A 72-Year-Old Kidney Transplant Recipient With Visual Changes.

A 72-year-old woman with a history of kidney transplant was referred for an eye examination because of visual changes in the left eye. Both optic discs were anomalous in appearance, with central excavation in the left eye greater than the right eye. What would you do next?

A simple saccadic reading test to assess ocular motor function in cerebellar ataxia.

Cerebellar ataxia is a neurological disorder due to dysfunction of the cerebellum that affects coordination of fine movement, gait, and balance. Although ataxic patients commonly exhibit abnormal eye movement and have difficulties with saccadic reading, quantification of ocular motor abilities during reading in the clinical setting is rarely done. In this study, we assess visual performance with simple reading tests that can be used in the clinical setting and performed video infrared oculography in 11 patients with hereditary or acquired cerebellar ataxia and 11 age-matched controls. We found that compared with controls, ataxic patients read significantly slower on regularly and irregularly spaced 120 single-digit number reading tasks (read aloud) (p = 0.02 for both) but not on a word reading task (read silently), although there was large variability on the word reading task. Among the 3 reading tasks, the regularly spaced number reading task had the greatest difference (44%) between ataxic patients and controls. Analysis of oculography revealed that ataxic patients had slower reading speeds on the regularly spaced number reading task because of significantly higher saccade and fixation counts, impairment of small amplitude progressive saccades as well as large amplitude, line-changing saccades, greater fixation dispersion, and irregularity of scan paths and staircase gaze patterns. Our findings show that infrared oculography remains the gold standard in assessment of ocular motor difficulties during reading in ataxic patients. In the absence of this capability in the clinical setting, a simple 120 regularly spaced single-digit saccadic number reading test, which most patients can perform in less than 2 minutes, can be a possible biomarker for ocular motor abilities necessary for reading.

Long-term management of orbital and systemic reactive lymphoid hyperplasia with rituximab.

Rituximab, a monoclonal antibody to the B cell marker CD20, is becoming increasingly popular in the treatment of various orbital disorders. In this university-based interventional case series, we describe two patients with bilateral orbital and extra-orbital reactive lymphoid hyperplasia (RLH) treated with rituximab. Initially both had favorable responses; but roughly a year later recurrent disease necessitated maintenance therapy in both cases. Both again responded to additional courses of rituximab. Although recalcitrant disease may persist after treatment, rituxmab may play a role in the management of RLH with widespread involvement.

Comparison of Humphrey Matrix frequency doubling technology to standard automated perimetry in neuro-ophthalmic disease.

PURPOSE: We compared Humphrey Matrix FDT 30-2 (FDT) and Humphrey Visual Field Analyzer 30-2 SITA standard (SAP) in the assessment of anterior (optic nerve or chiasm) and posterior (retro-chiasmal) afferent visual pathway defects. MATERIALS AND METHODS: In this retrospective comparative study, the charts of 37 patients (16 males, range 13-84 years, mean 72.1), with neuro-ophthalmic visual field defects who were tested with both FDT and SAP, were reviewed. Two masked graders assessed the concordance and extent of field defects between the perimeters. The mean concordance between anterior and posterior disease was compared using the Wilcoxon rank sum test. The mean deviation (MD) and pattern standard deviation (PSD) of each perimeter were correlated with the Spearman coefficient. RESULTS: Twenty-eight patients had anterior and nine had posterior disease. Most had a fair or good concordance (89.3% anterior, 88.9% posterior). When comparing anterior to posterior disease, the mean concordance of the defects of the two parameters was not statistically different (P = 0.94 and P = 0.61 for total deviation and pattern deviation, respectively). The MD and PSD between perimeters had a significant correlation. CONCLUSIONS: Our series, using 30-2 field analysis, demonstrates fair to good correlation between FDT and SAP in the majority of patients. In roughly 10% findings between FDT and SAP were discordant. This difference was similar for anterior and posterior disease.

Evisceration in the modern age.

Evisceration is an ophthalmic surgery that removes the internal contents of the eye followed usually by placement of an orbital implant to replace the lost ocular volume. Unlike enucleation, which involves removal of the entire eye, evisceration potentially causes exposure of uveal antigens; therefore, historically there has been a concern about sympathetic ophthalmic (SO) associated with evisceration. However, critical review of the literature shows that SO occurs very rarely, if ever, as a consequence of evisceration. Its clinical applications overlap with those of enucleation in cases of penetrating ocular trauma and blind painful eyes, but it is absolutely contraindicated in the setting of suspected intraocular malignancy and may be preferred for treatment of end-stage endophthalmitis. From a technical standpoint, traditional evisceration has a limitation in the orbital implant size. Innovations with scleral modification have overcome this limitation, and accordingly, due to its simplicity, efficiency, and good cosmetic results, evisceration has once again been gaining popularity.

