Dural carotid cavernous sinus fistula: ocular characteristics, endovascular management and clinical outcome.

OBJECTIVE: To describe the ocular findings, endovascular treatment, and clinical outcome in patients with dural carotid cavernous sinus fistula (CCF). MATERIAL AND METHOD: A retrospective evaluation of 80 consecutive patients who underwent examination and treatment for dural CCF between January 1997 and December 2004 was performed. RESULTS: Fifty females and 30 males, with an average age of 49 years (from 6 -80 years) participated in this study. All patients had more than one clinical signs and symptoms including proptosis (84%), arterialization of conjunctival vein (93%), chemosis (42%), cranial nerve palsy (52%), elevated intraocular pressure (51%), and optic neuropathy (13%). Diminished vision was found in 43% of the patients. The degree of visual deficit ranged from 20/40 to no light perception. After angiographic evaluation, patients were classified to CCF Barrow's type B 14%, type C 15%, and type D 71%. Endovascular treatment by transvenous and/or transarterial embolization was performed in 60 patients (75%). Carotid-angular compression therapy was solely performed in 19 patients (24%) and was used as an adjunct to endovascular treatment in 30 patients (38%). The follow-up period ranged from 6 to 94 months. Clinical cure was achieved in 41 patients (51%) and improvement in 30 patients (38%). Anatomical cure was demonstrated by angiogram in 50 patients (63%). Intra-operative complications were found in three patients including ophthalmic artery occlusion and cerebral infarction. Eight patients experienced transient aggravation of symptoms including increased proptosis, elevation of intraocular pressure, choroidal detachment that required suprachoroidal drainage, and venous stasis retinopathy. Ophthalmic vein thrombosis resulting in central retinal vein occlusion was developed in three patients and finally caused severe visual deficit. There was no operative mortality. CONCLUSION: Selective management with endovascular therapy and manual compression are the effective treatment for dural CCF. However sight-threatening complications can develop after therapy due to progressive ophthalmic vein thrombosis and should be carefully monitored.

Stereotactic radiation therapy for optic nerve sheath meningioma; an experience at Ramathibodi Hospital.

OBJECTIVE: To evaluate results of stereotactic radiotherapy for the treatment of optic nerve sheath meningioma (OM) at Ramathibodi Hospital. MATERIAL AND METHOD: Twelve patients with primary OM were treated with stereotactic radiation between 1998 and 2005. Five patients underwent surgery and had no light perception before radiation. All patients except one were treated with fractionated stereotactic radiotherapy (FSRT). Mean average dose of FSRT was 55. 7 Gy; 180 cGy/fraction. One patient was treated with 15-Gy stereotactic radiosurgery. RESULTS: With a median follow-up of 34 months, there was no visual improvement in the five patients who were completely blind before radiation. Visual acuity improved in four patients and remained stable in two patients. Four of six patients had improved visual field, and five of six decreased in proptosis. Follow-up images were available in six patients, showing minimal tumor regression in five and stable in one. No serious acute side effect was observed. Vision became worse in one patient, who developed vitreous hemorrhage two years after FSRT. CONCLUSION: Stereotactic radiotherapy is an effective treatment for primary OM. It provides tumor control and visual preservation with low risk of complications. However more patients and further follow-up are needed for long-term outcomes.

Four recti enlargement at orbital apex and thyroid associated optic neuropathy.

OBJECTIVE: To determine the site of muscle enlargement in thyroid-associated ophthalmopathy and optic neuropathy in Ramathibodi Hospital. MATERIAL AND METHOD: Data and images of MRI and CT of patients with thyroid-associated ophthalmopathy were retrospectively reviewed Neuro-ophthalmologic data and correlation to the imaging findings were analyzed. RESULTS: Among 19 patients with thyroid-associated ophthalmopathy, 20 imaging studies were collected. There were 4 patients with bilateral disease and crowdedness of 4 recti muscle. All of them had thyroid associated optic neuropathy. One out of 19 patients underwent two different sessions study. During her first study, right optic nerve was involved with the crowding of right optic nerve but 7 months later her left optic nerve became involved with crowding of the left optic nerve and the right 4 recti appeared smaller CONCLUSION: Four recti muscle enlargement in thyroid-associated ophthalmopathy, like four-leaf clover in coronal section of imaging study, may be an important sign of thyroid associated optic neuropathy.

POEMS syndrome with venous sinus thrombosis and visual failure: a case report.

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome is a multisystem disorder associated with plasma cell dyscrasia. Other clinical signs include clubbing of the fingers, edema, papilledema etc. Although papilledema and increased intracranial pressure are common features, their causes or pathophysiology have been uncertain. The authors report here a 16-year-old Thai patient with these features who also suffered from venous sinus thrombosis and visual failure which have never been reported before. The former is considered to be one of the possible causes of the intracranial hypertension and visual failure. MRI of the brain and optic nerve revealed enhancement and swelling of the optic nerve sheaths and optic discs. MRV findings were compatible with chronic veno-occlusive disease. Bone marrow aspiration and biopsy demonstrated an increase of aggregates of intermediate and mature plasma cells. The CSF pressure was markedly elevated. His clinical condition continued to deteriorate and he expired 3 years and 5 months from the onset of his illness. Although, overproduction of vascular endothelial growth factor has been reported and is being considered to be the possible cause of vascular hyperpermeability, the chronic venous sinus thrombosis may play an important role in the pathogenesis of intracranial hypertension and visual failure.

Retrobulbar injection of triamcinolone in thyroid associated orbitopathy.

OBJECTIVE: To identify the result of retrobulbar triamcinolone injection in patients with thyroid associated orbitopathy MATERIAL AND METHOD: Prospective noncomparative pilot study in 27 eyes of 19 patients who had been diagnosed as thyroid associated orbitopathy during October 2000 through April 2002. The patients received retrobulbar 40 mg triamcinolone treatment for each orbit weekly, totaling 4 applications. RESULTS: Three months after treatment, most of the patients demonstrated no significant change in visual acuity and visual field. Improvement of proptosis was observed in 15 eyes (56%) and stable in 10 eyes (37%). Seven patients (41%) had improvement of extraocular muscle function as demonstrated by Hess test. These results remained stable in the majority of patients at the 6 months follow up period. No systemic side effects were observed The only significant local side-effect was intraocular pressure elevation which was found in 8 eyes and responded to antiglaucoma therapy. CONCLUSION: This preliminary study demonstrated the potential benefit of retrobulbar triamcinolone injection in cases of thyroid associated orbitopathy. Long-term study in these patients is required.

Aneurysmal third nerve palsy.

To study the clinical characteristics, treatment and outcome of patients with aneurysmal third nerve palsy. Eleven patients with isolated third nerve palsy from an intracranial aneurysm from 1998 to 2002 at Ramathibodi Hospital were reviewed retrospectively. The average age was 60 years. Ipsilateral headache and/or retroorbital pain occured in 80 percent of the patients. Ten patients had unilateral disease, whereas one patient had bilateral involvement. The most common site of aneurysm was at the origin of the posterior communicating artery. Endovascular treatment with coil embolization provided successful occlusion as well as neurosurgical clipping. Recovery of third nerve function was found in all patients but had variable degrees. Patients who received early treatment, especially within 10 days after onset of oculomotor dysfunction appeared to have the best chance of recovery. Aberrant regeneration developed in 5 cases. The commonest sign was lid retraction during adduction and downward gaze.