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PubMed1975

Relationship of thyrotropin to exophthalmos-producing substance: formation of an exophthalmos-producing factor by pepsin digestion of mouse pituitary tumor and human thyrotropin preparations.

The present report demonstrates that mouse tumor thyrotropin preprarations have exophthalmogenic activity. In addition it shows that the exophthalmogenic activity of mouse tumor thyrotropin can be increased by partial pepsin digestion, whereas the thyroid stimulating activity of mouse tumor thyrotropin is rapidly destroyed by such treatment. Thus, after 30 min of pepsin digestion, mouse tumor thyrotropin has 130% of its initial exophthalmogenic action but only 10% of its thyroid stimulating activity. Preparations of human thyrotropin are similarly sensitive to partial pepsin digestion, i.e., there is a rapid destruction of thyroid stimulating activity but a very much slower destruction of exophthalmogenic activity. Thus, after 30 min of pepsin digestion, preparations of human thyrotropin retain 80% of their exophthalmogenic activity but only 20-30% of their thyroid stimulating action. Since these results are analogous to those obtained in studies of the partial pepsin digestion of bovine thyrotropin (1), partial pepsin digestion of both purified human and purified mouse tumor thyrotropin preparations should yield an exophthalmogenic fragment of the TSH molecule devoid of thyroid stimulating action.

Animals↗
PubMed1995

Grades of exophthalmos and thyrotropin-binding inhibitory immunoglobulin in patients with myasthenia gravis.

Exophthalmos and thyrotropin-inhibitory immunoglobulin (TBII) were examined in myasthenia gravis (MG) patients with normal thyroid function. Exophthalmos was found in 7 of 31 (22.6%) MG patients, and TBII was detected in 4 of 21 (19.0%) MG patients. The grades of exophthalmos did not decrease after extraocular muscle weakness was improved by treatment. Patients with MG whose diplopia was Tensilon unresponsive were only found among those with exophthalmos. TBII was more frequently detected in MG patients with exophthalmos than in those without. The present study revealed that exophthalmos is not rare in MG patients, even if they do not show any signs of Graves' disease other than exophthalmos and their thyroid functions are within the normal limit. The presence of TBII in MG patients may relate to the development of exophthalmos, and exophthalmos itself might enhance ocular symptoms of MG.

Adult↗
PubMed2006

Exophthalmos of patients with Graves' disease in Chinese of Taiwan.

PURPOSE: To determine the exophthalmos measurements in patients with Graves' disease (GD) and to compare with those of normal subjects among the Chinese of Taiwan. METHODS: Hertel's exophthalmometry were performed in 163 consecutive adult GD patients and 419 normal subjects. The values of exophthalmos, asymmetry, and the distance between lateral orbital rims (base) were evaluated. RESULTS: There was a significant difference between GD and normal adults for exophthalmos and asymmetry, with GD patients demonstrating a mean exophthalmos of 18.32 mm and asymmetry of 0.93 mm as compared with 13.91 and 0.55 mm for normal subjects (P<0.001). The mean value of base for GD adults (106.52 mm) is slightly greater than normal subjects (105.82 mm). There was no gender difference in exophthalmometric value or asymmetry in each group. A total of 79 (48.5%) GD patients had exophthalmos greater than the upper limit of normal Chinese in Taiwan (18.6 mm). Asymmetry over 2 mm was observed in 11 (6.7%) patients with GD but in none of the normal subjects. CONCLUSIONS: GD patients have significant exophthalmos and asymmetry compared to normal subjects. In comparison with previous studies, exophthalmos values for Chinese in Taiwan tend to be lower than those of Caucasians and black-American people, but are close to those of Korean, Japanese, Iranian, Indian, and Asian-American people. Adequate adjustment of normal and abnormal values of exophthalmos according to individual differences is important for the diagnosis of orbital morbidity and management of thyroid associated orbitopathy.

Adolescent↗
PubMed1996

Exophthalmos in Cushing's syndrome.

