephalus. Neurological examination revealed cerebellar ataxia without Romberg's sign. A nearly total excision of the cyst was safely performed via a median suboccipital approach. The patient's postoperative course was excellent and her neurological recovery was remarkable. CONCLUSION: Most cases of ACs located in the foramen of Magendie are reported in children, and it is extremely rare to observe such ACs in the elderly. In fact, to our knowledge, a symptomatic foramen Magendie AC has never been reported previously in an elderly person. Our results indicate that proper surgical intervention can yield highly positive outcomes in such cases.