Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyond Tolosa-Hunt syndrome - a case report.

BACKGROUND: The term "Tolosa-Hunt syndrome" (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of isolated CNS Rosai-Dorfman disease involving the cavernous sinus and presenting as "Tolosa-Hunt syndrome". CASE PRESENTATION: Our patient presented with horizontal diplopia due to impairment of cranial nerves III, IV and VI and a stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. There was a nonspecific enlargement of the left cavernous sinus on MRI and the patient had a dramatic response to steroids. Biopsy of a frontal meningeal lesion was compatible with RDD. CONCLUSIONS: We highlight the importance of including Rosai-Dorfman disease as a differential diagnosis in cavernous sinus syndrome and demonstrate a satisfactory long-term response to steroid treatment in this disease.

Drainage outflow restriction as a parameter associated with cortical venous reflux in craniofacial arteriovenous malformations with cavernous sinus drainage.

PURPOSE: Craniofacial arteriovenous malformations (CF-AVMs) are locally aggressive extracranial lesions. When CF-AVMs involve cavernous sinus (CS) as their draining vein, they represent a special subgroup which may interfere intracranial venous system. In this study, we aimed to analyze the venous drainage patterns of CF-AVMs with CS drainage and to demonstrate how it affected our treatment strategy. METHODS: Cases of CF-AVMs associated with CS drainage were collected from a prospectively collected database of patients with CF-AVMs who underwent endovascular treatment from September 2016 to March 2018. Clinical data and angioarchitectural findings were analyzed. Factors associated with the presence of venous reflux (cortical venous reflux (CVR) or dural sinus reflux (DSR)) were analyzed. RESULTS: Fifteen CF-AVM patients associated with CS drainage were analyzed. Three cases of venous reflux from the CS were identified (CVR, 2; DSR, 1). Lesions with unilateral venous drainage, ≤ 2 draining veins, and the absence of antegrade CS outflow were more likely to develop venous reflux from the CS. We successfully performed additional trans-venous coil embolization of the superior ophthalmic vein in two patients with malformations associated with venous reflux to close this venous connection to the CS. CONCLUSION: CF-AVMs associated with CS drainage confer an increased risk of CVR and DSR, especially in cases where the drainage outflow is restricted. Identification of this venous angioarchitecture is essential in the evaluation and treatment planning of CF-AVMs.

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.

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review.

BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS) is a rare condition characterized by connective tissue fragility. Direct spontaneous carotid-cavernous fistula (sCCF) is reportedly pathognomonic of vEDS. We conducted this study to understand the possible mechanisms of occurrence of sCCF in this subset of patients. METHODS: We conducted a retrospective analysis of a monocentric vEDS cohort along with a literature review regarding sCCF in this condition. RESULTS: Of 133 patients regularly followed in our centre between 2000 and 2017, 13 (9.8%) had a diagnosis of direct sCCF (92.3% female, median age 33.0 years, interquartile range (IQR) [26.0-39.5]). There were 7 Glycine missense and 6 splice-site variants but no variant leading to haploinsufficiency. The literature search identified 97 vEDS patients with direct sCCF (79.4% female, 7.2% sex not reported, median age 31.0 years, IQR [24.0-39.0]). Increased carotid circumferential wall stress, higher carotid distensibility and lower carotid intima-media thickness could contribute to a higher risk for direct sCCF in vEDS. There is no predictive factor for the occurrence of sCCF apart from female sex in vEDS. CONCLUSIONS: In vEDS, anatomical and pathophysiological features of the intra-cavernous internal carotid artery make it prone to shunting in the cavernous sinus, due either to a spontaneous rupture or to a spontaneous dissection with pseudoaneurysm formation. Direct sCCF in seemingly healthy young individuals should be highly suggestive of vEDS and prompt further investigation.

Caroticocavernous Fistula: Successful Reversal by Endovascular Treatment.

A 22-year male presented in outpatient department with right sided proptosis and chemosis, after a roadside accident two months back. He was on treatment for orbital cellulitis and taking steroids with antibiotics, both orally and topically. He was also on topical antiglaucoma agents. Detailed examination revealed bruit on auscultation. On magnetic resonance imaging (MRI), dilated superior ophthalmic vein led to diagnosis of caroticocavernous fistula (CCF) and the patient was referred to Neurosurgery Department of Lahore General Hospital (LGH), Lahore, where digital subtraction angiography (DSA) and balloon catheterization by a radiologist in the same sitting, resulted in successful closure of fistula and amelioration of all the signs and symptoms within six weeks.

