Leo Koornneef

What is orbital pseudotumor

We have reviewed the literature in order to delineate the clinicopathologic definition of orbital pseudotumor, also called idiopathic nonspecific orbital inflammation. The clinical picture of orbital pseudotumor varies widely, with signs of mass effect, inflammation and/or infiltration. On computed tomography, orbital pseudotumor presents as a unilateral focal or diffuse mass. The histopathologic hallmark of orbital pseudotumor is a mixed inflammatory infiltrate with fibrosis of varying degree. Contrary to an old belief, orbital pseudotumor is not related to orbital reactive lymphoid hyperplasia (pseudolymphoma) and is not a lymphoid tumor. Atypical histopathologic findings of orbital pseudotumor include dominant sclerosis, granulomatous inflammation, vasculitis, and tissue eosinophilia. In the absence of systemic fibroinflammatory, granulomatous, and vasculitic disease, these atypical histopathologic patterns can be considered to represent subclasses of orbital pseudotumors rather then distinct entities. Clinical and prognostic characteristics of both histopathologically classical and atypical orbital pseudotumors appear to be heterogeneous. The etiology of orbital pseudotumor is unknown, but infection, autoimmune disorder, and aberrant wound healing have all been put forward as possibilities. In conclusion, orbital pseudotumor is one distinct disease albeit with many clinical and histopathologic guises.

Optic nerve sheath meningiomas.

PURPOSE To study the natural history and growth of optic nerve sheath meningiomas and evaluate their management outcome. DESIGN Clinicopathologic retrospective noncomparative case series. METHODS A retrospective study of 88 patients who were treated between 1976 and 1999 at the University of British Columbia and the University of Amsterdam. Clinical reports, imaging studies, and histopathologic findings were reviewed. RESULTS The mean age at onset of symptoms was 40.3 years, and most were seen in middle-aged females. Patients typically presented with visual loss, frequently associated with optic atrophy or papilledema and occasionally optociliary shunt vessels. On imaging, the optic nerve demonstrated segmental or diffuse thickening of the sheath or globular growth. Calcification was seen in 31% of cases and was associated with slower tumor growth. Tumors with posterior components in the orbit had more frequent intracranial involvement. Intracranial extension was more frequent and had a greater growth rate in younger patients. Irregular margins in the orbit implied local invasion. A presenting visual acuity better than 20/50 correlated with longer preservation of vision. Patients who underwent radiotherapy showed improvement in their visual acuity, and tumor growth was halted. Optic sheath decompression did not preserve vision. En bloc tumor excision was associated with no detectable recurrence in contrast to debulked tumors that recurred. CONCLUSIONS Meningiomas show characteristic indolent growth. Management therefore should be conservative in most cases. Radiotherapy is indicated in patients with progressive visual deterioration. Surgery, when indicated, should be an en bloc excision.

Are Systemic Corticosteroids Useful in the Management of Orbital Pseudotumors

PURPOSE Systemic corticosteroids are the established primary treatment for orbital pseudotumors, but patients with recurrent and refractory pseudotumors commonly are observed. In this retrospective study, the authors aimed to assess the value of corticosteroids in the management of orbital pseudotumor. METHODS The clinical notes, computed tomography scans, and histologic specimens of patients with orbital pseudotumor seen at the Orbital Center, Amsterdam, between 1976 and 1994 were analyzed with particular regard to the clinical and visual outcome. The patients were categorized according to the anatomic location of the pseudotumor within the orbit. Patients with pseudotumors restricted to the lacrimal gland or an extraocular muscle were excluded. RESULTS The authors studied 32 patients with orbital pseudotumor, 20 of whom had histopathologic confirmation. Twenty-seven of these 32 patients received systemic corticosteroids as an initial treatment. Of these 27 patients, 21 (78%) showed an initial response and 10 (37%) obtained a cure. After the initial response, 11 (52%) of the 21 patients recurred. Optic nerve involvement was present in 13 (41%) of the 32 patients, and all except 1 patient recovered with corticotherapy. The mean follow-up of therapy was 4.3 years (range, 5.3 months-21.5 years). CONCLUSION The authors found a moderate response, a high recurrence, and a low cure rate in patients treated with corticosteroids for orbital pseudotumor, and they therefore challenge the value of corticosteroids in both diagnosing and treating this condition. However, in patients with pseudotumor-induced optic neuropathy, corticosteroids remain of value.

