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Fronto-orbitonasal intradiploic meningioma in a child.

Intradiploic meningioma, which may be classified as a subgroup of intraosseous meningioma, is a rarely encountered disorder. To date, less than 10 cases have been reported. Here, we report a case of fronto-orbitonasal intradiploic meningioma. A 12-year-old female with exophthalmos and diplopia was operated on for a cranial intradiploic mass lesion. Histopathological evaluation of the specimen confirmed the diagnosis of intradiploic psammomatous meningioma. Her exophthalmos did not change, but the diplopia disappeared. This case is unique in that it is an extensive case of intradiploic meningioma of the orbital roof and frontal base in a child. Intradiploic meningiomas generally are of psammomatous type. Especially tumors adjacent to the orbita cause exophthalmos; cases located on the other side of the calvarium may not cause any symptom or sign other than headache or sometimes a mass on the scalp. Treatment, as with meningiomas located in the intracranial cavity, is total resection of the lesion.

Biopsy↗

Primary localized orbital amyloidosis: a case report.

PURPOSE: Amyloidosis refers to a heterogeneous group of disorders associated with deposition of chemically distinct fibril proteins. Isolated orbital amyloidosis is a rare condition and requires systemic examination. The authors report a case of amyloid deposit in the orbit whose systemic investigation has been negative. METHODS: A 64-year-old woman presented to the eye clinic with left-sided orbital mass, mild exophthalmos with downward ocular displacement, and ptosis. The patient presented also visual acuity loss and ocular hypertension. No systemic involvement was noted by systemic workup and it confirmed the primary orbital amyloidosis. RESULTS: The patient was subject to full clinical examination, laboratory examinations, orbital echography, magnetic resonance imaging and total body computed tomography scans, rectal mucosa, and temporal artery biopsies. It was necessary to exclude systemic amyloidosis. The presence of amyloid deposits was confirmed by biopsy of orbital mass. The chemical nature of deposit was characterized using light microscopy, immunohistochemistry, and electron microscopy. CONCLUSIONS: Orbital amyloidosis is a very rare disease. It should be considered in the diagnosis of patients with ptosis and exophthalmos. The treatment usually consists of surgical removal of the amyloid mass and follow-up for a likely local recurrence. In our case, mass excision determined the decrease of exophthalmos and intraocular pressure.

Amyloid↗

Endoscopic orbital decompression.

Exophthalmos from Graves' disease can result in visual disability and cosmetic deformity. Surgical treatment of this disorder is now possible through an intranasal endoscopic approach that allows removal of the medial orbital wall and floor without an external incision. Endoscopic orbital decompression was performed on 22 orbits in 14 patients for treatment of progressive exophthalmos. Local anesthesia was used in five cases. Sixteen procedures involved a concurrent lateral orbital decompression performed through an external approach. There were no intraoperative or postoperative complications. Visual acuity remained stable or improved in all cases. Proptosis was reduced an average of 3.2 +/- 1.1 mm (range 2 to 4.5 mm) by endoscopic decompression alone. When a lateral decompression was also performed, proptosis was reduced by an additional 2.4 mm, for an average improvement of 5.6 +/- 1.7 mm (range 2 to 8 mm). Endoscopic orbital decompression appears to be a safe technique for the treatment of exophthalmos that can be performed effectively with the patient under general or local anesthesia.

Adult↗

Thyroid-stimulating antibody in a patient with euthyroid Graves' disease.

We report an 11-year-old girl with euthyroid Graves' disease. She was referred to our clinic because of left exophthalmos without other symptoms suggestive of hyperthyroidism. Her serum concentration of free thyroxine (FT4) and free triiodothyronine (FT3) were normal, but thyroid-stimulating hormone (TSH) was below normal and impaired TSH response to TSH releasing hormone (TRH) was found. Although the sera were positive for anti-TSH receptor antibody (TRAb) and thyroid-stimulating antibody (TSAb), both titers were not as high as usually observed in Graves' disease. Three months later, she developed hyperthyroidism and was treated with propylthiouracil. Within 2 weeks of the initiation of therapy, all symptoms except exophthalmos disappeared, and after 2 months of treatment TRAb was negative though TSAb remained positive. TSAb is therefore a good indicator to use in the diagnosis and follow-up of euthyroid Graves' disease and should be measured in patients with exophthalmos of unknown origin, even in children.

