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Oskar Hirsch and surgery for exophthalmos.

Despite a better appreciation of the relationship between the eye and the thyroid gland in Graves' disease, the treatment of moderate to severe exophthalmos remains surgical. For many years, standard textbooks recommended a risky, transfrontal approach for exophthalmos that had to be performed by a neurosurgeon. A preferable transantral procedure, which can be performed by a plastic surgeon, was originally described by Oskar Hirsch, a Viennese otorhinolaryngologist, in 1930. Although his technique offers the advantage of causing less morbidity, it was several decades before its merits were widely recognized by practitioners.

Austria↗

Exophthalmos and elevated thyroxine levels in association with lithium therapy.

Although goiter formation and hypothyroidism are not infrequent following lithium therapy, the association of exophthalmos and/or elevated thyroxine levels with lithium is uncommon. We describe a 62 year old man who rapidly developed exophthalmos, elevated circulating thyroxine, elevated TSH levels and elevated radioiodine uptake within six weeks of reexposure to lithium carbonate. When the medication was stopped, all indices of thyroid function returned to normal and the proptosis receded from 22 to 17mm. Lithium decreases secretion of preformed thyroid hormone and the secondary elevation of TSH levels usually restores euthyroid status. We propose that in our patient, because triiodothyronine levels remained normal, the pituitary was refractory to the normal inhibitory feedback effect of thyroxine and that continued hypersecretion of TSH resulted in hyperthyroidism

Bipolar Disorder↗

Orbital decompression of exophthalmos.

In summary, we have presented our clinical experience, diagnostic techniques, surgical recommendations, and the results in 252 patients who have undergone transantral orbital decompression for malignant exophthalmos. Reduction of proptosis up to 12 mm. has been obtained. In 80 per cent of the patients the eyes were balanced within 1 mm. Complications have been minimal. We believe that this procedure has proved its efficacy in the treatment of malignant exophthalmos. In most cases it makes possible the preservation or improvement of vision and arrests the progressive extraocular muscle imbalance. The importance of close cooperation between the otolaryngologist, endocrinologist, and ophthalmologist cannot be overemphasized in the management of this disorder.

Adolescent↗

Blepharoplasty in the patient with exophthalmos.

The plastic surgeon performing blepharoplasty should maintain a high degree of surveillance for the presence of exophthalmos. Exophthalmos may be masked by the presence of eyelid compensations and substantial amounts of eyelid fat. The surgeon should know how to detect the condition and measure its extent.

Exophthalmos↗

Indium-111 octreotide in Graves' disease and in the evaluation of active exophthalmos.

Thyroid Associated Ophthalmopathy (TAO) is an autoimmune disorder generally associated with Graves' disease (GD). The aim of our study was to evaluate the uptake of indium-111 Octreotide (111In-OCT), a somatostatin (SS) analogue able to bind specific SS receptors, at the level of the thyroid and orbits in patients with TAO. Seven patients with exophthalmos were investigated: six had GD while one was affected with a non small cell lung cancer (NSCLC). One patient with GD had undergone total thyroidectomy (TT) for a thyroid cancer. At the time of the study two patients were hyperthyroid, four were euthyroid and one was hypothyroid. 111 MBq of 111In-OCT were i.v. injected and two 30-minute scans were performed at 4 and 24 hours; 5 minute planar images were also obtained at 25, 60 and 120 minutes. A 180 degrees SPECT was carried out 5 hours after the injection in one patient. A qualitative analysis was performed, comparing these images with those obtained in 7 control patients without thyroid illness or exophthalmos. Moreover, in the TAO patients thyroid, orbit and brain counts were evaluated in comparison with background (BK) and blood activity (BA), measured at the level of the venous longitudinal sinus. In GD intense thyroid uptake was demonstrated independently of the functional state, with highest ratio compared to BK seen at 24 hours. Low uptake in the patient with NSCLC, no activity in the patient with GD that underwent TT, and slight or absent thyroid uptake in the controls were observed. Intense uptake was seen in the orbits of the patient who clinically had the most severe ophthalmopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Nonpulsatile exophthalmos in carotid-cavernous sinus fistula.

