Contrasting presentations of the same disease: A comparison of two cases of amyloidosis presenting with eyelid involvement.

Purpose: Localized amyloidosis can affect numerous tissues throughout the body and can also affect a variety of peri-ocular tissues including the conjunctiva, extra-ocular muscles, peri-orbital soft tissue, and lacrimal gland. We report two cases of amyloidosis presenting with eyelid involvement. Observations: The first case represented a more subtle presentation of skin thickening with a pre-septal cellulitis, while the second case had a dramatic presentation of edema evolving into tissue dehiscence and spontaneous hemorrhage with ongoing angioedema and systemic coagulopathy. Conclusions and importance: The two cases of biopsy-proven orbital/peri-ocular amyloidosis demonstrate the different clinical presentations that may go from the subtle to dramatic, depending on which peri-ocular tissues are affected and to what degree. Standards for treatment of amyloidosis remain conservative initially with surgery or radiation recommended only for refractory cases, but additional therapies are under investigation. Clinicians should have high clinical suspicion for amyloidosis with findings such as skin thickening or significant periorbital edema and should always consider tissue biopsy and further workup for amyloidosis if the findings worsen or do not resolve with treatment of more common conditions such as cellulitis.

A novel telemedicine technique for evaluation of ocular exam findings via smartphone images.

In this study, we compared the assessment of remote smartphone photographs to in-office exams in the diagnosis of two groups of external eye diseases, red-eye pathology and post-operative eyelid surgery complications. Participants were examined and received an in-office diagnosis by either a corneal or oculoplastic specialist. After viewing an educational video on smartphone photography, the patient's companion then took a series of standardized photographs. Two additional corresponding specialists then made a separate diagnosis via the interpretation of only smartphone images and the patient's history. 'Remote' and in-office diagnoses were compared using a kappa test for agreement. The remote and in-office diagnoses were in agreement for 27 of 28 eyes, representing a chance-corrected Kappa agreement rate of 93% (95% confidence interval: 79-99%). Among the 16 red eyes, the diagnoses were in agreement for 15 of 16 red eyes, representing a chance-corrected Kappa agreement rate of 92% (95% confidence interval: 77-99%). Among the 12 eyes with post-operative eyelid surgery complications, the diagnoses were in perfect agreement. Our results suggest that the diagnosis of 1) red-eye pathology and 2) post-operative eyelid surgery complications based on smartphone images may be comparable to in-office exams.

Punctal Congestion Syndrome: A Reversible, Functional Punctal Stenosis Causing Epiphora in the Setting of Chronic Pretarsal Conjunctivitis.

PURPOSE: To describe a reversible syndrome of epiphora, functional punctal stenosis, and chronic pretarsal conjunctivitis associated with corticosteroid or corticosteroid-antibiotic eyedrop use. METHODS: This is an Institutional Review Board-approved retrospective review of patients diagnosed with epiphora, punctal stenosis, and chronic conjunctivitis by a single surgeon (B.J.W.). These patients were subsequently invited to participate in a prospective study involving allergy skin patch testing for ophthalmic drops, common excipients, and active ingredients. RESULTS: Thirteen patients received a diagnosis of punctal congestion syndrome. The average age was 63 years (range, 41-93) and 69.2% were female. Findings were bilateral in 61.5%. All had used preserved drops in the affected eye(s). Various antecedent diagnoses resulted in treatment with preserved drops. Patients experienced epiphora for an average of 3.8 months (median, 3 months; mode, 3 months; range, 1-8 months) prior to presentation. Two patients had undergone punctoplasty which failed to resolve symptoms. 92.3% of patients had been taking tobramycin-dexamethasone drops, loteprednol drops, or a combination of both prior to presentation. All were taken off preserved drops. 69.2% were also treated with a preservative-free loteprednol etabonate 0.5% ophthalmic ointment taper. All improved. Partial relief of symptoms was achieved by an average of 1.6 months (median, 2 months; mode, 2 months; standard deviation, ±0.7 months) and resolution of symptoms by 2.5 months (median, 2 months; mode, 2 months; standard deviation, ±1.7 months). One patient underwent patch testing with strong positive reactions to formaldehyde and neomycin and a weak positive reaction to gentamicin. CONCLUSIONS: Functional punctal stenosis is associated with topical ophthalmic preparations, especially preserved corticosteroids and antibiotic-corticosteroid combinations. Treatment consists of removal of all preserved eyedrops. Symptoms often improve over several months.