Antithyroid Agents↗

Orbital decompression in Graves' orbitopathy: a combined endoscopic and external lateral approach.

AIMS: First, to assess the outcomes of endoscopic and external lateral orbital decompression in patients with dysthyroid orbitopathy. Second, to establish a correlation between the percentage of postoperative diplopia and the technique used. METHODS: A retrospective review of 40 patients (73 eyes) who underwent endoscopic medial orbital decompression and external lateral orbital decompression between 1997 and 2003 at the Hôpital Enfant-Jesus in Quebec City. Some of these patients also had an inferior endoscopic decompression. All patients had exophthalmos. The principal indications for surgery were 42 eyes with compressive optic neuropathy, 13 eyes with other ocular disorders, and 18 eyes with exophthalmos only. All patients with neuropathy underwent endoscopic decompression of the optic canal. RESULTS: All patients had satisfactory improvement of their ocular pathology. Optic neuropathy was completely resolved in 92.85% (39 of 42 eyes). In patients who did not have preoperative diplopia (26), diplopia developed in 70% (14 of 20) of those who underwent medial and inferior decompression compared with 16.6% (1 of 6) of patients who underwent medial decompression only. CONCLUSION: Endoscopic medial orbital decompression is very effective in resolving compressive optic neuropathy. Preservation of the orbital floor, when the degree of exophthalmos allows, reduces the incidence of postoperative diplopia.

Adult↗

[Exophthalmia].

The authors present the association of signs and symptoms in exophthalmos syndrome, which occurs in many diseases. Although exophthalmos was considered to be of ophthalmological interest, according as researches were made on its pathogeny and according as investigation methods were developed, exophthalmos is no more considered a symptom, but a syndrome. After defining the syndrome, the present study shows its classifications, clinical and complementary investigations, and its treatments.

Diagnosis, Differential↗

[Acoustic investigations in orbital tumor diagnosis (author's transl)].

360 patients with unilateral exophthalmos were examined with echoophthalmograph to study the diagnostical possibilities of a method of echography in orbital tumors. The findings of para- and transbulbal methodics were checked in 140 patients. The tonometry of tumors and soft orbital tissues was performed. The methodics of acoustic orbitonometry based on the measurement of retrobulbal neoplasms at the moment of their step-by-step compression was worked out. The echographic symptoms of different orbital neoplasms is described as well as those of pseudotumor and tireotropic exophthalmos. The possibility of acoustic measurement of orbital neoplasms is shown. The dependence between the extent and progress of deformation in the exophthalmed eye on one hand-nature of exophthalmos, character and localization of the tumor on the other was revealed. The quantitative data about the compression of different orbital tumors were obtained. It was shown that the surveying echography, biometry and acoustic orbitonometry facilitate the task of differential diagnosis, allow the localization and size of the tumor to be detected and help to control the results of the treatment.

Anthropometry↗

[Digital subtraction angiography of cerebrum for diagnosis of cavernous sinus fistula and its intravascular treatment].

OBJECTIVE: To evaluate the diagnosis of digital subtraction angiography (DSA) of cerebral arteries and the intravascular treatment for carotid artery-cavernous sinus fistula (CCF) mainly manifesting as pulsating exophthalmos. METHODS: Digital subtraction angiography (DSA) of cerebral arteries was carried out for 15 patients with pulsating exophthalmos. Of them, detectable balloon and tungsten filament microcoil were used for intravascular embolism treatment in 12 cases, and the 3 cases with CCF of the branch of external carotid artery were not treated. RESULTS: Of the 15 cases, 12 were fallen in the CCF of high flow type of unilateral internal carotid artery, and 3 in the CCF of low flow type of unilateral external carotid artery. The symptoms and signs of intravascular embolism disappeared after treatment in 11 cases. CONCLUSION: Cerebral artery DSA and intravascular therapy are the ideal methods for the diagnosis and treatment of CCF mainly manifesting as pulsating exophthalmos.

Adult↗

Ultrasonic evidence of inflammatory thickening and fluid collection within the retrobulbar fascia: the T sign.