OBJECTIVE: Exophthalmos was noted in 4 of the 12 patients reported by Harvey Cushing in 1932. Although exophthalmos has often been included in clinical descriptions, no previous study has reported actual measurements in patients with active and treated Cushing's syndrome, and in control patients. The aim of this study was to obtain these measurements. PATIENTS: Thirty-one patients with active Cushing's syndrome (19 iatrogenic), 15 with treated Cushing's syndrome, 18 with Graves' ophthalmopathy, 59 control patients, and 3 patients with active Cushing's syndrome plus a family or personal history of thyroid disease. DESIGN AND MEASUREMENTS: A consecutive series of patients with active and treated Cushing's syndrome were assessed. They were compared with patients with Graves' ophthalmopathy, and with control patients. Exophthalmos was assessed by the author using a Hertel meter. Urinary free cortisol was measured on patients with Cushing's syndrome, and serum thyroxine was estimated for them, and for the patients with Graves' ophthalmopathy. RESULTS: Exophthalmos exceeding 16 mm (> 2 SD above normal mean) was found in 45% of active Cushing's syndrome, 21% of iatrogenic Cushing's syndrome, 20% of treated Cushing's syndrome, 2% of normal controls, and 77% of patients with Graves' ophthalmopathy. No patient with Cushing's syndrome had significant symptoms due to exophthalmos. CONCLUSION: Patients with active Cushing's syndrome have statistically significant exophthalmos. This rarely causes symptoms, and diminishes when cortisol concentrations become normal. Cushing's syndrome and autoimmune thyroid disease may coexist in patients with exophthalmos.

Adolescent↗
PubMed2002

Significance of orbital fatty tissue for exophthalmos in thyroid-associated ophthalmopathy.

PURPOSE: To correlate exophthalmos with the volume of extraocular muscle and orbital fatty tissue in thyroid-associated ophthalmopathy (TAO), using MRI that enables the orbital soft tissues to be well defined. METHODS: Thirty-three orbits, 20 from 10 patients with TAO and 13 from 13 controls, were employed. T1-weighted orbital MR slices 2 or 3 mm thick were obtained in axial, coronal and sagittal planes. Tracing the outlines of each structure, we measured the total sectional areas. Volumes of the extraocular muscle, of the fatty tissue and of the bony orbital cavity were calculated by multiplying the slice thickness. Exophthalmos was also measured using axial MRI. RESULTS: In TAO the volume increment of orbital fatty tissue (6.19 cm(3)) was much greater than that of extraocular muscle (1.16 cm(3)). Increase of exophthalmos by 1 mm needed a total orbital volume increment of 0.92 cm(3). The total orbital fatty tissue volume (correlation coefficient 0.70, P=0.06%) and the anterior orbital fatty tissue volume (0.64, P=0.23%) were more closely correlated with the degree of exophthalmos than was extraocular muscle volume (0.58, P=0.8%). Moreover, the volume increment of extraocular muscle and orbital fatty tissue was not always proportional. CONCLUSION: The results show that the orbital fatty tissue involvement is closely related to the degree of exophthalmos. For studying exophthalmos in TAO, the volumetric change, not only in ocular muscles, but also in orbital fatty tissue, should be taken into consideration.

Adipose Tissue↗
PubMed2005

The removal of the deep lateral wall in orbital decompression: its contribution to exophthalmos reduction and influence on consecutive diplopia.

PURPOSE: To evaluate the contribution of maximal removal of the deep lateral wall of the orbit to exophthalmos reduction in Graves' orbitopathy and its influence on the onset of consecutive diplopia. DESIGN: Case-control study. METHODS: The medical records of two cohorts of patients affected by Graves' orbitopathy with exophthalmos > or = 23 mm, without preoperative diplopia, were retrieved at random from the pool of patients decompressed for rehabilitative reasons at our institution (01/1990 to 12/2003), and retrospectively reviewed. They had been treated with an extended (cases, group 1, n = 15) or conservative (controls, group 2, n = 15) 3-wall orbital decompression performed through a coronal approach. The deep portion of the lateral wall had been removed in the extended decompression group while preserved in the conservative decompression group. Demographics, preoperative characteristics, and surgical outcome were compared. The difference in mean exophthalmos reduction between groups 1 and 2 was considered to be the contribution of the deep lateral wall to reduction of exophthalmos. RESULTS: Groups 1 and 2 were drawn from a pool of 37 and 335 patients, respectively. Demographics and preoperative characteristics of the two groups were not significantly different. The mean contribution of the deep lateral wall to exophthalmos reduction was 2.3 mm. The onset of consecutive diplopia was not significantly different between the two groups (case n = 2/15, controls n = 5/15; P = .203). Diplopia resolved spontaneously in all the patients of group 1, while all the patients of group 2 required surgery. CONCLUSIONS: Removal of the deep lateral orbital wall as part of a coronal-approach, 3-wall decompression, enhances the degree of exophthalmos reduction without increasing the risk of consecutive diplopia.