Factors Associated with Abducens Nerve Recovery in Patients Undergoing Surgical Resection of Sixth Nerve Schwannoma: A Systematic Review and Case Illustration.

BACKGROUND: Limited or no literature exists identifying factors associated with functional nerve recovery in patients undergoing resection of sixth cranial nerve (CN VI) schwannomas. METHODS: A systematic review of literature was performed on CN VI schwannomas that were treated surgically. Synthesizing the findings pooled from the literature, we investigated associations of patient demographics and clinical characteristics with postsurgical CN VI functional recovery in multivariable regression models. In addition, we present the findings of an adolescent woman surgically managed for intracavernous CN VI schwannoma. Complete encasement of the cavernous segment of the internal carotid artery is unique to our case. RESULTS: We synthesized data of 32 patients from 29 studies, and our index case. Overall, the mean age of the patients was 44.0 ± 16.5 years, and approximately 52% (n = 17) were female. Most tumors were left-sided (n = 18; 54.5%), with an average size of 3.46 ± 1.71 cm. The most common location was cisternal (n = 11; 33%), followed by cavernous sinus (CS) proper (n = 9; 27%), cisterocavernous (n = 8; 24%), orbital (n = 4; 12%) and caverno-orbital (n = 1; 3%). CN VI recovery was reported in less than half the cohort (n = 14; 45%). Tumor extension in the CS was significantly associated with lesser likelihood (odds ratio [OR], 0.07; 95% confidence interval [CI], 0.01-0.98; P = 0.048) of postsurgical CN VI recovery. Although female gender (OR, 0.86; 95% CI, 0.07-10.09; P = 0.906), large tumor size (>2.5 cm) (OR, 0.45; 95% CI, 0.07-2.89; P = 0.397), and solid consistency (OR, 0.37; 95% CI, 0.03-4.19; P = 0.421) were associated with lesser odds for recovery, these were not statistically significant. Likewise, although gross total resection (OR, 6.28; 95% CI, 0.33-118.25; P = 0.220) was associated with higher odds of nerve recovery, the estimates were statistically insignificant. CONCLUSIONS: CS involvement is associated with lesser odds for functional nerve recovery in patients undergoing surgical resection for CN VI schwannoma.

Cavernous sinus syndrome: A prospective study of 73 cases at a tertiary care centre in Northern India.

OBJECTIVES: To study the clinical and etiological profile of patients with cavernous sinus syndrome (CSS) and identify factors which could determine the etiology and influence the outcome of these patients. PATIENTS AND METHODS: This prospective observational study included 73 consecutive patients satisfying the criteria of CSS (i.e. involvement of any 2 of the 3rd, 4th, 5th and 6th cranial nerves or any one of them with radiological evidence of cavernous sinus involvement). All these patients were subjected to detailed haematological, biochemical and radiological investigations and diagnosed and treated as per guidelines. The clinical and investigational data was recorded and analysed meticulously. RESULTS: A definitive etiological diagnosis of CSS could be achieved in 86% of patients. Tumours, fungal infections and Tolosa Hunt syndrome (THS) were most common causes. On univariate analysis, diabetes, severe vision loss (visual acuity of <3/60 in at least one eye), and presence of nasal discharge showed a significantly positive association with a fungal CSS. Evidence of paranasal sinusitis, bone erosion and ICA (internal carotid artery) involvement on Gadolinium enhanced MRI (magnetic resonance imaging) of brain were significantly associated with a fungal CSS (p=0.0001), whereas involvement of orbital apex had a negative association with a neoplastic etiology (p=0.014). On multiple logistic regression, orbital apex involvement on MRI was associated with diagnosis of THS (p=0.019, OR: 18.7; 95% CI: 1.6-217.4) while MRI evidence of paranasal sinusitis (p=0.014, OR: 45; 95% CI: 2.1-94.3) and bone erosion ((p=0.019, OR: 12.5; 95% CI: 1.5-103) correlated with diagnosis of fungal CSS. 65.2% of patients (fungal CSS- 70%) had a good prognosis at six months follow up. CONCLUSION: Most patients with CSS can be diagnosed accurately and managed properly with good outcomes.

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.