A case of nonfebrile and nonpyogenic but inflammatory exophthalmos in a 60-year-old woman is presented in illustration of a diagnostic enigma which has previously eluded physical and roentgen examination. With painful limitation of rotation, a less than satisfactory diagnosis of tenonitis or, less commonly, orbital pseudotumor usually has been made. The convenient assistance of contact (nonimmersion) B-scan ultrasound gives objective evidence of fluid within Tenon's space and communicating into the vaginal spaces beneath the optic nerve sheath appearing as homogeneous and echolucent areas. Concurrently there is thickening of the posterior bulbar complex and muscle sheaths evidenced by increased echo density. A definitive finding as the echolucent "T sign" lends substantial and specific basis for clinical management. Rapid improvement in exophthalmos, rotations, and discomfort following oral steroids (without antibiotics) further supports the etiologic concept of both noninfectious and nonendocrine ophthalmopathy. The T sign is suggested as a specific finding, rather than an artifact, differentiating this exophthalmos from neoplastic invasion or thyroidopathy.

Diagnosis, Differential↗

Radiological diagnosis of orbital tumours.

Apart from plain X-rays of the orbit, the principal radiological methods of examination of exophthalmos are: orbital phlebography, carotid arteriography and, recently, computer tomography. The value of each of these methods is discussed with reference to four patients suffering from exophthalmos of varying aetiology. Computer tomography supplies the most comprehensive information on localization and extent of retro-orbital processes as causes of exophthalmos. Phlebography would seem to be the method of choice in demonstrating processes close to the osseous boundaries of the orbit. Information on the aetiology of retro-orbital processes is rarely obtained by radiological methods. In this respect carotid arteriography would seem to make the most important contribution.

Adult↗

[Thyroid ophthalmopathy: clinical and tomographic study].

BACKGROUND: To evaluate exophthalmos by Hertel exophthalmometer (HE) compared to computed tomography (CT), and extraocular muscle width on CT and its relation with the clinical course in patients with thyroidal ophthalmopathy. METHODS: On reviewed 118 orbits from 59 patients (50 women, mean age 40.6 y., range 12-84) who suffered a thyroidal ophthalmopathy (Graves' disease, n = 57; Hashimoto's thyroiditis, n = 2). Muscle width was evaluated using coronal and mid-axial scans in all cases. Medial rectus (MR) width was analysed by Hallin and Feldon technique. RESULTS: Exophthalmos by HE was 22.5 +/- 2.5 and 23.2 +/- 3.2 mm (right-RE/left eye-LE), and CT 20.8 +/- 3.2 and 20.9 +/- 2.9 mm (p < 0.00001, r = 0.72 and 0.65, respectively). MR width on mid-axial scan was 4.1 +/- 1.6 and 4.2 +/- 1.5 mm (RE/LE). On coronal scans, MR was the muscle more often enlarged followed by superior, inferior and lateral rectus. CONCLUSIONS: Exophthalmos measured by HE was greater than by CT-measured one, but with a good correlation. Muscle were not equally affected, being MR the most frequently enlarged. MR-width was not related to duration and severity of disease.

Adolescent↗

[Exophthalmias in Zaire].

PURPOSE: To determinate the frequencies of causes of exophthalmos in Zaïre. METHODS: In a retrospective study, the records of 129 patients with exophthalmos seen from 1986 to 1994 were reviewed. All patients were examined ophthalmologically and most of them medically. X-Rays, orbital CT Scan, were performed most frequently. RESULTS: The sex distribution was: 79 (61%) male patients and 50 (39%) female patients. The sex ratio being 1.50/1. The ages of patients ranged from one to 68 years. Unilateral exophthalmos was frequent (76% of cases). Tumors were present in 42% patients, followed by inflammatory diseases in 26% of patients, thyroid eye disease in 9% and traumatic aetiology in 1.6%; the etiology was not determined in 14%. Retinoblastoma (8%), lymphoma (7%) and rhabdomyosarcoma (5%) were the most frequent tumors seen. Orbital cellulitis (26%) was frequently associated with adjacent sinus infection. CONCLUSION: The etiologic factors found in this study could be compared to those published in the World with the exception of orbital cellulitis and retinoblastoma that showed a high incidence in this study.

Adolescent↗

Enlargement of the tensor intermuscularis muscle in Graves' ophthalmopathy. A computed tomographic and magnetic resonance imaging study.