Sunken eyes, sagging brain syndrome: bilateral enophthalmos from chronic intracranial hypotension.

PURPOSE: To explain the mechanism for acquired enophthalmos after ventriculoperitoneal shunting (VPS). DESIGN: Case series and a case-control study. PARTICIPANTS AND CONTROLS: Four study patients with bilateral enophthalmos after VPS and 10 control subjects. METHODS: Case description of 4 study patients. Calculated orbital volumes for 2 study patients were compared with controls using the Wilcoxon rank-sum test. MAIN OUTCOME MEASURES: Exophthalmometry measurements and total orbital and fat volumes. RESULTS: Patient 1 is a 25-year-old man who presented with progressive enophthalmos 3 years after VPS for traumatic intracranial bleeding. Imaging demonstrated upward expansion of the orbital roof and evidence of intracranial hypotension. The intracranial pressure (ICP) was 20 mm H2O. The enophthalmos improved after shunt revision. Patient 2 is a 19-year-old man who presented with progressive enophthalmos 18 months after VPS for traumatic intracranial hemorrhage. Patient 3 is a 38-year-old woman who presented with bilateral enophthalmos 15 years after VPS after a ruptured aneurysm. Imaging showed orbital expansion. Patient 4 is a 16-year-old man who presented with severe enophthalmos 5 years after a VPS for aneurysm-related hemorrhage. Imaging demonstrated orbital enlargement and findings of intracranial hypotension. Intracranial pressure ranged between -200 and 0 mm H2O. Shunt revision improved the enophthalmos. Total orbital volumes were significantly greater in the study patients than in the controls. Control subjects (5 male, 5 female, ages 23-45 years) had an average right orbital volume of 24.6 ± 3.3 cm³ (n = 10). In comparison, the right orbital volumes of patients 1 and 3 were 32.6 and 32.1 cm³. Similar results were found for the left orbits (23.9 ± 2.7 cm³ [control average] vs. 35.9 and 32.6 cm³). In patient 1, the post-shunt volumes increased 14% (right) and 23% (left) from pre-shunt volumes. In contrast, orbital fat volume was not statistically significantly different between the control group and enophthalmic patients (right orbit control mean 7.94 ± 3.1 cm³ [n = 10] vs. 7.9 and 9.8 cm³; left orbit control mean 7.88+3.1 cm³ vs. 9.2 and 10.0 cm³). CONCLUSIONS: Enophthalmos after VPS results primarily from chronic intracranial hypotension. Low ICP causes expansion of orbital volume with no fat atrophy. In such patients, shunt revision with a pressure-regulating valve to correct intracranial hypotension should be considered. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Acquired pediatric blepharoptosis related to linear scleroderma.

Periocular linear scleroderma coup de sabre has been seen in association with blepharoptosis. The authors describe a 4-year-old girl who developed left blepharoptosis simultaneously with a remote scalp lesion consistent with linear scleroderma coup de sabre. This case is unique in that the scalp lesion was distant from the affected eye.

Giant cell arteritis in Asians: a comparative study.

BACKGROUND: Giant cell arteritis (GCA) is a common systemic vasculitis, with a presumed Caucasian predominance. The occurrence of GCA in Asians has rarely been addressed. This study aims to assess the incidence of giant cell arteritis in Asians. METHODS: In this retrospective review, the self-reported ethnicities of patients with biopsy-proven GCA at the University of California-San Francisco (UCSF) were recorded. Ethnic distribution of the patient population served by UCSF was estimated from an age- and sex-matched control group. The odds ratio for each ethnicity (Asian and Caucasian) was determined and compared using Fisher's exact test and logistic regression analysis. RESULTS: The ethnic distribution of the 38 patients with positive temporal artery biopsies were as follows: Caucasian n=31 (81.6%), Asian n=1 (2.6%) and other n=6 (15.8%). The ethnic distribution of the patient population served by UCSF was as follows: Caucasian 42%, Asian 28% and other 30%. The difference in the proportion of GCA in Asians and Caucasians was statistically significant (OR 0.049 (95% CI 0.0065 to 0.374), p=0.0036). CONCLUSIONS: In our patient population, GCA was seen 20 times less frequently in Asian than Caucasian patients. Although this difference is significantly different (p=0.036), given the small sample size and wide CI this should be viewed as a rough estimate.