Orbital septa: anatomy and function.

As a consequence of some puzzling motility disturbances in patients with blow-out fractures, a new anatomic approach to the human orbit was developed. This new approach revealed unknown connective tissue septa inside the orbit. These connective tissue septa were highly organized and formed an accessory locomotor system. This system is involved in pathologic circumstances like blow-out fractures and can account for the motility disturbances in these cases; additionally, it plays an important, yet to be unravelled, role when normal eye movements are performed.

Current Status in the Treatment of Orbital Myositis

PURPOSE Orbital myositis is described as exquisitely sensitive to therapy with systemic corticosteroids. However, it may turn into a recurrent or chronic disease, requiring repeated courses or maintenance of corticotherapy that often is complicated with serious side effects. The authors conducted this study to evaluate their experience and the current status in the literature as to treatment of orbital myositis with corticosteroids and corticosteroid-sparing alternatives. METHODS The clinical records and computed tomography scans of patients with orbital myositis who presented at the Orbital Center Amsterdam between 1977 and 1991 were studied for the clinical course and therapeutic outcome. Recurrences were defined as a new attack of the same or another extraocular muscle as the primary affected, in the same or the other orbit. RESULTS The study group comprised 16 patients. All patients responded to initial treatment with oral corticosteroids (14/16) or nonsteroidal anti-inflammatory drugs (NSAID) (2/16). Of these 16 patients, 9 (56%) had 1 or more recurrences. Recurrences responded to repeated courses of corticosteroids, NSAID, or 20 Gy irradiation. Despite radiotherapy in six patients, recurrences continued to occur within a mean period of 2.7 years (range, 0.5 month-8.8 years). The mean follow-up after initial therapy in general was 9.7 years (range, 2.6-16.9 years), and after radiotherapy, in particular, was 7.4 years (range, 4.1-10.5 years). CONCLUSIONS Orbital myositis responds well to oral corticosteroids, but recurs in 50% of the cases. Based on a long-term follow-up of six patients who received irradiation of 20 Gy, radiotherapy appears ineffective in stopping recurrent orbital myositis.

Temporal relationship between onset of Graves’ ophthalmopathy and onset of thyroidal Graves’ disease

The temporal relationship between the onset of Graves’ ophthalmopathy and the onset of thyroidal Graves’ disease was evaluated in 125 consecutive patients with Graves’ ophthalmopathy. Thyroidal Graves’ disease — past or present — was clinically evident in 99 patients (79%): hyperthyroidism in 96 and hypothyroidism in 3 cases. Thyroid disease preceded the eye disease in 37 patients, it occurred simultaneously with the eye disease in 39 patients, and it developed after the eye disease in 23 patients (in 16 cases within one yr after the onset of eye disease). The age at the onset of thyroid disease (38.7 ± 12.9 yr) was lower than the age at the onset of ophthalmopathy (41.8 ± 12.5 yr; p< 0.001). Among the 26 clinically euthyroid patients (21%) laboratory evidence of thyroidal Graves’ disease was found in 14 cases (11%): abnormal TRH test, n = 9; normal TRH test but abnormal T3-suppression test, n = 4; normal TRH and T3-suppression tests but positive thyroid stimulating antibodies, n = 1). We conclude that Graves’ ophthalmopathy as a rule develops at a time when thyroid autoimmunity also exists. This strongly suggests a common factor in the pathogenesis of thyroidal and ocular expressions of Graves’ disease.

Results of radiotherapy in patients with stage I orbital non-Hodgkin's lymphoma

This paper describes the results of radiotherapy in early stage orbital non-Hodgkins lymphoma. From 1970 to 1985, 33 orbital localizations in 30 patients were treated. The total dose applied ranged from 21 to 57 Gy (2 Gy per fraction), two-thirds of all patients received a dose of 40 Gy. The complete-response rate was 94% and the 10 years actuarial survival was 90%; no significant difference in survival was observed between patients with low grade or intermediate grade lymphoma. No local recurrence was detected during follow up and 20% of the patients developed generalized disease. Two optic nerve neuropathies and three retinopathies were observed in five patients, four of these occurred at a dose level of less than 43 Gy. Keratitis occurred in 58% of the patients treated, a sicca syndrome in 30% and cataract of different grades in 58% of the patients treated. Although local control was excellent, severe complications were observed in 13% of the patients who received a dose of less than 43 Gy.

