Acute pseudotumor of the orbit

A case is presented of a 22-year-old man with a history of bilateral eye injection, lacrimation, and rhinorrhea. The right orbit was inflamed and its globe proptotic, with medial and lateral gaze deficits. Tomography revealed swelling about the right lacrimal gland. He was admitted and treated with high-dose steroids, which caused regression of his symptoms. This presentation and response to steroids is pathognomonic for acute pseudotumor of the orbit, a condition rarely described in the emergency medicine literature. If left untreated, treudotumor of the orbit may progress to blindness and ophthalmoplegia.     

Noonan syndrome associated with central giant cell granuloma

We report a case of Noonan syndrome associated with central giant cell granuloma. The patient was a 101/2-year-old boy with the chief complaint of proptosis of the right eye. He also had various malformations such as short stature, webbed neck, pectus excavatum, cubitus valgus, pulmonary valve stenosis and patent foramen ovale, a characteristic face appearance and cryptorchidism and so on. Chromosome analysis showed a 46, XY karyotype. A computed tomographic scan and magnetic resonance imaging showed a mass originated from the lateral wall of the right maxillary sinus. The patient underwent Caldwell-Luc operation. Histological examination of the mass showed the characteristics of central giant cell granuloma. This case report describes a patient with the features of the recently described Noonan-like/multiple giant cell lesion syndrome.     

Blepharoptosis in association with ipsilateral adduction and elevation palsy A form of fascicular oculomotor palsy

A 16-year-old girl hospitalized with a sudden onset of blepharoptosis and diplopia revealed a combined paresis of the elevator palpebrae, the superior rectus, and the medial rectus muscles of the left eye as quantitatively demonstrated by the Hess chart and levator action test. A small midbrain lesion confined to the left cerebral peduncle and tegmentum, presumably by an ischemic or demyelinative process, was identified on magnetic resonance imaging. Despite uncertainty in its pathology, the incomplete oculomotor palsy of this case is certainly a consequence of oculomotor fascicular involvement in the intra-axial nerve root which initially fans out and then converges into the peripheral compact bundle. We speculate on the revised version of the oculomotor fascicular arrangements by which the neurophthalmologic features of the current case are better explained.     

Limited Wegener’s Granulomatosis Presenting with an Isolated Abduction Deficit

Wegener’s granulomatosis often affects the orbit, typically presenting with painful proptosis. The authors describe a 14 year-old girl, with limited Wegener’s granulomatosis, who initially presented with an isolated painless abduction deficit that spontaneously resolved over several weeks. She subsequently developed painful proptosis and diplopia, followed by facial and oral nodules. This case demonstrates that limited Wegener’s granulomatosis can rarely present with an isolated painless abduction deficit.     

Technique for modified transantral orbital decompression for improved cosmesis in stable thyroid eye disease

Functional and aesthetic rehabilitation of exophthalmos in stable thyroid eye disease (TED) can be achieved with a variety of surgical approaches. This article illustrates modifications of the classic transantral technique to provide a graded orbital decompression and achieve improved cosmesis. A retrospective chart review was performed of stable TED patients who elected to undergo the modified transantral decompression; illustrative cases are described. This modified transantral orbital decompression allows for graded orbital decompression surgery, adding to the range of treatment options for stable TED patients.     

The synkinesis between antero-posterior eye position and lid fissure width

We have investigated the antero-posterior position of the eye during normal blinking, gentle and hard lid closing and voluntary lid widening with 12 young subjects. Computer- assisted video analysis was used to measure relative eye position. Involuntary blinks were accompanied by retraction of the eye, which was of similar magnitude to the retraction measured witfi lids restrained, while the lids of the contralateral eye gently closed. Eye retraction is accentuated with hard voluntary lid closure, while voluntary lid fissure widening produced slight proptosis of the globe. Preliminary data suggests a small degree of instability of antero-posterior eye position between blinks. Our results are consistent with previous studies of the association between antero-posterior eye position and lid fissure width.     