Spontaneous angiographic changes in venous drainage patterns related to symptom changes in patients with untreated cavernous sinus dural arteriovenous fistula.

INTRODUCTION: We evaluated the relationship between symptomatic and angiographic changes in untreated cavernous sinus dural arteriovenous fistulas (CSdAVFs), focusing on venous drainage patterns. METHODS: The clinical and radiologic features of 34 cases of untreated CSdAVF were retrospectively reviewed. We classified venous drainage patterns as type I (only antegrade drainage), type II (combined antegrade drainage and venous reflux), type III (venous reflux without antegrade drainage), or type IV (stasis or occlusion of venous reflux). Symptom changes were categorized as improvement, aggravation of initial symptoms, or symptom pattern change. RESULTS: Twenty-one patients (61 %) showed symptom changes during follow-up (median, 12; range, 3-151 months). In the symptom improvement group (n = 10), patients who underwent follow-up angiography (n = 4) exhibited spontaneous occlusion. In the symptom aggravation group (n = 4), new venous reflux developed in 2 patients (type I to type II) and spontaneous occlusion in 2 patients (type III to spontaneous occlusion). In the symptom pattern change group (n = 7), 2 patients showed new venous reflux (type I to type II), and 5 showed stasis or occlusion of an engorged ophthalmic vein (type II or III to type IV). Angiographic regression was observed in all type III and IV patients, and cortical venous reflux (CVR) developed in 1 type I patient. CONCLUSION: Symptom changes correlated with chronological angiographic changes. Without treatment, most CSdAVFs behaved benignly and had a low incidence of CVR. Therefore, close observation is a possible protocol for managing CSdAVFs that have tolerable symptoms, no CVR, and no antegrade drainage despite aggravation or fluctuation in symptoms.

Cavernous sinus syndrome: need for early diagnosis.

Cavernous sinus syndrome (CSS) is a rare condition characterised by ophthalmoplegia, proptosis, ocular and conjunctival congestion, trigeminal sensory loss and Horner's syndrome. These signs and symptoms result from the involvement of the cranial nerves passing through the cavernous sinus. We report the case of a 53-year-old man with a history of daily stabbing headache associated with dizziness, progressive blurred vision, right ocular pain, ptosis and ophthalmoplegia. After working up the patient, a meningioma was identified as the cause of the CSS. Despite advances in neuroimaging techniques, in some cases, the aetiology of CSS remains difficult to determine. We highlight the clinical and radiological features of a meningioma, one of the causes of CSS. Early diagnosis and treatment of CSS play a key role in a better prognosis.

[Cluster-like headache cause by cavernous sinus dural arteriovenous fistula, cured by transvenous embolization: a case report].

We report a unique case of cavernous sinus(CS)-dural arteriovenous fistula(dAVF)with cluster headache-like ophthalmalgia without chemosis and exophthalmos, that was successfully treated by transvenous embolization. A 25-year-old man presented with severe right cluster headache-like ophthalmalgia and could not get complete relief of symptoms by medical treatment. MRI showed a hyper-intensity signal in the right cavernous sinus and cerebral angiography revealed Barrow type D cavernous sinus dural arteriovenous fistula, which is thought to be the cause of ophthalmalgia. Percutaneous transvenous embolization of the anterior part of the right CS and superior ophthalmic vein was performed and complete obliteration of dAVF was obtained followed by complete cure of cluster headache-like ophthalmalgia. We can detect neither the recurrence of dAVF on MRI nor any clinical symptom after 23 months of the treatment. This is the first clinical presentation of cluster headache-like ophthalmalgia caused by CS dAVF and it emphasizes the unique and important potential relationship between them.

Superior petrosal sinus: hemodynamic features in normal and cavernous sinus dural arteriovenous fistulas.

