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The case of a 64-year-old man who presented with unilateral pulsatile exophthalmos is described. A defect in the supero-medial wall of the orbit and an extraconal low density mass were demonstrated by orbital radiographs and CT. Positive contrast CT cisternography confirmed the diagnosis of a leptomeningeal cyst.
In a 20-year-old man with a history of sinonasal polyps, bilateral ophthalmoplegia, exophthalmos, and optic nerve dysfunction developed. Radiologic imaging showed a polypoid mass filling the nose and sinuses, eroding into the pituitary fossa, basal cisterns, and orbits, and compressing the cavernous sinuses. Six months after bilateral maxillary, ethmoid, and sphenoid exploration, ocular motility returned to normal, but there remained mild generalized visual field loss in the right eye. The pathologic specimen showed polypoid respiratory mucosa with acute and chronic inflammation as well as eosinophiles.
Exophthalmos is the most commonly measured sign of Graves ophthalmopathy, whereas enlargement of the extraocular muscles is the principal pathologic abnormality. The purpose of this article is to explore possible etiologies of increased volume of orbital fat and of proptosis in patients with no substantially increased total extraocular muscle volume. Computed tomographic scans of the 13 orbits reviewed in this study had the following characteristics in common: a fine, reticular pattern within the orbital fat, a prominent superior ophthalmic vein, and an enlarged superior rectus muscle. Quantitative analysis revealed that superior rectus muscle volume showed a statistically significant correlation with proptosis, whereas medial, lateral, and inferior rectus muscle volumes did not correlate with proptosis. Based on anatomic considerations, the authors postulate that superior rectus muscle enlargement alone may produce reduced venous outflow from the orbit, thereby expanding the apparent orbital fat volume and producing proptosis.
Erdheim-Chester disease is a rare visceral xantho-granulomatosis, the 17th case of which is reported here. The initial symptom, bilateral exophthalmos, was uncommon. The picture was completed by a retroperitoneal xanthogranuloma and by bilateral and symmetrical osteosclerosis of the long bones. The lack of X-bodies at electron microscopy differentiated the disease from Hand-Schüller-Christian disease. The patient's condition improved with chemotherapy (vinblastine and doxorubicin) combined with corticosteroid therapy. After a 5-month remission period, he died of an intercurrent infection. No autopsy was performed.
We describe a 58-year-old woman who presented with bilateral ophthalmoplegia, exophthalmos, and headache and was found to have retrograde internal jugular vein flow secondary to a high-grade obstruction of the ipsilateral brachiocephalic vein from a previous hemodialysis catheter placement. The patient had also a high-flow dialysis graft in the ipsilateral arm. The cranial and extracranial venous system congestion resolved, and the signs disappeared soon after a balloon angioplasty and stent placement at the level of the obstruction.
Exophthalmos and exorbitism, although both manifesting protrusion of the eye, differ in their aetiology and treatment. Illustrative cases are presented from both catagories, with a short experimental section illustrating on the degree of orbital expansion obtained with various procedures. Methods for accurately measuring orbital volume clinically still require development.
A patient with pulsating exophthalmos is presented. He had facial asymmetry, multiple cutaneous café-au-lait spots and a subcutaneous nodule, biopsy of which showed features consistent with neurofibromatosis. Radiographs of the orbit showed a deficiency of the superolateral orbital wall, a feature previously recognized as part of neurofibromatosis. This feature probably occurs as a result of mesodermal dysplasia. The differential diagnosis and management are discussed.
BACKGROUND/PURPOSE: Postoperative diplopia remains a significant complication of orbital decompression in dysthyroid orbitopathy. This study evaluated the results of orbital decompression treatment using a transforniceal approach to sculpt the lateral orbital deep bone area. METHODS: The two areas of bone in the deep lateral orbit (the basin of the inferior orbital fissure and the sphenoid door jamb) were exposed using a transforniceal swinging eyelid approach. An electric drill was used to sculpt these deep bone areas of the lateral orbit, and approximately 1 mL intraconal fat was removed simultaneously. Between October 1999 and March 2003, transforniceal lateral deep bone decompression was performed in 35 consecutive patients (62 orbits) with disfiguring dysthyroid orbitopathy. Data on proptosis reduction effect, new-onset diplopia and other complications of lateral wall decompression were analyzed. RESULTS: The average preoperative Hertel value was 21.2 +/- 1.3 mm (range, 18-23 mm) and decreased to 17.4 +/- 1.2 mm (range, 15-19.5 mm) postoperatively. The mean decrease in proptosis 3 months postoperatively was 3.8 +/- 0.91 mm (range, 1.5-4.6 mm). New-onset downgaze diplopia occurred in two (5.7%) of the 35 patients. Persistent trigeminal paresthesia was noted in one patient (2.8%). No cerebrospinal fluid leak, globe injury or vision deterioration was noted during 9.5 +/- 1.7 months of follow-up. The cosmetic appearance was improved in all patients after surgery. CONCLUSION: Transforniceal lateral deep bone decompression produces less new-onset, persistent diplopia than traditional inferomedial wall decompression, and provides good cosmesis by using a hidden small incisional wound. This approach appears to be a safe and effective procedure for patients with disfiguring exophthalmos, especially for Asian patients without crease fold.