Acquired enophthalmos in lupus erythematosus profundus.

Lupus erythematosus profundus (LEP) is an uncommon subtype of lupus erythematosus. A 76-year old man presented with inflammation of the eyelids and underlying orbital soft tissue. A biopsy disclosed inflammation and atrophy of the orbital fat consistent with LEP. Systemic corticosteroid treatment produced resolution of the inflammation. but as the edema subsided, enophthalmos became apparent. LEP should be considered in patients with a characteristic rash and orbital inflammation and may cause acquired enophthalmos.

Adult orbital trapdoor fracture.

Trapdoor fractures occur almost exclusively in the pediatric population. The authors describe an adult with an entrapped inferior rectus muscle sheath in a trapdoor fracture. A 37-year-old man presented with persistent diplopia 3 weeks after blunt right orbital trauma. The only abnormal findings on clinical examination were limited vertical ductions. No bony defect or displacement was evident on CT. However, several small pockets of air were visible adjacent to the inferior rectus muscle. On surgical exploration, a linear nondisplaced orbital floor fracture was confirmed, and the entrapped inferior rectus muscle was released. One month postoperatively, extraocular motility had improved with no diplopia in primary or reading positions. This case demonstrates that trapdoor fractures can occur in adults and should be considered when suggestive findings are encountered. Clinicians should be aware of this because timely diagnosis and treatment might achieve more favorable outcomes.

Blindness from orbital varices: case report.

A 41-year-old woman presented with a 21-year history of a left orbital mass. She reported 3 distinct episodes of sudden proptosis, periorbital bruising, pain, nausea and vomiting with resulting stepwise deterioration in her vision. Her symptoms resolved spontaneously over several days, with the exception of loss in vision, which persisted. Examination was notable for ipsilateral enophthalmos in primary gaze. With Valsalva she developed proptosis. Magnetic resonance imaging (MRI) demonstrated a left orbital apex malformation consistent with a varix. She had no light perception on the left with end-stage optic atrophy. This case illustrates the severity of visual loss that can occur with orbital varices.

Marsupialization of orbital conjunctival inclusion cysts related to strabismus surgery.

A 52-year-old woman presented with a giant conjunctival cyst related to strabismus surgery that was adjacent to the medial rectus muscle. Management consisted of excision of the anterior cyst wall with suturing of the remaining cyst lining to overlying conjunctiva. Following marsupialization, the cyst did not recur with persistence of the conjunctival drainage site.

Amyloidosis-associated neurotrophic keratopathy precipitated by overcorrected blepharoptosis.

PURPOSE: To describe amyloidosis-associated neurotrophic keratopathy. METHODS: In this interventional case report, we describe the clinical findings of a patient with amyloidosis-associated neurotrophic keratopathy, precipitated by exposure from overcorrected blepharoptosis. RESULTS: A 70-year-old woman with a history of amyloidosis was referred with bilateral eyelid retraction 1 month after levator aponeurosis advancement surgery. Examination demonstrated visual acuities of count-fingers OD and 20/400 OS and severe corneal epithelial irregularity with diffuse fluorescein staining. She had marked eyelid retraction with 5-mm right and 3-mm left superior scleral show and incomplete closure. Corneas were anesthetic, and neurologic examination was notable for peripheral sensory loss. Despite eyelid position normalization, the corneal abnormalities progressed to large epithelial defects refractory to aggressive lubrication, punctal occlusion, bandage contact lenses, extended patching, and ultimately tarsorrhaphy consistent with neurotrophic keratopathy. One year postoperatively, the epithelial disease persists. CONCLUSION: Neurotrophic keratopathy can occur in association with amyloidosis and may be precipitated by exposure related to blepharoptosis repair.

Hypotony maculopathy after eyelid massage for overcorrected blepharoptosis.

A 57-year-old woman with a history of left eye trabeculectomy was evaluated for gradual ipsilateral visual loss. Several months prior, she had undergone levator advancement of the left upper eyelid. For management of resulting retraction, she was instructed to "massage" her eyelid. Examination was notable for left eye visual acuity of 20/200 and an intraocular pressure of 5 mm Hg. On fundoscopic examination, the macula was edematous with multiple folds, consistent with hypotony maculopathy. Two years later, after obliteration of the bleb and placement of a Seton valve, the intraocular pressure has increased to 8 mm Hg with an acuity correctable to 20/50. Ocular hypotony may result from digital eyelid massage in patients with filtering blebs. Clinicians should bear this in mind when managing patients with eyelid retraction after blepharoptosis repair.

