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Orbital pseudotumor: case report and overview.

BACKGROUND: The most common cause of a sudden onset of painful proptosis with diplopia in an otherwise healthy adult is orbital pseudotumor. However, there are other conditions that mimic this presentation and must be ruled out with laboratory testing and imaging studies. CASE REPORT: A 41-year-old Hawaiian man sought treatment for an acute, progressive, painful, left ophthalmoplegia without exophthalmos. During the next week, a loss of accommodation and associated pupillary reaction, decreased visual acuity, color vision deficits, and a paracentral scotoma developed. CT and MRI revealed a mass in the orbital apex. All systemic findings were negative, and high-dose systemic steroid therapy was initiated. Symptoms resolved within hours of the first dose, and signs were completely absent 1 month later. Follow-up MRI revealed complete absence of the previously noted mass. CONCLUSION: This is an atypical case of orbital pseudotumor, since there was no exophthalmos. It was diagnosed by clinical presentation, laboratory and imaging studies, and response to therapy.

Adult↗

Ethmoidal mucoceles.

Twenty ethmoidal mucoceles were treated and observed for a minimum of two years after surgery. Unilateral exophthalmos was the initial complaint in all cases and nasal polyposis was a noteworthy finding in more than half of the patients. The lesions always extended into the orbit and usually eroded the floor of the frontal sinus. Exenteration through an ethmoidectomy approach was successful in 16 cases. Failure was due to recurrence of the mucocele in one case and in three cases to uncontrolled polypoid disease that produced recurrence of the exophthalmos. Long-term follow-up is mandatory since recurrences may take several years to occur.

Adolescent↗

Distal osteolysis, short stature, mental retardation, and characteristic facial appearance: delineation of an autosomal recessive subtype of essential osteolysis.

We describe the concurrence of severe distal osteolysis, mental retardation, short stature, and characteristic facial appearance with maxillary hypoplasia and relative exophthalmos in two adult sibs, a 57-year-old woman and her deceased brother. Apparently they represent a distinct, autosomal recessive entity in the group of the so-called essential osteolysis. Furthermore, this observation confirms that the facial changes that may occur in patients with essential osteolysis, ie, maxillary hypoplasia and relative exophthalmos, may be present in all types of osteolysis, independent of their localisation or inheritance.

Female↗

Embolisation of orbital varix.

A 34-year-old man with intermittent exophthalmos, found to have a large varix in the right orbit, was treated by endovascular surgery. Percutaneous transfemoral venous catheterisation and embolisation of the orbital varix was performed on two occasions. A Tracker 18 microcatheter was introduced through the right inferior petrosal sinus, cavernous sinus, superior ophthalmic vein and then into the varix, following a guidewire. Superselective venography of the right ophthalmic vein showed the varix. A total of 204 platinum microcoils was used to pack the varix. At the time of discharge, the exophthalmos had largely resolved. As this technique is much less invasive than surgical resection, we recommend an attempt at endovascular embolisation of orbital varices prior to surgical removal.

Adult↗

Schwannomas of the orbit.

The authors describe a case of intraorbital schwannoma. The tumour caused exophthalmos of 11 mm, and displacement with limitation of mobility of the eyeball. A review of cases from other studies is presented. This rare tumour orginates from the fifth cranial nerve. Exophthalmos is the primary clinical symptom. The value of several radiological examination techniques and possible surgical approaches are discussed.

Aged↗

[Orbital decompression in treatment of endocrine orbitopathy].

