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Intraosseous meningioma: a rare cause of chronic optic neuropathy and exophthalmos.

BACKGROUND: The association of optic neuropathy, proptosis and chorioretinal folds is highly suggestive of an orbitopathy. Usual etiologies include an inflammatory orbitopathy, optic nerve sheath meningioma, sphenoid wing meningioma, exterioration of a sinus disorder, or an orbital tumor be it primary or secondary. HISTORY AND SIGNS: A 40-year-old man treated for systemic hypertension complained of decreased vision and floaters in his right eye. Initial examination revealed decreased visual acuity to 20/50 of the right eye with a slight dyschromatopsia, but a lack of afferent pupillary defect and normal visual fields. Fundus examination showed the presence of a slightly swollen right optic disc and chorioretinal folds. A diagnosis of presumed anterior ischemic optic neuropathy was made. Symptoms persisted and, five months later, right proptosis was noted. Magnetic resonance imaging revealed a diffuse thickening of the parieto-temporal bone and the greater wing of the sphenoid bone on the right side. Radiological differential diagnosis included fibrous dysplasia and metastasis. THERAPY AND OUTCOME: Bone biopsy revealed a grade I intraosseous meningioma. Conservative management was chosen because the lesion was too extensive to be resected and radiotherapy is usually not efficient on grade I meningiomas. CONCLUSIONS: Intraosseous meningiomas are benign tumors which are due to meningeal cells entrapment during vaginal delivery. It is a rare tumor of slow progression. Therapy usually consists of resection and cranioplasty and/or radiotherapy. In the present case, decompression of the optic canal remains feasible in case of further visual loss.

Adult↗

[Endoscopically-controlled endonasal orbital decompression in malignant exophthalmos].

In 6 patients with endocrine ophthalmopathy, indications, surgical technique and results of the endoscopic controlled endonasal orbital decompression are described in comparison to the common surgical procedures. When medical and radiation therapy fail, indications for decompression are a) loss of visual acuity or visual field defects, b) increasing strabismus, c) severe keratopathy due to eyelid retraction. The endoscopic-controlled endonasal surgical decompression technique is proceeded in three steps. First, an endonasal ethmoidectomy with resection of the middle turbinate is performed and the medial wall of the maxillary sinus is widely opened. Second, the medial and inferior wall of the orbital walls are removed, preserving the infraorbital nerve. In the last step, the periorbital area is incised and the orbital fat herniates. The advantages of this procedure consist in the absence of exterior scars and the known morbidity of a Caldwell-Luc antrotomy. The results were documented by computed tomographic scans (CT), magnetic resonance imaging (MRI), Hertel measurements, evaluation of ocular motility and ophthalmoscopy. An average of 3-4 mm improvement in Hertel-measurements could be reached. All patients had a postoperative improvement of visual acuity. 2 patients developed more significant diplopia postoperatively, whereas in all other patients ocular motility either improved or rested unaffected. Therefore, the endoscopic controlled endonasal procedure allows to obtain comparable results to the common extranasal and transantral procedures without the disadvantages of the latter.

Aged↗

Reliability of exophthalmos measurement and the exophthalmometry value distribution in a healthy Dutch population and in Graves' patients. An exploratory study.

AIM: The purpose of this study was to test the reliability of an exophthalmometer commonly used in the Netherlands; to determine the exophthalmometry value distribution with this instrument and to assess the upper exophthalmometry limits of normal in a healthy, adult, Caucasian, Dutch population. Furthermore, to assess the effects of gender and age on exophthalmometry readings in this group and in a group of Graves' patients by comparing healthy, adult, Caucasian, Dutch individuals with adult, Caucasian, Dutch Graves' patients. METHODS: To test the reliability of our Hertel exophthalmometer, we determined the interobserver variation between two observers by measuring 160 eyes in healthy, adult, Caucasian, Dutch females and males (10 females and 10 males in each decade between 20 and 60 years of age). These data were also used for the assessment of the Hertel value distribution and for defining the upper limits of normal in these individuals by logistic regression analysis. The effects of disease, age and gender were established using these data plus data of a retrospective study of 393 adult, Caucasian, Dutch females (n=294) and males (n=99) with Graves' orbitopathy in whom Hertel values were measured with the same exophthalmometer. RESULTS: Exophthalmometry using an Hertel exophthalmometer appeared reliable (Pearson correlation coefficient for interobserver variation 0.89; 96% of the Hertel values measured by two observers were within the limits (of 2 mm) of agreement). Hertel values usually show a normal distribution in healthy individuals and in Graves' patients and are sex- and age-dependent, but there was no dependence on age in this small series in adults. Logistic regression analysis revealed an upper limit of normal of 16 mm in females and 20 mm in males in our group, using the exophthalmometer described. CONCLUSIONS: Exophthalmometry is reliable and absolute measurement of proptosis is feasible. International standardization of Hertel exophthalmometry is required in order to compare exophthalmometry data in the literature reliably.

Adult↗