Delusions of parasitosis of the eyelids.

The authors describe preseptal cellulitis and corneal ulceration due to secondary infection of self- inflicted wounds in a patient with delusions of parasitosis. A 42-year-old man presented with a 3-day history of progressive painful, purulent, periocular erythema. He reported that "little black bugs and whitish eggs" had infiltrated his body including the left eye and eyelids. On examination, he was agitated with superficial wounds covering the majority of his body. Ophthalmic evaluation was notable for markedly erythematous and mildly edematous eyelids resulting in complete blepharoptosis. Excoriations with yellow-brown serous crusting carpeted the periocular region. A 1-mm pericentral corneal ulcer was also noted. No objective evidence of parasites was found and his infection resolved with antibacterial therapy (intravenous ceftriaxone and vancomycin, and topical vancomycin and ceftazidime). Delusions of parasitosis may result in self-mutilation with secondary infection. Appropriate psychiatric care is needed to prevent ongoing destructive behavior.

Interactive image-guided endoscopic dacryocystorhinostomy.

The authors describe dacryocystorhinostomy performed with CT guidance. A 53-year-old man with a history of cocaine abuse was referred for bilateral nasolacrimal duct obstruction. Nasal speculum examination revealed an oronasal fistula and obliteration of the inferior nasal septum. Bilaterally, the inferior and middle turbinates were contracted, forming thick scar tissue conglomerates. The loss of normal anatomic landmarks and extensive contracted scar tissue precluded standard dacryocystorhinostomy approaches. Endoscopic dacryocystorhinostomy was therefore undertaken using CT-guided navigation. The location of the superior nasolacrimal duct was identified and subsequently exposed. Dissection was continued superiorly, marsupializing the lacrimal sac. Silicon stents were placed and tied intranasally. Right and left sides were managed similarly. Several months later, following stent removal, the patient was without epiphora. This case demonstrates that CT guidance can be a useful adjunct to standard dacryocystorhinostomy techniques, allowing otherwise challenging surgical cases to be managed effectively and safely.

Levator superioris muscle function in involutional blepharoptosis.

PURPOSE: To assess the role of muscular degeneration, we evaluated the correlation between ptosis severity and levator muscle function. DESIGN: Retrospective cohort study. METHODS: The medical records of 136 patients (53 men and 83 women; mean age, 67 years) with acquired blepharoptosis were reviewed for levator function (LF), margin reflex distance (MRD), age, and gender. Multivariate linear regression was performed for statistical analysis. RESULTS: A significant correlation (P < .001) was seen between MRD (mean, 1.0 + 1.0 mm; range, -3.0 to 3.0 mm) and LF (mean, 15.0 + 1.0 mm; range, 11.0 to 20.0 mm). On average, a 0.5-mm reduction in LF was observed for each 1.0-mm decrease in MRD. This was independent to other variables assessed. CONCLUSIONS: In patients with involutional blepharoptosis, a directly proportional decrease in levator function and eyelid height was observed. This may implicate an abnormality of the levator muscle itself as a contributing factor in the development of involutional blepharoptosis.

Posterior retinal breaks as a cause of vitreous hemorrhage in diabetes.

PURPOSE: To describe posterior retinal breaks as a cause of vitreous hemorrhage in diabetic patients. METHODS: In two institutional practices, six posterior retinal breaks were identified in five eyes of five diabetic patients with vitreous hemorrhage. All eyes underwent fundus photography and fluorescein angiography. Four eyes received barrier photocoagulation. The outcome measures included retinal nonperfusion, proximity to retinal vessels, and progression to retinal detachment. RESULTS: All six posterior breaks were in areas of retinal ischemia. No eyes had neovascularization. Three breaks had a bridging vessel, and three were in a paravascular location. One untreated eye had progression to a retinal detachment. CONCLUSIONS: The differential diagnosis for vitreous hemorrhage in diabetic patients should include posterior retinal breaks, particularly in the absence of proliferative disease. These breaks are paravascular, are located in areas of retinal ischemia, and may involve avulsed bridging vessels. We suggest treatment with barrier rather than panretinal photocoagulation.