Graves' ophthalmopathy (Graves' disease) is a grave, rare immunological inflammatory reaction of the postorbital connective, adipose and ocular muscle tissue. Graves' disease occurs only within the scope of immunothyropathies and constitutes the most frequently encountered extrathyroidal manifestation. Typical symptoms are a result of the volume increase of the postbulbar connective and adipose tissue and of the interstitial swelling of the ocular muscles. Clinically, we find a spectrum of increasingly grave changes in the orbita, such as infiltration of the eyelid and connective tissue, exophthalmos, swelling of the muscles, damage to the cornea and involvement of the optic nerve with loss of vision. Regarding functional impairment of the optic nerve (optic nerve compression), orbita decompression represents an operative ultima ratio. Between January 1992 and April 1997, 11 patients (22 orbitae) from a group of more than 600 patients with Graves' disease with vision involvement were treated surgically. All treatment data were documented prospectively. Surgical intervention was performed only in cases where a progressive loss of vision existed in spite of retrobulbar irradiation and high-dose glucocorticoid therapy. Surgical therapy consisted of decompression of the medial, inferior and lateral orbital wall and of the orbital contents via combined bi-coronary and anterior access. The long-term results demonstrated improved vision conditions with 17 of the 22 eyes operated on. Parameters such as vision, exophthalmos, VEP, motility, double vision and field of vision were documented pre- and post-operatively. The prediction of post-operative deviations of the bulb axis was limited, and these were rectified through secondary displacements of the ocular muscle. No complications worth mentioning were encountered. In extreme events of therapy-resistant Graves' ophthalmopathies with progressive loss of vision, the three-wall decompression method has proved to be the correct one.

Adult↗

The diagnosis and prognosis of atypical carotid-cavernous fistula (red-eyed shunt syndrome).

Nineteen patients who complained of red eyes had the characteristic clinical signs of an atypical carotid-cavernous fistula. Episcleral veins were dilated, intraocular pressure and episcleral venous pressure were high, and blood filled Schlemm's canal during gonioscopy. Most of the patients had mild exophthalmos, but in none was the exophthalmos obviously pulsatile, and in only two patients could a bruit be heard. Orbital echography disclosed either a dilated superior ophthalmic vein or congestion of the orbital soft tissues. Selective carotid angiography, done in seven patients, disclosed the fistula to be a dural-cavernous fistula. The serious complication of the fistulas was open-angle glaucoma resulting from the high episcleral venous pressure. None of the fistulas was treated surgically, but six closed spontaneously and three closed soon after carotid angiography.

Adolescent↗

Three-wall orbital decompression superiority to 2-wall orbital decompression in thyroid-associated ophthalmopathy.

PURPOSE: We evaluated the results of the 2-wall and 3-wall orbital decompression in patients with Graves' disease. PATIENTS AND METHODS: In this study, we present a consecutive series of 12 patients (18 orbits) who were submitted to orbital decompression by endoscopic transnasal medial wall combined with transantral inferior wall approach and 7 patients (8 orbits) who were submitted to orbital decompression by endoscopic transnasal medial wall, transantral inferior wall combined with transcutaneous lateral wall approach. The degree of exophthalmos was evaluated with the Hertel exophthalmometer preoperatively and postoperatively in the 24th hour, and first, third, and ninth months. RESULTS: At the end of the third month, the exophthalmos decreased by a mean of 4.38 mm (range, 3 to 7 mm) with the 2-wall decompression and 7.75 mm (range, 5 to 12 mm) with the 3-wall decompression. Visual acuity maintained or improved during the follow-up period. Ocular motility disturbance occurred in 1 patient after 2-wall decompression and in 1 patient after 3-wall orbital decompression. Postoperatively, new-onset diplopia was seen in only 1 patient after 2-wall orbital decompression. CONCLUSION: The 3-wall (endoscopic transnasal medial wall, transantral inferior wall combined with transcutaneous lateral wall) approach is as safe as the 2-wall approach. Proptosis reduction is much better with the 3-wall orbital decompression.

Adult↗

Early versus late orbital decompression in Graves' orbitopathy: a retrospective study in 125 patients.