BARTONELLA HENSELAE NEURORETINITIS ASSOCIATED WITH CENTRAL RETINAL VEIN OCCLUSION, CHOROIDAL ISCHEMIA, AND ISCHEMIC OPTIC NEUROPATHY.

PURPOSE: We describe a healthy 37-year-old man with Bartonella henselae (B. henselae) neuroretinitis with concurrent central retinal vein occlusion and ischemic optic neuropathy resulting in optic atrophy and choroidal ischemia. METHODS: Case report. RESULTS: A 37-year-old man presented with unilateral decreased vision and a fundus examination consistent with neuroretinitis. Further imaging review supported a concurrent diagnosis of central retinal vein occlusion. Although initially negative, repeat serological testing for B. henselae infection was positive. Multimodal imaging displayed severe outer retinal disruption, ischemic optic neuropathy, and choroidal ischemia. The patient demonstrated near complete resolution of fundus findings and restoration of outer retinal architecture. Residual findings included optic disk pallor and ischemic choroidopathy. DISCUSSION: B. henselae neuroretinitis may be associated with concurrent retinal vascular occlusive disease and ischemic optic neuropathy. Central retinal vein occlusion and choroidal ischemia leading to optic nerve atrophy are additional sequelae further expanding the clinical spectrum of this entity.

Horner's Syndrome Following Varicella Vaccination.

A 52-year-old female with no significant past medical history presented with disseminated herpes zoster as well as a Horner's syndrome and retinal haemorrhages 3 weeks after receiving the varicella vaccine. The patient began treatment with valacyclovir, 1g three times per day, and gabapentin, 300mg three times a day, for pain. Over the following month, the rash and the retinal haemorrhages resolved. The ptosis and anisocoria also resolved over the following two months.

CONTRALATERAL ANESTHESIA IN TWO PATIENTS AFTER RETROBULBAR BLOCK.

PURPOSE: To report a case series of two patients with contralateral anesthesia after retrobulbar block. METHODS: Retrospective review of two cases and review of the literature. RESULTS: Two patients of one practitioner received contralateral anesthesia after retrobulbar block for posterior segment surgery. Patient 1 suffered from transient contralateral akinesia, whereas Patient 2 experienced transient contralateral amaurosis. CONCLUSION: Posterior spread of anesthetics is a rare but potentially serious complication of retrobulbar anesthesia caused by spread of anesthetics along the optic nerve sheath. Modification of injection technique can decrease the risk of this complication.

Sagging Eye Syndrome or Nemaline Rod Myopathy? Divergence Insufficiency with Levator Dehiscence as an Overlapping Symptom between Two Diagnoses.

A 78-year-old woman complained of gradual, painless onset of horizontal binocular diplopia associated with progressive axial weakness. Physical examination revealed esotropia that was greater at distance than at near vision, bilateral levator dehiscence, and normal abducting saccadic speeds. Given the age of the patient and compatible clinical findings, the diagnosis of Sagging Eye Syndrome (SES) was made. However, further work-up with a muscle biopsy suggested Sporadic Late-Onset Nemaline Myopathy (SLONM) as the cause of her progressive muscle weakness. Although rare, external ophthalmoplegia has been described in the literature as a presenting symptom in SLONM. To elucidate the pathological mechanism for the patient's diplopia, an MRI of the orbits was performed, which revealed findings consistent with SES. This case aims to highlight the importance of integrating clinical findings during the diagnostic process and serves as a reminder that diplopia can be a common symptom for an uncommon diagnosis.

Microvascular Free Flap for Total Eyelid Reconstruction With a Visually Useful Eye.

Total eyelid loss, full thickness loss of the upper and lower eyelids, is uncommon and surgically challenging. Eyelid reconstruction after such injuries can be further complicated by loss of adjacent tissue. When tissue for local flaps is unavailable, free flaps must be considered. Few cases of total eyelid reconstruction via microvascular free flap have been reported, especially with an intact globe and good visual acuity. In this report, we describe the use of a microvascular free flap based on the radial artery for total eyelid reconstruction in a patient with an intact globe and useful visual acuity.