Treatment of failed Adult Chiari Malformation decompression with CSF drainage: observations in six patients.

OBJECTIVE: We report the use of CSF drainage for the management of failed Adult Chiari Malformation (ACM) decompression. METHODS: All patients with more than one year follow-up after treatment of their failed ACM were included in this study. They underwent initial decompression between September 1998 and April 2000. Clinical and radiological data were collected initially and at recurrence. Lumbar punctures (LP) were done at recurrence for diagnostic and therapeutic purposes. Opening pressures and symptomatic relief were recorded. Therapeutic options included intermittent LP and ventriculo-peritoneal shunting (VPS). RESULTS: There were 6 patients (5 females and one male). Their age ranged from 19 to 43 years. Tonsillar descent ranged from 5 to 21 mm. The symptoms recurred 1.5 to 9 months postoperatively (average 5.6 months). Postoperative imaging revealed the presence of CSF flow behind the tonsils and the formation of a retrotonsillar neocistern in all patients. On LP, the opening pressure ranged from 17 to 31 cm of water (average 23 cm). All patients improved after CSF drainage, and four patients underwent VPS. The other patients were treated with repeat LP+/-Acetazolamide. There was significant improvement in all patients, with 18 months follow-up after CSF drainage (range 16-21 months). CONCLUSIONS: Our results suggest a role for CSF drainage in the treatment of some patients with failed ACM surgery. Possible explanations for the failure of ACM surgery in this subgroup include: surgical complications leading to neural hydrodynamic alteration, inadequate initial surgery, and coexistence with another pathology, possibly a mild form of intracranial hypertension. More prospective and hydrodynamic studies are needed to further clarify these issues.

A reappraisal of surgery for orbital tumors. Part I: extraorbital approaches.

Orbital lesions are variable in nature and location. Their management can be challenging, and surgical intervention is often needed. Although a significant percentage of these tumors are treated by the ophthalmologist alone, collaboration with a neurosurgeon is often required, especially for tumors that are located deep within the orbit, are large, or have an intracranial extension. Technical advances and modifications in surgical technique have decreased surgery-related morbidity and increased its success. The authors describe their rationale in the choice of a surgical approach, the surgical techniques for extraorbital approaches, and the new surgical adjuvants.

Surgery for orbital tumors. Part II: transorbital approaches.

Orbital tumors can be excised or biopsy samples obtained via transorbital approaches, especially those located in the anterior two thirds of the orbit. The indications and various surgical steps will be reviewed for the anterior, the anteromedial, and the lateral approaches. Some of these approaches can be combined or extended to accommodate large or deep-seated tumors.

Melkersson-Rosenthal syndrome: new clinicopathologic findings in 4 cases.

OBJECTIVE: To define the clinicopathologic features of eyelid involvement in Melkersson-Rosenthal syndrome (MRS). METHODS: Four patients with eyelid edema consistent with MRS were evaluated clinically, including diagnostic imaging in 2 patients. Eyelid tissue from these patients was examined by light microscopy and immunohistochemistry. Polymerase chain reaction for herpes simplex virus was performed in 1 case. RESULTS: The 3 men and 1 woman ranged in age from 33 to 74 years. All patients had insidious, painless, nonpitting eyelid edema. Three patients had unilateral edema; one had bilateral, asymmetric involvement. Ipsilateral lip edema was present in 1 case. Computed tomography demonstrated periorbital heterogeneous thickening that corresponded to the microscopic finding of scattered granulomas. All 4 patients demonstrated epithelioid granulomas inside and adjacent to dilated lymphatic vessels. Polymerase chain reaction testing was negative for herpes simplex virus. CONCLUSIONS: Isolated eyelid swelling that mimics thyroid-associated ophthalmopathy may occur in MRS. Computed tomography may be useful in the diagnosis. Biopsy should be performed in all cases of unexplained nonpitting eyelid edema. In the eyelid, MRS is characterized histopathologically by a granulomatous lymphangitis, a finding that seems to be unique to this condition.

The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases.