PURPOSE: To determine if early rehabilitative orbital decompression in Graves' orbitopathy (GO) leads to a more effective postoperative outcome than the same intervention performed at a later, more likely, fibrotic stage. DESIGN: Retrospective comparative case series. PARTICIPANTS: The medical records of all GO patients treated with a 3-wall orbital decompression at our institution between 1990 and 2000 were reviewed retrospectively. Only patients operated bilaterally for aesthetic rehabilitation, without preoperative diplopia, were included. They were divided into group 1 (duration of GO < 4 years) and group 2 (duration > or = 4 years). METHODS AND MAIN OUTCOME MEASURES: The 2 groups were compared for demographics, smoking habits, preoperative characteristics (immunosuppressive treatments, Hertel values, score in NOSPECS [no signs or symptoms, only signs, soft tissue involvement with symptoms and signs, proptosis, extraocular muscle involvement, corneal involvement, sight involvement] class 2, degree of extraocular muscle enlargement), and surgical outcome (mean reduction of exophthalmos, symmetry of exophthalmos reduction, reduction in upper and lower lid retraction, any persistent periorbital swelling requiring cosmetic eyelid surgery, postdecompression diplopia). RESULTS: The medical records of 125 of 376 patients were selected for this study. There were no differences between group 1 (n = 70) mean GO duration (2.2+/-0.8 years) and group 2 (n = 55) mean GO duration (9.0+/-5.4 years) with respect to demographics, smoking habits, and preoperative characteristics except for the degree of extraocular muscle enlargement, which was significantly greater in group 1 (P = 0.039). There was no difference in surgical outcomes between the 2 groups, with the exception of postdecompression diplopia, which was significantly more frequent in group 1 than in group 2 (29% vs. 13%, P = 0.033). CONCLUSIONS: In GO, early rehabilitative orbital decompression does not improve surgical outcome and is associated with a higher risk of postdecompression diplopia.

Adult↗

Diagnostic criteria for Graves' ophthalmopathy.

PURPOSE: To propose criteria for the diagnosis of Graves' ophthalmopathy. METHODS: We reviewed the evolution of nomenclature describing Graves' ophthalmopathy. and the diagnostic schema used in key published reports. RESULTS: A laboratory test or clinical finding pathognomonic for Graves' ophthalmopathy currently is not available or recognized. Extant diagnostic criteria may exclude appropriate cases. CONCLUSIONS: Graves' ophthalmopathy is considered to be present if eyelid retraction occurs in association with objective evidence of thyroid dysfunction or abnormal regulation, exophthalmos, optic nerve dysfunction, or extraocular muscle involvement. The ophthalmic signs may be unilateral or bilateral, and confounding causes must be excluded. If eyelid retraction is absent, then Graves' ophthalmopathy may be diagnosed only if exophthalmos, optic nerve involvement, or restrictive extraocular myopathy is associated with thyroid dysfunction or abnormal regulation and if no other cause for the ophthalmic feature is apparent.

Eye Diseases↗

Ophthalmoplegia resulting from an intraorbital hematoma.

BACKGROUND: A case is described in which an intraorbital hematoma was found to complicate recovery from attempted aneurysm clipping 5 days into the postoperative period. The etiology, management, and complication avoidance are discussed. CASE DESCRIPTION: Five days after attempted surgical clipping of an internal carotid artery aneurysm via a frontotemporal craniotomy with orbital osteotomy, a patient underwent coiling of the aneurysm. Shortly after the endovascular procedure, the patient developed exophthalmos and ophthalmoplegia involving the right side followed by decline in her level of consciousness. An emergency computed tomography (CT) scan revealed an epidural hematoma with intraorbital extension. After evacuation of the hematoma, the patient recovered extraocular function and returned to her baseline mental status. CONCLUSION: Exophthalmos and ophthalmoplegia in a patient recovering from cranial surgery using skull base techniques warrants immediate attention, especially after endovascular procedures. Delay in intervention may result in loss of neurologic function or life. The authors discuss the relevant literature and management of this uncommon complication.

Cerebral Angiography↗

Treatment of a sphenoid sinus mucocele using an endoscope combined with a navigating system: a case report.