BACKGROUND AND PURPOSE: Normal hemodynamic features of the superior petrosal sinus and their relationships to the SPS drainage from cavernous sinus dural arteriovenous fistulas are not well known. We investigated normal hemodynamic features of the SPS on cerebral angiography as well as the frequency and types of the SPS drainage from CSDAVFs. MATERIALS AND METHODS: We evaluated 119 patients who underwent cerebral angiography by focusing on visualization and hemodynamic status of the SPS. We also reviewed selective angiography in 25 consecutive patients with CSDAVFs; we were especially interested in the presence of drainage routes through the SPS from CSDAVFs. RESULTS: In 119 patients (238 sides), the SPS was segmentally (anterior segment, 37 sides; posterior segment, 82 sides) or totally (116 sides) demonstrated. It was demonstrated on carotid angiography in 11 sides (4.6%), receiving blood from the basal vein of Rosenthal or sphenopetrosal sinus, and on vertebral angiography in 235 sides (98.7%), receiving blood from the petrosal vein. No SPSs were demonstrated with venous drainage from the cavernous sinus. SPS drainage was found in 7 of 25 patients (28%) with CSDAVFs. CSDAVFs drained through the anterior segment of SPS into the petrosal vein without draining to the posterior segment in 3 of 7 patients (12%). CONCLUSIONS: The SPS normally works as the drainage route receiving blood from the anterior cerebellar and brain stem venous systems. The variation of hemodynamic features would be related to the relatively lower frequency and 2 different types of SPS drainage from CSDAVFs.

Tolosa-Hunt syndrome in a patient with autoimmune hemolytic anemia.

Tolosa-Hunt syndrome is a steroid responsive painful opthalmoplegia due to a nonspecific inflammation of the cavernous sinus. Autoimmune hemolytic anemia is caused by antibodies directed against unmodified autologous red cells. They are both rare conditions. Here we describe the simultaneous occurrence of Tolosa-Hunt syndrome and severe hemolytic crisis in the same patient.

Gamma knife surgery for cavernous hemangiomas in the cavernous sinus.

BACKGROUND: Cavernous hemangioma in the cavernous sinus (CS) is a rare vascular tumor. Direct microsurgical approach usually results in massive hemorrhage. Radiosurgery has emerged as a treatment alternative to microsurgery. OBJECTIVE: To further investigate the role of Gamma Knife surgery (GKS) in treating CS hemangiomas. METHODS: This was a retrospective analysis of 7 patients with CS hemangiomas treated by GKS between 1993 and 2008. Data from 84 CS meningiomas treated during the same period were also analyzed for comparison. The patients underwent follow-up magnetic resonance imaging at 6-month intervals. Data on clinical and imaging changes after radiosurgery were analyzed. RESULTS: Six months after GKS, magnetic resonance imaging revealed an average of 72% tumor volume reduction (range, 56%-83%). After 1 year, tumor volume decreased 80% (range, 69%-90%) compared with the pre-GKS volume. Three patients had > 5 years of follow-up, which showed the tumor volume further decreased by 90% of the original size. The average tumor volume reduction was 82%. In contrast, tumor volume reduction of the 84 cavernous sinus meningiomas after GKS was only 29% (P < .001 by Mann-Whitney U test). Before treatment, 6 patients had various degrees of ophthalmoplegia. After GKS, 5 improved markedly within 6 months. Two patients who suffered from poor vision improved after radiosurgery. CONCLUSION: GKS is an effective and safe treatment modality for CS hemangiomas with long-term treatment effect. Considering the high risks involved in microsurgery, GKS may serve as the primary treatment choice for CS hemangiomas.

Neuroendovascular management of carotid cavernous fistulae.

Carotid-cavernous fistulae are abnormal arterial communications within the cavernous sinus. Endovascular obliteration is the mainstay therapy for the definitive treatment of these lesions. Several approaches have been described. These include transarterial or transvenous embolization with balloons, coils, particles, or covered stents, and arterial sacrifice. The preferred technique is based on the fistula type (direct or indirect), microanatomy, chronicity, and the extent of the arterial defect.

Cavernous sinus thrombophlebitis caused by porphyromonas gingivalis with abscess formation extending to the orbital cavity. Case report.

A 67-year-old man presented with a rare case of cavernous sinus thrombophlebitis (CST) caused by Porphyromonas gingivalis with abscess formation extending to the orbital cavity. Neuroimaging demonstrated a cystic lesion in the right cavernous sinus that was hyperintense on diffusion-weighted imaging. The patient was successfully treated with surgical drainage and antibiotic administration. CST is rare and often has a fulminant progression with high rates of morbidity and mortality. The differential diagnosis of cavernous sinus lesions should include CST. Early recognition and differentiation from other diseases with aggressive medical and possible surgical intervention are necessary to reduce mortality and long-term sequelae. Diffusion-weighted imaging is useful for the early recognition and differentiation of CST from other diseases.