Adult↗
PubMed1975

Evaluation of unilateral exophthalmos with ultrasonography: analysis of 258 consecutive cases.

Ultrasound examination has emerged as a valuable technique for defining soft tissue abnormalities of the eye and orbit. As high frequency sound waves (5 to 20 megahertz) are projected through soft tissues, echoes are produced at tissue interfaces. These echoes are displayed on oscilloscopes as either one-dimensional amplitude spikes (A-mode ultrasonography), or as dots integrated into a two-dimensional image representing a thin section through the entire orbit (B-scan ultrasonography). A-mode indicates tissue substance, while B-scan illustrates tissue morphology. With this technique, orbital tumors are clearly distinguished in terms of their location, size, and configuration, as well as the basic tissue type (cystic, solid, angiomatous or infiltrative). Other orbital processes are defined by ultrasonography also, including inflammatory conditions such as diffuse pseudotumor or cellulitis, congestive conditions such as dysthyroid exophthalmos, and orbital hemorrhage. Intracranial and vascular etiologies of exophthalmos have no specific ultrasound signs. In a series of 258 consecutive patients with unilateral exophthalmos examined with ultrasonography, diagnosis of tumor was made in 26 percent (66 cases). Overall accuracy of orbital tumor diagnosis by ultrasonography was 94 percent in this series. Tumor localization, configuration, and extent indicated by the test aided in selecting surgical approaches. Other patients in the series were diagnosed ultrasonically as a variety of other entities, mostly inflammatory and dysthyroid exophthalmos. Normal ultrasound studies were found in 19 percent. None of these patients had subsequent diagnosis of tumor. Diagnostic ultrasonography is capable of detecting orbital tumors and distinguishing them from inflammatory, congestive, and other causes of exophthalmos with a high degree of reliability. The test is painless, well tolerated, non-invasive, and non-toxic. It now has a prominent role in evaluation of any patient with exophthalmos.

Abscess↗
PubMed2002

[Unilateral exophthalmos caused by a prolactin producing ectopic pituitary adenoma: case report].

OBJECTIVE: We report a case of a patient with a prolactin (PRL) producing ectopic pituitary adenoma presenting a unilateral exophthalmos. CASE: This 70-year-old woman presented an ophthalmologist with progressive left-sided exophthalmos over the previous 2 months. Bone window CT scan revealed extensive bony destructions of the skull base including the clivus, sphenoid sinus and medial aspect of the middle cranial fossa. Gd-DTPA MRI revealed an abnormal enhancement lesion in the sphenoid sinus, but no abnormal enhancement was seen in the sella turcica. Since these findings suggested malignant tumors of the cranial base, several biopsies through the transnasal route were carried out to confirm the diagnosis. This procedure caused the complication of cerebrospinal fluid (CSF) leakage. Because the biopsy specimen revealed a PRL producing adenoma (serum PRL-level 645.7 ng/ml), the patient was admitted to our department. On admission neurological examination showed an exophthalmos with external ocular movement disorders and disturbance of visual acuity on the left side. She underwent transsphenoidal surgery to remove the tumor and to reconstruct the sphenoid sinus and the sellar floor. Surgical exploration revealed a yellowish and soft tumor underneath the normal mucous membrane in the sphenoid sinus. The sellar floor was destructed extensively, but the dura mater of the pituitary fossa was intact except for a small pin-hole which was thought to be produced during the several biopsy procedures. No surgical procedure was applied to the intrasellar region. The sphenoid sinus was packed with a piece of fascia and fat applied with the aid of fibrin glue to prevent CSF leakage. RESULT: The patient followed a satisfactory postoperative course. Her visual acuity disturbance and exophthalmos disappeared one year after surgery. Postoperative serum PRL level remained high (66.9 ng/ml), but, subsequently, was normalized (9.5 ng/ml) with a bromocriptine therapy (15 mg daily). CONCLUSION: As far as we are aware, this is the first case report of an ectopic pituitary adenoma causing unilateral exophthalmos. Although it is extremely rare, pituitary adenomas should be kept in mind in a differential diagnosis of exophthalmos.