An unusual case of carotid artery-cavernous sinus fistula with pulseless exophthalmos is presented in a two and one-half year old child. Though exophthalmos and increased episcleral venous distention were present, the ophthalmic artery pressure was not significantly altered as determined by ocular pneumoplethysmography. Consequently there was no evidence of hypoxic sequelae which frequently occur with this arteriovenous shunt. The combined use of arteriography and ocular pneumoplethysmography is advocated for determining and characterizing the presence of significant intracranial arteriovenous disease.

Arteriovenous Fistula↗

Successful treatment of exophthalmos and pretibial myxoedema with plasmapheresis.

A patient with Graves's disease with acute progressive exophthalmos and pretibial myxoedema was treated twice with plasmapheresis. Two weeks after the first treatment the symptoms recurred, but 20 weeks after the second treatment the exophthalmos was much improved and the pretibial myxoedema had disappeared. Analysis of sequential serum IgG concentrations and the thyroid-stimulating immunoglobulin index suggested that the two conditions were caused by specific IgGs. The results suggest that plasmapheresis has a useful place in the treatment of acute and rapidly progressive ophthalmopathy and pretibial myxoedema in patients with Graves's disease.

Acute Disease↗

Surgical treatment of endocrine exophthalmos by removal of orbital fat: clinical experience.

PURPOSE: To report a series of 73 patients with endocrine exophthalmos treated by removal of orbital fat using the transpalpebral approach during the period 1989 to 1999. METHODS: The operation was performed according to the technique described by Olivari. Aesthetic analysis was done preoperatively and postoperatively (more than 6 months after surgery). The number of complications was also observed. RESULTS: The average volume of resected fat was approximately 7.6 mL per orbit. No major complication was observed. In 9 patients with epiphora, all improved. One patient developed postoperative diplopia and 5 complained of temporary diplopia. Appearance improved in 62 patients (85%). CONCLUSION: Surgical removal of orbital fat associated with endocrine exophthalmos provides consistent improvement in appearance with a low rate of complications. Additional procedures may be indicated to improve the cosmetic outcome.

Adipose Tissue↗

Graves' disease. Initial presentation with exophthalmos and solitary hot nodule.

A 52-year-old man presented with left exophthalmos. A thyroid scan showed a right lobe hot nodule with suppression of the remainder of the gland. Thyroid function tests were normal. In less than two years, the patient had worsening of the exophthalmos. Thyroid indices then revealed hyperthyroidism and the thyroid image had markedly altered (with evidence of diffuse function). This change, initially showing a hot nodule and then diffuse thyroid overactivity, has been reported previously in three cases (all women). Characteristics of the disorder in these four individuals were reviewed. It is possible that the patients had two distinct diseases, separated temporally.

Graves Disease↗

[The surgical treatment of thyrotoxic exophthalmos using Ogura's operation (author's transl)].

Ogura's operation consists of resection via De Lima's approach of the bony floor of the orbit and the lateral wall of the ethmoid. The orbital periosteum is then incised in order to allow the orbital fat to enter the sinuses and thus decompress the orbit. In most cases the operation is performed on both sides at the same time. It is used in cases of malignant or severe thyrotoxic exophthalmos, or in the presence of optic neuritis. The results obtained were good in 9 cases with a marked reduction in exophthalmos and rapid cure of optic neuritis. An additional ophthalmological procedure is sometimes necessary in order to perfect the anesthetic result.

Ethmoid Sinus↗

Ophthalmic surgery in malignant exophthalmos.

Malignant exophthalmos or the ophthalmic component of Graves' disease is a functionally impairing and cosmetically deforming disorder of orbital tissue, separate from the sympathetic effects of thyrotoxicosis. The deposition of immune complexes of thyroglobulin and its antibodies within orbital muscles results in progressive exophthalmos, ophthalmoplegia, chemosis, and lid fullness. Increasing intraorbital edema and optic nerve tension may produce irreversible retinal ischemia. If unresponsive to medical treatment, patients exhibiting increasing loss of visual acuity are therefore candidates for emergency surgery. Early attempts at surgical correction of the mechanical aspect of Graves' ophthalmopathy were unsuccessful. The current technique of transantral orbital decompression, when done before extreme advancement of the ocular disease, is successful and meets the following objectives: It restores visual loss and prevents further deterioration of visual acuity, protects the cornea, restores extraocular muscle movements, decreases intraorbital pressure, produces a satisfactory cosmetic result, and avoids serious complications. Patients with persistent extraocular muscle imbalance or lid lag may ultimately require oculoplastic surgery.