Schwannomas (neurilemomas) are benign tumors that arise from Schwann cells in the peripheral nervous system. The most commonly involved nerves that cause neuro-ophthalmic manifestations are cranial nerves V and VIII. In this series of three women, schwannomas presented as intraconal masses that mimicked a cavernous hemangioma, a superior orbital mass transgressing the superior orbital fissure, and an expansive frontal lobe mass with clinical symptoms and signs of increased intracranial pressure. Although all three complained of visual blurring, none of our patients presented with Vth or VIIIth cranial nerve dysfunction. Histopathologic studies demonstrated well-circumscribed, encapsulated spindle-cell lesions with classic Antoni A and B patterns. Histopathologic examination is essential to confirm the diagnosis of a schwannoma that may be otherwise clinically confusing. Direct optic nerve compression, globe indentation with induced hyperopia, or increased intracranial pressure with optic nerve compromise may be responsible for visual symptoms. A multidisciplinary approach is often required because of the size and location of schwannomas.

Retained stenting material: an unusual cause of dacryocystorhinostomy failure.

OBJECTIVE: To highlight a troubling cause of dacryocystorhinostomy (DCR) failure and to alert ophthalmologists to the potential problems that can result when stenting material is not removed and becomes retained after DCR. DESIGN: Consecutive noncomparative case series. PARTICIPANTS: Twelve patients who underwent revision DCR from February 1994 to January 1997. INTERVENTION: Endoscopic DCR, pre- and postoperative nasal endoscopy, preoperative computerized tomography (CT), and pre- and postoperative Jones testing. RESULTS: Fourteen revision endoscopic procedures were performed on 12 patients with recurrent epiphora following DCR. Failure was due to retained stenting material in six patients, a small bony rhinostomy in three patients, excessive scar formation within the rhinostomy in two patients, and improper location of the rhinostomy in one patient. Preoperative endoscopy and CT scan each correctly identified the retained sponge or tubing in four of six patients. CONCLUSIONS: Fastening a small sponge to Silastic tubing and positioning it within the DCR site in an attempt to retard DCR stenosis can be associated with a poor outcome and should be avoided. The nasal endoscope provided excellent visualization of pathology within the lacrimal sac and was a valuable tool. Retained stenting material should be considered in patients with persistent epiphora following DCR or intubation prior to any decision to commit a patient to permanent Jones tube placement.

Liposarcoma of the orbit: a management challenge.

A previously healthy 35-year-old man experiencing slowly progressive, painless proptosis of the right eye. Visual function was normal, but supraduction was limited. Computed tomography revealed a superior, extraconal orbital mass. Subtotal excision was performed, and a diagnosis of liposarcoma was rendered only with expert analysis. Despite subsequent orbital exenteration and postoperative radiation, a local recurrence developed 5 years later. The clinical features that predict recurrence, and management options that may promote longevity, are discussed.

The prevalence and implications of ocular hypertension and glaucoma in thyroid-associated orbitopathy.

PURPOSE: The authors determined the prevalence of ocular hypertension and its association with progression to glaucomatous damage in patients with thyroid-associated orbitopathy (TAO). METHODS: The charts of 500 consecutive patients with TAO seen at the Allegheny General Hospital (Pittsburgh, PA) between 1985 and 1995 were analyzed. The amount of proptosis, degree and duration of myopathy, exposure to corticosteroids, prior glaucoma treatment, and family history of glaucoma were evaluated. RESULTS: One hundred twenty (24%) patients with TAO were noted to have an intraocular pressure (IOP) greater than 22 mmHg but less than 30 mmHg. This ocular hypertensive group was composed of 34 men and 86 women with a mean age of 55 years and mean follow-up of 4 years. Seven patients were defined as glaucoma suspects, based on increased but nonprogressive cup-to-disc ratios or nonprogressive, atypical visual field changes in the presence of increased IOP. Two patients demonstrated progressive visual field abnormalities and cupping. Of the factors evaluated, only the duration of active orbital involvement was statistically associated with progression to glaucomatous damage. The mean duration of TAO was 3, 8, and 12 years for ocular hypertensives, glaucoma suspects, and glaucomatous damage, respectively. CONCLUSIONS: Only a prolonged duration of active TAO in association with ocular hypertension correlated with progression to glaucomatous damage. These patients with chronic TAO deserve special attention and close follow-up to prevent optic nerve damage.

Orbital disease, optic nerve and chiasm.

The diagnosis and management of diseases of the orbit, optic nerve and chiasm continue to evolve. Advances in neuroradiology are remarkable and continue to expand diagnosis and management. This review will highlight the clinical assessment of disease, orbital and neuroimaging, medical and surgical management.