Increased elastin expression in astrocytes of the lamina cribrosa in response to elevated intraocular pressure.

PURPOSE: To determine whether abnormal elastin synthesis in the glaucomatous optic nerve head and lamina cribrosa is due to elevated intraocular pressure (IOP) or secondary to axonal injury, monkeys with elevated IOP and with optic nerve transection were compared. METHODS: Unilateral, chronic elevated IOP was induced in 11 rhesus monkeys by laser scarification of the trabecular meshwork. IOP was monitored weekly and maintained within 25 to 45 mm Hg for 7 to 36 weeks. In 6 monkeys, unilateral, optic nerve transection was performed, and monkeys were killed after 4 weeks. Optic nerve damage was assessed by stereoscopic slit-lamp biomicroscopy and fundus photography and by confocal scanning laser ophthalmoscopy. The eyes were enucleated and processed for immunohistochemistry and in situ hybridization and for electron microscopic immunogold detection of elastin. Axonal loss was evaluated in cross sections of the optic nerve stained with phenylenediamine. RESULTS: Compared with normal contralateral controls, the lamina cribrosa of eyes with elevated IOP exhibited markedly increased elastin and the presence of elastotic aggregates in the extracellular matrix and upregulation of elastin mRNA in the astrocytes. In transected eyes, elastin appeared as fine fibers in the lamina cribrosa, without elastotic aggregates, and without new synthesis or abnormal deposition of elastin. At the transected site, new synthesis of elastin was present in the pia mater but not in astrocytes in the glial scar. CONCLUSIONS: This study demonstrates that abnormal elastin synthesis in experimental glaucomatous optic neuropathy in the monkey is specific to elevated IOP and not secondary to axonal loss. The mechanisms by which elevated IOP induces enhanced elastin synthesis in laminar astrocytes are unknown but differ from those involved in acute axonal injury such as transection, where inflammation and breakdown of the blood-nerve barrier occur.

Treacher Collins syndrome: phenotypic variability in a family including an infant with arhinia and uveal colobomas.

We report extreme expression of Treacher Collins syndrome in an infant with arhinia, anotia, absent zygomatic bones, hypoplastic mandibular rami, and bilateral coloboma of iris, choroid plexus, and optic nerves. The Treacher Collins phenotype was mildly expressed in the mother and moderately in the sister. The father had no signs and was not ruled out as the father by DNA fingerprinting, thus making homozygosity by descent in the severely affected son very unlikely.

Optic nerve avulsion.

OBJECTIVE: To characterize the presentation, role of diagnostic imaging, and course in patients with optic nerve avulsion. METHODS: A retrospective review of medical records of all 6 patients with optic nerve avulsion who were seen at the Massachusetts Eye and Ear Infirmary, Boston, from January 1, 1991, to July 31, 1995. RESULTS: The initial visual acuity ranged from 20/100 to no light perception. All 6 patients underwent neuroimaging, including computed tomography, magnetic resonance imaging, or both. B-scan ultrasonography was performed on 4 patients, and the condition of 1 patient was evaluated with color Doppler ultrasonography to assess the optic nerve vasculature. In 1 patient, a computed tomographic scan was suggestive of an optic nerve avulsion. Neuroimaging in the other 5 patients, including 2 patients who underwent magnetic resonance imaging, failed to demonstrate an avulsion. During a follow-up period of up to 25 months, 4 patients showed no improvement in visual acuity, 1 patient improved from no light perception to bare light perception, and 1 patient improved from 20/100 to 20/25. CONCLUSIONS: These data suggest that final visual outcome was dependent on initial postinjury visual acuity. Neuroimaging, B-scans, and Doppler ultrasonography were usually not helpful in establishing the presence of optic nerve avulsion, although they may be useful in evaluating comorbid conditions.

Ancient schwannoma of the orbit.

The ancient schwannoma is a rare variant of a neurilemoma with a course typical of a slow-growing benign neoplasm. Histologically, it can be confused with a malignant mesenchymal tumor because of increased cellularity, nuclear pleomorphism, and hyperchromatism. Despite the degree of nuclear atypia, mitotic figures are absent. We describe the clinical and histopathologic features of an ancient schwannoma of the orbit.