A new ultrasonographic method to detect disease activity and predict response to immunosuppressive treatment in Graves ophthalmopathy

Purpose: To determine whether the internal reflectivity of the eye muscles on Amode ultrasonography serves as indicator for disease activity in Graves ophthalmopathy, thereby predicting response to immunosuppressive treatment. Methods: Eye muscle reflectivity, expressed as percentage of the initial scleral spike, was measured in the superior, medial, and lateral recti, and normal values were determined in 23 healthy controls. These were compared with values in 16 consecutive patients with untreated, moderately severe Graves ophthalmopathy. The accuracy of the measurement was assessed by calculating the intra- and interobserver coefficient of repeatability. Baseline eye muscle reflectivity in the 16 patients was correlated to the therapeutic outcome 24 weeks after start of immunosuppressive therapy. The response to treatment served as indicator for pretreatment disease activity. Results: Eye muscle reflectivity could be measured reliably, with an intraobserver coefficient of repeatability of 8.2% in controls and 10.2% in patients. Patients had lower reflectivity than controls (54.6 ± 17.4% versus 70.7 ± 6.6%; P P = 0.02). From the individual data, a cut-off value of 40% was derived, which appeared as an accurate predictor of outcome (positive predictive value 73%; 95% confidence interval 39%–94%). Conclusions: Measuring eye muscle reflectivity in Graves ophthalmopathy appears to be a reliable new method to determine disease activity, with a promising accuracy in predicting therapeutic outcome of immunosuppressive treatment.

Coronal approach for rehabilitative orbital decompression in Graves' ophthalmopathy

AIMS To determine the effectiveness and safety of three wall orbital decompression by the coronal approach in Graves’ ophthalmopathy. METHODS The records of 125 patients with Graves’ ophthalmopathy, who had undergone three wall orbital decompression by coronal approach between April 1984 and October 1993, were studied retrospectively. Special attention was paid to proptosis reduction, changes in ocular motility, and complications. RESULTS The preoperative Hertel values ranged from 15 to 30 mm (mean 22.43 mm). The mean proptosis reduction was 4.34 mm (range 0–10 mm). Proptosis reduction in patients with preoperative Hertel values higher than 27 mm was significantly more than in patients with preoperative values between 25 and 27 mm (p < 0.05). This last group showed significantly more proptosis reduction than patients with preoperative Hertel values of 23 and 24 mm (p < 0.01). Postoperatively, 3.2% of the patients showed new diplopia in primary and/or reading position. In 4% of the patients with normal ocular motility preoperatively, diplopia in the extreme directions of gaze developed. In 4% of the patients, preoperative motility disturbances decreased or disappeared postoperatively. CONCLUSION Three wall orbital decompression by coronal approach is a safe and effective technique, to achieve proptosis reduction in patients with Graves’ ophthalmopathy, with fewer complications than other techniques thus far described.

Measuring Eye Movements in Graves Ophthalmopathy

Background: The current grading of class IV (extraocular muscle involvement) of the NO SPECS classification for Graves ophthalmopathy is subjective and, in practice, virtually limited to three grades (grade c rarely occurs). The subjectiveness results in a high degree of observer disagreement, as illustrated by kappa values of 0.48 to 0.64 for the agreement between the authors in grading of class IV changes in 13 patients. Methods: The authors investigated the significance of a quantitative approach in assessing eye motility and modified a hand perimeter to measure the monocular eye movements in degrees. Results: The mean maximal excursions in the four main directions of gaze, obtained with this device in 40 healthy control subjects (5 men and 5 women from each decade between 20 and 60 years of age), were as follows: elevation=34°, depression=58°, adduction=48°, and abduction=46°. The ductions showed an age-related decline. The intraobserver and interobserver variations were assessed in 12 and 18 patients with Graves ophthalmopathy, respectively, by calculating the coefficients of repeatability, which varied from 5° to 8°. The authors compared the current grading of NO SPECS class IV with quantitative measurements of elevation in 11 patients by calculating the kappa values for agreement. These were found to be 0.57 and 0.80, respectively. Conclusions: The quantitative measurement of eye movements with a modified perimeter appears to be precise and reproducible. In addition, it should replace the current grading of the extraocular muscle movements of Graves ophthalmopathy patients in the NO SPECS classification, which is less reliable, as evidenced by a higher degree of observer disagreement.