A sphenoid sinus mucocele extending into the middle cranial fossa and the pterygomaxillary fissure was decompressed using an endoscope in combination with a navigation system. A 59-year-old woman was admitted with a 3 month history of retro-ocular pain and right sided exophthalmos. To evaluate the cystic lesion, a fibreoptic endoscope was inserted into the cyst through an erosive defect in the temporal bone. The histopathological appearance of cyst wall tissue confirmed the diagnosis of mucocele. After 4 months, the mucocele had refilled and the exophthalmos had recurred. At reoperation, the cyst was fenestrated using a rigid endoscope combined with a navigation system inserted via a sublabial transmaxillary approach. The proper site of cyst fenestration was precisely determined using the navigation system, permitting complete evacuation of this large lesion with minimal invasiveness.

Endoscopy↗

[Ophthalmo-rhinosurgical decompression operation in malignant endocrine exophthalmus].

The thyroid metabolism has to be treated first. If this is not followed by regression of the exophthalmos, treatment with prednisone over a period of several weeks is indicated. If this does not result in permanent improvement, surgical decompression of the orbit is indicated, especially if the exposition of the cornea or compression of the optic nerve represent an imminent danger. In such cases surgical decompression is preferable to multiple series of prednisone or radiation treatment (Wright 1981, Trokel 1982). Both the Krönlein-Berke operation for resection of the lateral orbital wall as well as neurosurgical resection of the orbital roof have proved to be excellent for removal of correspondingly located orbital tumors; effective decompression of the orbit in malignant endocrine exophthalmos, however, cannot be achieved this way, as the temporal muscle and the brain, respectively, prevent protrusion of the swollen orbital tissues through these apertures. Resection of the orbital floor is somewhat more effective. The mucosa of the maxillary sinus can easily be moved by the protruding orbital tissues, resulting in a reduction in the size of the maxillary sinus. This decompression, however, is effective only in the anterior two-thirds of the orbit, while the optic nerve must be relieved from the pressure of the swollen muscles in particular in the posterior one-third of the orbit. The only effective way is to remove the medical orbital wall up to the tip of the orbit. Surgical techniques for this purpose have already been recommended, but appeared unsatisfactory from a rhinological point of view.(ABSTRACT TRUNCATED AT 250 WORDS)

Graves Disease↗

Results of orbital decompression in Taiwan.

Orbital decompression was performed on 116 orbits with Graves' ophthalmopathy. The indications for decompression were dysthyroid optic neuropathy (DON), recalcitrant corneal exposure (EXP) and disfiguring exophthalmos (COS). All cases but one (in the DON group) had improved or unchanged vision. The average retinal sensitivity improvement in the DON group was 6.7 +/- 6.1 dB and 85% had a significant retinal sensitivity improvement (>5 dB). The average retroplacement effect was 4.4 +/- 2.1 mm and only five cases (7%) had postoperative asymmetry of more than 2 mm by Hertel's exophthalmometry. The most frequent sequela was diplopia, which tended to occur in more severely myopathic eyes. In our series, 21% (10/48 cases without preoperative diplopia) developed diplopia after decompressive surgery. Hypoglobus is another complication, noted in two cases, which was successfully repositioned. In conclusion, decompressive surgery is a safe and effective procedure to restore vision and reduce exophthalmos in Graves' ophthalmopathy. Careful evaluation of clinical parameters, individualization of surgical goals and intraoperative titration of the retroplacement effect are the key to optimal results.

Journal Article↗

Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease.