Aged↗
PubMed1982

Effect on exophthalmos of various methods of treatment of Graves' disease.

Patients with both exophthalmos and hyperthyroidism were treated with different modes of therapy for their hyperthyroidism. Propylthiouracil followed by surgery, propylthiouracil followed by radioactive iodine, propylthiouracil alone, and radioactive iodine alone were used. Some of the patients became hypothyroid and were made euthyroid with levothyroxine sodium. Based on the mode of therapy and whether or not hypothyroidism occurred, each patient was assigned to one of seven groups and followed up for 18 months or longer. Careful exophthalmometry was performed at six-week intervals in all patients. Though progression of exophthalmos was noted in all groups, the group that received propylthiouracil demonstrated the greatest progression of exophthalmos. In the group receiving sodium iodide l 131 therapy and in the group treated surgically, the rate of progression of exophthalmos was lessened with the development of hypothyroidism. Since these hypothyroid patients were made euthyroid with supplemental levothyroxine, it appeared that loss of thyroid tissue, rather than the hypothyroidism per se, was responsible for the decrease in progression of the exophthalmos. The continued progression of exophthalmos in the propylthiouracil-treated group may be related to effects of propylthiouracil on the immune system.

Exophthalmos↗
PubMed1982

Exophthalmos, global luxation, rapid weight gain: differential diagnosis.

Exophthalmos is an uncommon finding in the clinical setting; exophthalmos with global luxation is distinctly rare. Although the differential diagnosis of exophthalmos can be extensive, the most common causes are thyroid dysfunction and orbital neoplasia. We recently encountered a patient with exophthalmos and global luxation, the onset of which coincided with a weight gain of 45.5 kg (100 lb). An extensive evaluation failed to show any of the common states associated with either exophthalmos or global luxation. The ocular problems resolved with weight reduction only; the cause of both problems seems to have been the deposition of fat in the orbital fat pads. We found two previous reports of this condition in the older medical literature, with minimal clinical evaluations. Our patient was thoroughly studied to clarify the pathophysiologic characteristics of this unusual clinical appearance, and we reviewed the causes of exophthalmos and global luxation.

Body Weight↗
PubMed2006

[Familial primary pulmonary hypertension revealed by the association of bilateral chemosis, subacute myopia, and exophthalmos].

PURPOSE: To report a rare case of primary pulmonary hypertension (PPH) revealed by a relapsing chemosis, exophthalmos, and subacute myopia. PATIENTS AND METHODS: A 46-year-old woman presented with recurrent bouts of chemosis, exophthalmos, and subacute myopia. Her visual troubles were triggered by physical effort. She had a history of a longstanding high blood pressure that was noncompliant to all conventional hypotensive therapy. Automated refraction under cycloplegia showed recent myopia. At observation she had a bilateral, moderate chemosis. Hertel exophthalmometer measured a symmetric exophthalmos of 23 mm. Intraocular pressure was 20 mmHg OD and 24 mmHg OS. Eye fundus was strictly normal. RESULTS: Laboratory analysis showed no thyroid or inflammatory disease. HIV serology was negative as were antinuclear antibodies. Orbital CT confirmed the axial, symmetric exophthalmos. Echocardiogram brought out the diagnosis of PPH. Genetic studies by PCR localized the BMPR2 mutation that is characteristic of the familial type of PPH. Symptoms decreased under treatment. DISCUSSION: PPH is a rare disease caused by thickening of the arteriolar walls in the pulmonary bed, which results in a right-heart hypertension with venous stasis. Many authors have reported different posterior segment diseases secondary to PPH. However, anterior segment manifestations are extremely rare. These may be caused by a ciliary body congestion and retrobulbar vasodilatation. CONCLUSION: The association of chemosis, exophthalmos, and subacute myopia is unusual in PPH. We recommend keeping in mind the PPH as a differential diagnosis in those cases, especially in patients who suffer from high blood pressure.

Conjunctival Diseases↗