Endogenous Pseudallescheria boydii endophthalmitis. Clinicopathologic findings in two cases.

Two cases of endogenous Pseudallescheria boydii endophthalmitis are presented. One patient had severe pulmonary fibrosis but no history of ocular trauma and no clinical or laboratory evidence of immunocompromise. Despite therapy with repeated intravitreal miconazole nitrate injections and systemic fluconazole, enucleation of the globe was required, and the patient eventually died with disseminated pseudallescheriasis. The other patient was an immunosuppressed cardiac transplant recipient who also received systemic fluconazole therapy. The infected eye eventually required evisceration, but there was no evidence of disseminated pseudallescheriasis before his death of unrelated causes.

Protection of ganglion cells in experimental glaucoma by retinal laser photocoagulation.

OBJECTIVE: To test a hypothesis of photoreceptor involvement in retinal ganglion cell (RGC) death in chronic glaucoma. METHODS: Laser spots were applied to 6 eyes of 3 rhesus monkeys, causing focal destruction of the outer retina, including the photoreceptors. After 3 to 4 weeks, experimental glaucoma was induced in the right eyes of each monkey using argon laser trabecular destruction (ALTD). The intraocular pressures in these eyes were elevated for 3 to 7 months. As a control, 1 additional monkey underwent retinal laser photocoagulation followed by optic nerve transection instead of ALTD. Following enucleation, the retinas were embedded and sectioned for histologic evaluation. RESULTS: There was extensive loss of RGCs in the eyes with ALTD except over the large retinal laser spots, where there was an increased survival of RGCs. The RGC protection was not observed in the monkey that had undergone optic nerve transection. CONCLUSION: Photocoagulation of the outer retina that completely destroys the photoreceptors results in survival of the overlying RGCs in experimental glaucoma in monkey eyes. CLINICAL RELEVANCE: Although this is an experimental model and not a therapeutic option, these results suggest that treatments other than lowering intraocular pressure may be potential therapies for preventing RGC death in glaucomatous eyes. Arch Ophthalmol. 2000;118:1242-1250

Primary orbital Ewing sarcoma in a middle-aged woman.

A 43-year-old woman had unilateral exophthalmos caused by primary orbital Ewing sarcoma. Specialized immunohistochemical stains, primarily MIC-2 (CD99), aided in the diagnosis of Ewing sarcoma. Twenty-two months after radiotherapy and multiagent chemotherapy, the patient remained tumor free. To our knowledge, this is the first reported case of orbital Ewing sarcoma to present in an adult beyond the fourth decade of life.

Small incision external levator repair: technique and early results.

PURPOSE: To describe a new surgical technique and early results of external levator repair performed through a small skin incision. METHODS: A chart review of consecutive patients undergoing small incision external levator repair was conducted. This modified external levator repair was performed through an 8-mm eyelid crease incision. Patients with unilateral or bilateral aponeurogenic blepharoptosis were candidates for the technique. Patients with excessive horizontal upper eyelid laxity and those requiring blepharoplasty in addition to blepharoptosis surgery did not undergo this technique. Patients who underwent previous upper eyelid surgery or concurrent brow surgery were excluded from the review. Preoperative measurements included upper eyelid margin reflex distance, levator function, and degree of dermatochalasis, as well as Goldmann visual field results. Outcome measures included incidence and type of intraoperative complications, postoperative upper eyelid position (including margin reflex distance, eyelid contour, and symmetry), incidence and type of postoperative complications, and revisions or additional necessary surgery. RESULTS: Twenty-eight eyelids of 17 patients met study inclusion criteria. Preoperative margin reflex distance +/- SD averaged 0.8 +/- 0.4 mm. Average length of follow-up was 28 +/- 5 weeks (range, 15 to 52 weeks). No significant intraoperative complications occurred. Postoperative margin reflex distance averaged 3.7 +/- 0.3 mm. Two eyelids were mildly undercorrected, and one demonstrated moderately peaked contour postoperatively. Satisfactory eyelid position and contour were achieved in 25 of 28 treated eyelids. No patient elected reoperation. CONCLUSIONS: Early results demonstrated that small incision levator repair is safe and generally effective. This minimally invasive external levator repair is useful for a carefully selected subset of patients with aponeurogenic blepharoptosis.