OBJECTIVE: To determine whether the tensor intermuscularis muscle (TIM), which consists of muscle fibers in the superolateral intermuscular orbital septum, is involved in Graves' ophthalmopathy (GO). DESIGN: The computed tomographic (n = 24) and magnetic resonance imaging (n = 10) appearances of the TIM were retrospectively examined in 34 patients with known GO. The severity of GO was assessed by applying a scoring system from 0 to 3 (ie, normal [0], mild [1], moderate [2], and severe [3]) to each of the rectus muscles and superior oblique muscle. The severity of exophthalmos, enlargement of the superior ophthalmic vein, and displacement of the lacrimal gland were also recorded. RESULTS: The TIM appeared as thickening of the septum immediately behind the globe, and it was best seen on coronal magnetic resonance images. There was enlargement of the TIM in 19 of the 34 patients, and it was bilateral in 17. Enlargement was present only in patients with moderate or severe involvement of other muscles (muscle index, > 7/15), and it was significantly correlated with the muscle index (P < .05), exophthalmos (P < .05), enlargement of the superior ophthalmic vein (P < .005), and anterior displacement of the lacrimal gland (P < .01). Severe enlargement of the TIM was seen in only five of the 34 patients, and it showed a close correlation with the muscle index (P < .005), exophthalmos (P < .001), enlargement of the superior ophthalmic vein (P < .001), and anterior displacement of the lacrimal gland (P < .001). CONCLUSIONS: Enlargement of the TIM in GO can be identified on computed tomographic and magnetic resonance imaging scans. It is invariably associated with moderate or severe involvement of other extraocular muscles, and it correlates closely with other well-recognized imaging features of severe GO.

Adult↗

[Lateral orbital decompression for Graves' orbitopathy. Indication, surgical technique, and treatment success].

PURPOSE: In Graves' disease a discrepancy between volume increase of the orbital soft tissues and fixed volume of the orbital cavity leads to exophthalmos. The patients do not only feel cosmetically disfigured, they often complain about more or less painful retroorbital pressure sensation or show symptoms of compressive optic neuropathy or corneal exposure because of a significant lid lag. To solve this problem, different orbital decompression techniques have been developed. This is to report about our results with a modified Dollinger technique for lateral orbital decompression. PATIENTS AND METHODS: A total of 27 patients aged 19-76 years (mean: 45.1 years) with Grave's ophthalmopathy were recorded who had undergone orbital decompression by a lateral approach between June 1999 and April 2003. The modified Dollinger technique was performed by deepening the osteotomy to the level of the sphenoid wing and by additional resection of intraconal fat. RESULTS: The reduction of exophthalmos achieved after decompressive surgery averaged 2.9 +/- 1.1 mm. Of the patients whose indication for orbital decompression was a compressive neuropathy, the visual acuity improved postoperatively for 3 lines. Of the 16 patients with preoperative retrobulbar pressure sensation, 12 had no complaints after the operation. Remarkably no significant impairment of the ocular motility resulted after surgery. CONCLUSIONS: Decompression of the orbit by the modified Dollinger technique is a safe and effective approach to reduce exophthalmos, retrobulbar pressure sensation, and compression neuropathy as a result of diffusely elevated orbital tissue tension. In the case of direct compression of the optic nerve in the orbital apex, additional medial orbital wall decompression has to be considered.

Adult↗

Testicular seminoma metastatic to the orbit.

Painful exophthalmos, ophthalmoplegia, and visual loss in the right eye developed in a 28-year-old man four months after he underwent a radical orchiectomy and irradiation for a seminoma of the right testicle. Prednisone therapy failed to relieve the symptoms, and an orbital biopsy found a metastatic testicular seminoma. Orbital irradiation with radioactive cobalt reduced the exophthalmos, reversed the ophthalmoplegia, and improved his visual acuity. Previous cases of nonmetastatic exophthalmos in patients who had testicular seminoma have been reported, but, to the best of our knowledge, this is the first report of a patient who had orbital seminoma metastatic from the testis. Successful irradiation of the orbital focus can minimize ocular morbidity and may improve the prognosis.

Adult↗

Treatment of Graves' disease and the course of ophthalmopathy.