Diagnosis, Differential↗

Recurrent Churg-Strauss vasculitis. With exophthalmos, hearing loss, nasal obstruction, amyloid deposits, hyperimmunoglobulinemia E, and circulating immune complexes.

Churg-Strauss vasculitis in remission for 4 1/2 years recurred, with new, previously undescribed features, after a lapse in corticosteroid therapy. Bilateral exophthalmos, unilateral hearing loss, and nasal obstruction accompanied fever, severe asthma, and palpable purpura. Leukocytosis with eosinophilia, an elevated ESR, hyperimmunoglobulinemia E, an pulmonary infiltrates were seen again. Circulating immune complexes were detected, and microamyloid deposits were found in the conjunctiva and skin. All clinical and laboratory manifestations responded to corticosteroid therapy. Monitoring the levels of IgE may add a diagnostic and prognostic feature to the classification of necrotizing vasculitides.

Adult↗

Subacute bilateral malignant exophthalmos due to orbital medulloblastoma metastases.

A 26-year-old woman complained of subacute bilateral exophthalmos, one year after the extirpation of a cerebellar medulloblastoma. The clinical picture and the orbital computed tomographic scan suggested orbital pseudotumor or endocrine ophthalmopathy rather than orbital medulloblastoma metastases. The latter diagnosis was confirmed by histologic examination. The occurrence of extraneural medulloblastoma metastases including bilateral orbital involvement is discussed.

Acute Disease↗

Malar fractures associated with exophthalmos.

Although fracture of the malar bone is often associated with enlargement of the orbit and subsequent development of enophthalmos, occasionally, a blowin type of orbital floor fracture and exophthalmos occurs. The causes of the injury and the differential diagnosis of the blowin fracture are reviewed. An explanation is offered for the development of the symptoms. Early recognition, open reduction and fixation of the malar bone, and repair of the floor of the orbit defect are important for successful management of the injury.

Adolescent↗

Unilateral endocrine exophthalmos. Diagnostic problems in association with computed tomography.

Swollen orbital extraocular muscles may mimic an orbital apex tumor on computed tomography. In five of our patients, in spite of highly suggestive findings on CT scanning, indicating the presence of an orbital apex lesion, the correct diagnosis proved to be endocrine exophthalmos. The value of complementary CT projections and of orbital ultrasonography to assist in the differential diagnosis is discussed, and the additional contribution of orbital phlebography for the confirmation of lesions in the orbital apex is emphasized.

Adult↗

Epidural haematoma and unilateral exophthalmos--a review.

Based on 9 cases in the literature and one of our own a review is made on the occurrence of an association of an intracranial epidural haematoma with unilateral exophthalmos. The possible pathogenesis of this infrequent phenomenon is discussed.

Brain Concussion↗

Studies in relation to endocrine exophthalmos: the biochemical composition of human retrobulbar connective tissue.

As part of studies on the pathogenesis of exophthalmos of Graves' disease, the biochemical composition of human retrobulbar tissue was investigated. The connective tissue was composed of 72.7% lipid, 23.8% water and 3.5% dried defatted tissue. Total tissue glycosaminoglycan (GAG) amounted to 0.18% of dried defatted tissue. Approximately 27% of the total hexosamine and 19% of the total tissue galactosamine were recovered in the GAG fraction. Cellulose microcolumn fractionation of GAG showed that the hyaluronic acid and dermatan sulfate were the two major GAG species.

Aged↗

[Cerebellar syndrome, exophthalmos and secondary hypogonadism in Erdheim-Chester disease].

We present a 50-year-old patient with a slowly progressive cerebellar syndrome, left-sided exophthalmos, secondary hypogonadism, and multiple pleomorphous skin alterations. The diagnosis of Erdheim-Chester disease was established by the radiological detection of a left-sided retrobulbar space-occupying mass, a hypophysial stalk lesion, alterations in both cerebellar hemispheres, retroperitoneal imbibition, osteolytic/osteosclerotic changes in the metaphysis and diaphysis of the long bones, and a skin biopsy with histological detection of a non-Langerhans-cell histiocytosis. The etiology of the Erdheim-Chester disease is unknown. Cerebral manifestations of this rare disease have been documented in only a very few cases. Whereas the extracranial alterations are due to pathologic histiocyte proliferation, cerebellar changes are considered to be the result of demyelinisation or infiltration of xanthogranulomas.

Biopsy↗