Partial Removal of Orbital Tumor in Rosai-Dorfman Disease

Background Rosai-Dorfman disease is a rare idiopathic histiocytic proliferation disorder that typically presents with painless cervical lymphadenopathy. We report our experience with the management of a case of Rosai-Dorfman disease with compressive optic neuropathy.Case Rosai-Dorfman disease involving the bilateral orbital and paranasal sinuses was diagnosed in a 14-year-old boy. Diagnosis was based on the characteristic histopathologic features of sinus histiocytosis, composed of large, round S-100 protein-positive histiocytes with striking emperipolesis. The boy received chemotherapy to resolve the bilateral proptosis and compressive optic neuropathy in the right eye, but this treatment failed. Orbital debulking surgery using the Lynch approach was performed.Observations Corneal exposure was resolved and visual acuity recovered from 14/20 to 20/20 after partial removal of the tumor mass. There were no complications after surgery. During the 22 months of follow-up, orbital tumor masses redeveloped to cause lagophthalmos again, but did not cause visual impairment.Conclusions Rosai-Dorfman disease is a rare disorder, especially in Asia. The disease is usually chronic with spontaneous remission and is refractory to treatment. Partial removal of tumor masses is a workable way to improve visual acuity and correct corneal exposure. Before carrying out this procedure, we discussed with the parents of the patient the potential complications that might follow surgery and secured their permission before proceeding further. Jpn J Ophthalmol 2004;48:154–157 © Japanese Ophthalmological Society 2004     

Spontaneous Subperiosteal Hematoma Precipitated by Anxiety Attack

Abstract

A 60-year-old woman presented with diplopia and left periorbital edema and pressure, which developed during an anxiety attack the previous day. Examination revealed left inferotemporal globe dystopia, periorbital edema, ecchymosis, and limitation in supraduction. Orbital MRI confirmed the diagnosis of a superior subperiosteal orbital hematoma. The patient’s signs and symptoms rapidly resolved with administration of oral corticosteroids. The patient remains asymptomatic with complete resolution of orbital signs at 3-month follow-up. Subperiosteal orbital hematoma (SOH) is a rare condition in which blood accumulates between the bony orbit and separated periosteum, and is often due to blunt head trauma. Non-traumatic SOH (NTSOH) is exceedingly rare and usually associated with known coagulopathies or tendency to bleed. However, few cases of spontaneous NTSOH have been reported without any such predisposition and are thought to be caused by sudden elevations in intrathoracic and intracranial venous pressure such as vomiting, coughing, SCUBA diving, weight lifting and labor. We herein describe the presentation, radiography and outcome of a unique case of spontaneous NTSOH following an anxiety attack.     

Risk of dry eye after mullerectomy via the posterior conjunctival approach for thyroid-related upper eyelid retraction

INTRODUCTION : Mullerectomy by a conjunctival approach is an excellent way to reduce thyroid-related superior lid retraction. Especially the lateral horn of the levator, into the lacrimal gland, has to be reduced. Alterations in lacrimal secretion have been hypothesized, but never studied. MATERIALS AND METHODS : The basal and reflex Schirmer test and functional status were studied in 39 patients with thyroid-related orbitopathy after mullerectomy by a conjunctival approach (12 functional, 27 cosmetic indications). In 24 patients, the results could be compared with those of other measures carried out pre- or postoperatively or, in case of unilateral surgery, the operated and non-operated sides were compared. RESULTS : The Schirmer test was reduced in 7 of 12 functional cases and in 4 of 27 cosmetic cases. Although tear production was reduced, only two patients had to have increased dosages of artificial tears and two other patients required punctum plugs. Among the 12 functional indications, 10 showed an improved corneal surface. CONCLUSION : Lacrimal production may be reduced after mullerectomy by the conjunctival approach. Nevertheless, the clinical risk is low in comparison with the functional and cosmetic results of the procedure. At present, we try to identify and preserve the lacrimal ostia during surgery.