Hydrops and spontaneous perforation in pellucid marginal corneal degeneration.

PURPOSE: Pellucid marginal degeneration of the cornea (1,2) is an idiopathic condition characterized by noninflammatory, nonulcerative thinning of the inferior, peripheral cornea. Occasionally the condition is complicated by acute corneal hydrops (3). We describe a patient with findings suggestive of pellucid marginal corneal degeneration who experienced acute hydrops and spontaneous perforation of the cornea. METHOD: A retrospective analysis of the case was conducted. RESULTS: A 56-year-old woman was seen with decreased vision in the left eye. Clinical findings, including computerized corneal topography, were suggestive of pellucid marginal degeneration. Two weeks later, the patient had hydrops and spontaneous perforation of the left cornea. Medical workup of the patient and histopathologic and microbiologic studies of the corneal specimen revealed no contributory disease process. The patient underwent emergency tectonic lamellar keratoplasty and is doing well after subsequent optical penetrating keratoplasty. CONCLUSION: We believe this is the first report of a case of pellucid marginal degeneration of the cornea complicated by hydrops and perforation.

Optic nerve decompression for compressive neuropathy secondary to neoplasia.

OBJECTIVE: To evaluate the outcome of extracranial optic nerve decompression in patients with compressive optic neuropathy secondary to intracranial, paranasal sinus, or orbital neoplastic processes. DESIGN: A retrospective chart review and clinical follow-up of patients who underwent optic nerve decompression. SETTING: Tertiary care referral center. PATIENTS: During an 8-year period, 95 extracranial optic nerve decompressions were performed by one of us (M.P.J.) for optic neuropathy resulting from traumatic, inflammatory, infectious, iatrogenic, neoplastic, and idiopathic processes. Thirty patients with compressive optic neuropathy secondary to histopathologically confirmed tumors were identified. INTERVENTION: Optic nerve decompression via external ethmoidectomy approach. RESULTS: Twenty (67%) of 30 patients showed improvement in vision. Improvement in 17 of the 20 patients has been stable. Seven patients (23%) showed no improvement but there was no further worsening of vision after surgery. In 3 patients (10%) vision deteriorated following surgery. CONCLUSION: Extracranial optic nerve decompression may be considered for the preservation or improvement of vision in selected patients with compressive optic neuropathy from neoplasms.

Normal anatomy and lesions of the lacrimal sac and duct: evaluated by dacryocystography, computed tomography, and MR imaging.

Diseases of the lacrimal system are optimally evaluated by dacryocystography, CT, MR imaging, and nuclear scintigraphy. Dacryocystography is selected to define the lumen of the lacrimal drainage system in inflammatory disease (dacryocystitis), dacryoceles, dacryoliths, and developmental abnormalities. Dacryocystography provides supplemental information in benign and malignant tumors, principally to differentiate an intrinsic lesion from an extrinsic lesion. CT is indicated for delineation of palpable mass lesions such as cysts, benign and malignant tumors, and diseases, especially in the paranasal sinuses, affecting the lacrimal apparatus secondarily. MR imaging has limited application but is useful to differentiate fluid from solid masses within the sac and to define tumor extension from the sac into the duct and anatomic regions outside the sac and duct. MR imaging with gadolinium enhancement should be used in suspected tumors and to separate a cyst from a solid mass. Radiologic methods fail to identify the histopathology of benign and malignant tumors. Nuclear scintigraphy is helpful in patients with epiphora with no demonstrable anatomic abnormality on dacryocystography.

Aneurysmal bone cyst of the orbit associated with fibrous dysplasia.