A modified surgical technique for the treatment of exophthalmos caused by Graves' disease or meningioma is presented. It is based on the orbital expansion as presented by Tessier (1969). It consisted of a lateral marginotomy with extension to the superior and inferior orbital rim and removal of the great wing of the sphenoid bone. The osteotomised orbital rim segment was advanced and stabilised by interposition of a calvarial bone graft. Concomitant lipectomy was carried out when indicated. Seven patients were operated on. No major complications occurred. The advantages of the method are discussed.
We report a case of vasculitis causing facial swelling and exophthalmos which on clinical and histological grounds is thought to be limited Churg-Strauss syndrome. An excellent response was achieved to high doses of systemic steroids and cyclophosphamide.
Exophthalmos is most commonly due to Graves' ophthalmopathy (GO), the most frequent extrathyroidal manifestation of Graves' disease. The clinical signs and symptoms of GO are the consequences of increased orbital connective and adipose tissue volume, and of interstitial enlargement of extraocular muscles, within the confines of the bony orbits. Diagnosis of GO is usually readily established by obtaining a careful history, detailed clinical examination of the thyroid gland, eyes and orbits, few laboratory tests and ultrasonography of the thyroid gland and the orbital contents. Additional procedures such as computed tomography or magnetic resonance imaging are rarely needed. A team approach (general practitioner, endocrinologist, ophthalmologist) is essential to assure state-of-the-art management of patients with GO, and to properly select the best choices from a still limited range of therapeutic options.
Communication concerning the diagnosis and treatment of noninflammatory vascular exophthalmos: (1) 12-year-old boy suffering from a 4 mm proptosis on the left eye, and a hemangioma of the left upper lid. Computerized tomography showed an orbital tumor whose dilated vessels extended into the foramen opticum. Since the patient was amblyopic the treatment was merely cosmetic, consisting in diathermic puncture of the lid hemangioma. -(2) 45-year-old woman suffering from an axial proptosis of the right eye. Computerized tomography showed a retrobulbar tumor compressing the optic nerve. Surgical removal of a cavernous hemangioma by the neurosurgeon led to restitutio ad integrum. -(3) 8-year-old boy with intermittent exophthalmus on the left. No tumor detectable in routine examinations. External carotid angiography finally revealed an arteriovenous shunt between the A. maxillaris and the V. orbitalis superior. -These three descriptions of patients suffering from exophthalmus illustrate currently available possibilities of ophthalmologic and neurosurgical collaboration for diagnosis and treatment.
Graves' disease is an autoimmune disorder of the thyroid gland with characteristic peripheral manifestations. The most common clinical findings include ophthalmopathy in 30% of patients, dermopathy (pretibial myxedema) in 4% of patients, and thyroid acropachy in 1% of patients. The triad of exophthalmos, pretibial myxedema, and acropachy occurs in less than 1% of patients. We present a case of Graves' disease with the clinical triad of eye disease, dermopathy, and acropachy.
Skull base venous fistulas are rare. A 15-year-old boy was stabbed in the left side of his neck, just below the mastoid process. Two years later, he presented with a protruded and pulsating left eye with red sclera. A soft murmur was audible all over his head and neck. Angiography revealed an arteriovenous fistula between the third portion of the vertebral artery (V3) and the vertebral venous plexus, as well as the ipsilateral jugular vein (VVJF). The fistula recruited several arterial feeders and rising the venous pressure along the outflow system of the skull base had led to unilateral exophthalmos.
Encephaloceles are rare congenital defects in the skull, through which the meninges and brain tissue have herilated. We report a case of an 11-year-old boy with a spheno-orbital encephalocele that presented with pulsatile exophthalmos. A two-stage operation resulted in a good outcome.
In the last few years, various surgical techniques to correct orbital decompression in patients with Graves ophthalmopathy have been proposed. In those patients in whom a high degree of exophthalmos is mainly the result of the hypertrophied endorbital muscles with respect to endorbital fat hypertrophy, lipectomy is insufficient to obtain an acceptable increase of endorbital volume. The authors propose a method that provides for the monobloc bilateral quadrantotomy by extracranial access. Despite traditional methods of decompression, the advantage of this technique is better advancement of the orbital margins. The bicoronal and endoral incisions guarantee no facial scars. Compared with the craniotomy, the extracranial access is also less stressful for the patient.
A 38-year-old physician presented with a 9-month history of progressive self-administration of oral prednisone < or = 160 mg per day for Addison's disease. Examination demonstrated typical Cushingoid features and bilateral proptosis with elevated intraocular pressure. Computed tomography disclosed increased intraorbital adipose tissue. We hypothesize that the increased intraorbital adipose deposition was due to the differential binding of glucocorticoids to adipose tissue receptors and an enhancement of lipoprotein lipase activity. We conclude that the findings in this case may be related to glucocorticoid-induced changes in the ocular and periorbital structures. Cushing's syndrome should be considered in the differential diagnosis of acquired exophthalmos and elevated intraocular pressure and findings of increased orbital fat on orbital imaging.
It is believed that a variation of the 3-wall orbital expansion of Tessier can be used to correct persistent exophthalmos, as well as exorbitism. The operation is described, and the results in 3 patients are shown.