PURPOSE: Contradictory results have been obtained with regards to the effect of various treatment modes on the exacerbation of Graves' ophthalmopathy, probably because the number of patients in each study was small and some studies were analyzed only in relation to one type of treatment. To circument these problems, we studied the course of Graves' ophthalmopathy after various modes of therapy for thyrotoxicosis among 537 patients with Graves' disease. PATIENTS AND METHODS: A total of 537 patients with Graves' disease were prospectively studied over an 11-year period. Thirty-one patients were lost to follow-up during the first six months after treatment and were excluded from the study. Of those remaining, 426 received one form of treatment, 79 received two kinds of therapy, and one received three kinds of therapy. Thus, surgical treatments numbered 164, radioactive iodine-131 (131I) treatments numbered 241, and medical treatments numbered 182. Ocular signs were considered improved or exacerbated by the following criteria: decrement or increment of the exophthalmos of 2 mm or more, improvement or deterioration of visual acuity, and regression or progression of extraocular muscle involvement causing diplopia. RESULTS: Among patients who did not have infiltrative ophthalmopathy before treatment, there was no difference in the occurrence of posttreatment exophthalmos in the surgically, medically, and 131I-treated patients (7.1%, 6.7%, and 4.9%, respectively). The incidence and the degree of progression of ophthalmopathy in patients who already had exophthalmos before treatment were similar in the medically, surgically, and 131I-treated groups (19.2%, 19.8%, and 22.7%, respectively). Most of the progression occurred in the posttreatment euthyroid stage. The incidence of improvement of ophthalmopathy was also similar (14.1%, 12.6%, and 12.3% in the medically, surgically, and 131I-treated patients). CONCLUSION: In conclusion, we found no influence of type of therapy for thyrotoxicosis on the clinical course of Graves' ophthalmopathy in this retrospective study of 537 patients.

Adult↗

Clinical features of Graves' ophthalmopathy in an incidence cohort.

PURPOSE: To determine the clinical characteristics of an incidence cohort of patients with Graves' ophthalmopathy. METHODS: We reviewed the community medical records of 120 patients residing in Olmsted County, Minnesota, in whom Graves' ophthalmopathy was diagnosed between 1976 and 1990. RESULTS: Among 120 patients with Graves' ophthalmopathy, 108 (90%) patients had Graves' hyperthyroidism, one (1%) had primary hypothyroidism, four (3%) had Hashimoto's thyroiditis, and seven (6%) were euthyroid. At some point in their clinical course, eyelid retraction was present in 108 patients, whereas the approximate frequency of exophthalmos was 62% (73 patients); restrictive extraocular myopathy, 43% (51 patients); and optic nerve dysfunction, 6% (seven patients). Only six (5%) patients had eyelid retraction, exophthalmos, optic nerve dysfunction, extraocular muscle involvement, and hyperthyroidism. At the time of diagnosis of ophthalmopathy, upper eyelid retraction and eyelid lag were documented in 85 and 52 patients, respectively, and the most frequent ocular symptom was pain (36 patients, 30%). Diplopia was noted at the initial examination by 20 patients, lacrimation was present in 25 patients, 19 patients had photophobia, and nine patients had blurred vision. Decreased vision from optic neuropathy was present in less than 2% of eyes at the time of diagnosis. Thyroid dermopathy and acropachy accompanied Graves' ophthalmopathy in five patients (4%) and one (1%) patient, respectively. Myasthenia gravis occurred in only one patient. CONCLUSIONS: Eyelid retraction is the most common clinical sign of Graves' ophthalmopathy. The complete constellation of typical features (hyperthyroidism, eyelid retraction, exophthalmos, restrictive extraocular myopathy, and optic nerve dysfunction) occurs relatively infrequently.

Cohort Studies↗

Prevalence of increased intraocular pressure in Graves' disease--evidence of frequent subclinical ophthalmopathy.

Graves' exophthalmos is frequently associated with elevated intraocular pressure on upgaze. Eighty patients with Graves' disease were evaluated prospectively by applanation tonometry to assess the prevalence of ophthalmopathy in this disorder. Whereas 21 (26 per cent) of the 80 patients had exophthalmos, 61 (76 per cent) had abnormal intraocular pressure (delta greater than or equal to 3 mm Hg). All patients with exophthalmos had elevated intraocular pressure on upgaze; 40 (68 per cent) of 59 patients without proptosis had abnormal pressure readings. The mean interval between the onset of Graves' disease and this study was 6.3 +/- 1.0 years (S.E.M.) for those patients who had exaggerated positional changes in intraocular pressure, as compared with 3.0 +/- 1.0 years for those with normal intraocular pressure (P less than 0.005). All but 1 of 15 patients in whom the diagnosis of Graves' disease had been documented 10 or more years earlier had increased intraocular pressure on upgaze. We conclude that Graves' ophthalmopathy is more common than is recognized clinically and that eye involvement is an inevitable complication of the disease.

Adult↗