Erdheim-Chester disease (ECD) is a rare multisystem histiocytosis syndrome of unknown cause that usually affects adults. Histiocytic infiltration of multiple end organs produces bone pain, xanthelasma and xanthoma, exophthalmos, diabetes insipidus, and interstitial lung disease. Differential diagnosis includes Langerhans cell histiocytosis, metabolic disorders, malignancy, and sarcoidosis. ECD can be diagnosed using a combination of clinical and histopathologic findings. Sites of involvement include lung, bone, skin, retroorbital tissue, central nervous system, pituitary gland, retroperitoneum, and pericardium. Symmetrical long bone pain with associated osteosclerotic lesions, xanthomas around the eyelids, exophthalmos, and/or diabetes insipidus suggest ECD. Approximately 35% of patients have associated lung involvement, characterized by interstitial accumulations of histiocytic cells and fibrosis in a predominantly perilymphangitic and subpleural pattern. This pattern distinguishes ECD from other histiocytic disorders involving the lung. The diagnosis is confirmed by tissue biopsies that contain histiocytes with non-Langerhans cell features. In general, the clinical course of patients with this disease varies, and the prognosis can be poor despite treatment. Clinical trials for treatment of ECD have not been conducted and treatment is based on anecdotal experience.

Adult↗

Monobloc distraction osteogenesis in pediatric patients with severe syndromal craniosynostosis.

The management of the hypoplastic midface in syndromic craniosynostosis remains a great challenge. Frequently, patients have to be operated on numerous times to achieve a satisfactory end result, partially because of the limited skeletal advancement possible when using traditional surgical techniques. During the last decade, however, methods for gradual midfacial distraction have been presented, whereby greater advancements can be obtained. We present four children aged 17 months to 15 years with severe syndromal craniosynostosis in need of midface advancements because of severe respiratory obstruction or severe exophthalmos. These patients were complex cases with several previous craniofacial surgeries (mean of three times, range of two to six times) that yielded insufficient skeletal advancements. They were operated on with gradual monobloc advancements using the Modular Internal Distraction System. The mean length of operations was 370 minutes (range: 240-455 minutes), and the mean amount of perioperative blood transfusion needed was 1,300 ml (range: 280-2,700 ml) or 66.9 ml/kg (range: 31.1-94.9 ml/kg). The patient with the greatest number of previous operations also had the longest operation time as well as the most blood loss. The average midface advancement obtained was 25 mm (range: 20-30 mm), resulting in cessation or a significant decrease of preoperative respiratory problems, reduced exophthalmos, and improved facial profile. Apart from a local infection in one patient with a connective tissue disorder and several previous wound infections, no major postoperative complications were recorded. Distraction osteogenesis has become a versatile and safe technique that allows for large advancements of the midface.

Adolescent↗

Computed tomographic findings of distensible orbital venous anomalies.

PURPOSE: To describe the CT findings of distensible orbital venous anomalies. METHODS: We reviewed the records of 17 consecutive patients who met the imaging criteria for orbital venous anomalies. We analyzed the clinical data and the CT scans qualitatively and quantitatively. RESULTS: Distensible venous anomalies usually occupied both intraconal and extraconal spaces of the orbit (65%). The contour of the mass was ovoid or fusiform in 3 of the orbits (18%) and tubular or lobular in 14 orbits (82%). The optic nerve was encased by the mass in 8 orbits. Changes in the adjacent bone were detected in 9 orbits. The average volume of the venous anomalies was 1.1 mL in the axial scan and 5.7 mL in the coronal scan. The patients who showed clinically evident exophthalmos on the Valsalva maneuver had larger volumes than those who did not. CONCLUSIONS: The orbital structures adjacent to the distensible orbital venous anomalies were vulnerable to intermittent compression. CT scans, including both postcontrast axial and coronal images, were useful in demonstrating the presence of distensible venous anomalies, even in cases with no apparent exophthalmos on the Valsalva maneuver.

Adolescent↗

Graves' disease manifesting after maintenance lithium.

A 36-year-old woman with no prior history of thyroid dysfunction developed Graves' disease (hyperthyroidism with exophthalmos) 4 months after her maintenance lithium therapy was stopped. Although a causal relationship between these events cannot be established, several hypotheses are presented. The possibility that lithium may mask the hyperthyroidism of Graves' disease but allow the exophthalmos to develop cryptically is discussed. Hyperthyroidism and mania have similar symptoms, but in the case presented two psychiatrists were able to distinguish between these two conditions by careful examination.

Adult↗