Aneurysmal bone cyst is a rare but recognized osseous lesion of the orbit. We report the presentation, treatment, and postoperative course of an orbital aneurysmal bone cyst in a patient with McCune-Albright syndrome. To our knowledge, this report represents the first description of aneurysmal bone cyst of the orbit in a patient with fibrous dysplasia. Although visual loss in patients with fibrous dysplasia is most commonly due to narrowing of the optic canal, aneurysmal bone cyst should be considered in a patient with fibrous dysplasia who presents with decreasing vision in the face of a rapidly expanding orbital mass. The treatment in this case was drainage and debridement with prompt resolution. Although not necessary in this case, an alternative treatment includes packing with bone chips. Radiation is usually avoided because of the risk of iatrogenically induced sarcoma.

Monopolar electrosurgical flash fire.

A 78-year-old man underwent ectropion repair with a monopolar electrosurgical unit. A flash fire occurred, resulting in a loss of eyelashes of the left upper and lower eyelids. A retrospective analysis of the case was conducted, including a review of the relevant literature. Although rare, the possibility of a flash fire should be considered when performing surgery with an electrosurgical unit. Minimizing supplemental oxygen and electrosurgical power settings may help to avoid such an incident.

Spontaneous superior ophthalmic vein thrombosis: a rare entity with potentially devastating consequences.

PURPOSE: Spontaneous superior ophthalmic vein thrombosis (SOVT) is a rare entity. We describe three patients with spontaneous ophthalmic vein thrombosis, each with various risk factors. PATIENTS AND METHODS: A retrospective review of three patients with a diagnosis of superior ophthalmic vein thrombosis. Clinical characteristics, radiographic features, management techniques and outcomes are described. RESULTS: All patients presented with unilateral painful proptosis. Two patients had intact light perception, whereas one patient presented with absent light perception. All patients had identifiable risk factors for thrombosis, which included sickle cell trait, hereditary hemorrhagic telangectasia and colon cancer with recurrent deep vein thrombosis. Anticoagulation was initiated in two patients. Resolution of proptosis was seen in all patients, with no recovery of vision in one patient. CONCLUSIONS: Risk factors for spontaneous superior ophthalmic vein thrombosis are multifactorial. MRI and MRV confirm the diagnosis of SOVT. Despite urgent intervention devastating visual loss may occur.

Traumatic hyphema: outcomes of outpatient management.

OBJECTIVE: This study aimed to compare the outcomes of outpatient and inpatient management of layered hyphema. DESIGN: The charts of all patients with traumatic layered hyphema treated in the Massachusetts Eye and Ear Infirmary Emergency Ward between January 1991 and November 1995 were analyzed retrospectively. Patients with a diagnosis of microscopic hyphema, ruptured globe, or posterior segment injury other than commotio retinae on their initial emergency department visit were excluded. The study patients were compared with an historic control group of patients with hyphema who had been treated at the same institution from July 1986 to February 1989. PARTICIPANTS: A total of 154 patients met the study criteria. These were compared with 119 patients in the historic control group. INTERVENTION: Of the study patients, 5% were admitted on the day of presentation, 95% were treated initially as outpatients, and 4% subsequently were admitted. All of the patients in the historic control group were treated with initial hospital admission. MAIN OUTCOME MEASURES: The rebleed rates of the study and control groups were compared. The final recorded visual acuity and causes of best-corrected visual acuity worse than 20/30 were analyzed for the study group. RESULTS: The rebleed rates of the study group and the historic control group were 4.5% and 5.0%, respectively (P > 0.05). The rebleed rates of the study patients initially treated as outpatients and the historic control group were 3.4% and 5%, respectively (P > 0.05). The rebleed rates of study patients who did not receive aminocaproic acid and the subset of historic control patients who received aminocaproic acid were 3.3% and 4.8%, respectively (P > 0.05). Ninety-six percent of study patients achieved a final best-corrected visual acuity of 20/30 or better. Causes of a final documented visual acuity worse than 20/30 included loss of patient follow-up before resolution of the hyphema, traumatic cataract, macular hole, and macular degeneration. CONCLUSIONS: In the authors' predominantly white patient population, close outpatient follow-up of traumatic hyphemas appears to be safe and effective. Hospitalization for hyphema does not appear to decrease the rate of rebleeding. Decreased vision in the setting of traumatic hyphema generally results from comorbidities not affected by inpatient management.