Progressive myelinated retinal nerve fibers in a 10-year-old boy with Crouzon syndrome after craniofacial surgery.

Purpose: We present a case of a 10-year-old boy with Crouzon syndrome that demonstrates progressive myelinated retinal nerve fibers (MRNF). Observations: A 10-year-old boy was referred for ophthalmic examination due to clusters of opaque white fibers around his optic nerve. Past surgical history includes craniofacial surgery at 3 years of age secondary to the deteriorating vision from increased intracranial pressure and papilledema. Upon examination (now 6.5 years post-craniofacial surgery), the patient denied any ocular complaints. The fundus examination showed progressively enlarged myelination of the retinal nerve fiber layer (right eye > left eye). Although previous cases of MRNF with Crouzon syndrome have been reported, our case is unique given its post-operative status with early onset of MRNF. Conclusion and importance: This case report documents the photographic progression of bilateral myelinated retinal nerve fibers (MRNF) in a pediatric case of Crouzon syndrome post-craniofacial surgery secondary to increased intracranial pressure and papilledema. Based on our patient, craniofacial decompression surgery may not prevent the development of MRNF. The exact mechanisms of MRNF are still being studied. Further investigations correlating craniofacial surgeries, increased intracranial pressure, and progression of myelinated retinal nerve fibers are needed to understand this process.

Cost-effective treatment of ocular surface squamous neoplasia for an undocumented and uninsured New York City patient: a case report.

BACKGROUND: New York City has a heterogeneous population with many undocumented and uninsured immigrants from equatorial areas who have a higher incidence of ocular surface squamous neoplasia. To the best of our knowledge, this is the first documented selection of this cost-effective treatment of ocular surface squamous neoplasia (the use of absolute ethanol along the corneal margin, primary excision, double freeze-thaw cryopexy, and primary conjunctival closure) for an undocumented and uninsured New York City patient. CASE PRESENTATION: A 35-year-old man from Ecuador presented to a New York City emergency department due to worsening discomfort of a long-standing left eye pterygium. A slit-lamp examination of the left eye demonstrated a nasally located conjunctival mass measuring 6 × 8 mm extending onto the cornea (3 mm superiorly and 6 mm inferiorly on the cornea). Histological diagnosis confirmed squamous cell carcinoma in situ arising from the pterygium. Surgical excision with adjunctive absolute alcohol with additive double freeze-thaw cryopexy was performed. Our patient has remained free of tumor recurrence at year 2 postoperative visit. CONCLUSIONS: Our case highlights the need to choose a cost-effective treatment for ocular surface squamous neoplasia in an at-risk population among undocumented and uninsured patients. Areas in the world with similar types of populations or treatment challenges may need to consider this approach as a primary treatment option.

PSEUDO UVEAL MELANOMA CAUSED BY OPTIC DISK DRUSEN WITH JUXTAPAPILLARY CHOROIDAL NEOVASCULAR MEMBRANE.

PURPOSE: To describe two cases of choroidal hemorrhage caused by optic disk drusen-induced choroidal neovascularization simulating uveal melanoma. METHODS: Observational case reports of two patients and brief review of the literature. RESULTS: Two patients were referred with pigmented juxtapapillary lesions concerning for choroidal melanoma. Multimodal imaging revealed the presence of optic disk drusen with overlying choroidal neovascular membranes and peripapillary choroidal hemorrhage. Both patients were treated with antivascular endothelial growth factor and the lesions resolved. CONCLUSION: In the setting of diagnostic uncertainty, careful multimodal imaging can assist in distinguishing between malignant choroidal melanoma and a benign simulating lesion. Optic disk drusen with associated neovascularization and hemorrhage should be included in the list of pseudomelanomas.

Peripapillary Choroidal Neovascularization Associated with Optic Nerve Head Drusen Treated with Anti-VEGF Agents.

Optic nerve head drusen can be associated with peripapillary choroidal neovascularization, in both the pediatric and adult population. These membranes can involve the macula, causing significant visual loss. Herein, we present a case that required treatment with an anti-VEGF agent. The patient failed to respond to the initial agent, but subsequently responded to a change of agent. Adult patients with macular degeneration involving peripapillary choroidal neovascularization associated with optic nerve head drusen may require individualized treatment plans.

Ocular Toxoplasmosis: Controversies in Primary and Secondary Prevention.

Ocular toxoplasmosis is the most common cause of posterior uveitis in the United States and worldwide. It commonly follows a relapsing course with the potential for multiple adverse visual sequelae and, rarely, blindness. Both immune-competent and immune-suppressed populations can develop severe relapsing disease. There is considerable debate regarding the initial treatment of ocular toxoplasmosis in both groups, with multiple antiparasitic agents commonly used with little evidence to support any particular regimen. The precise role of corticosteroids in treatment also has yet to be rigorously defined. Secondary prevention is commonly implemented in the immune suppressed population, but there is a lack of level one evidence to support its role in immune-competent patients, though it warrants future investigation.

Medulloblastoma presenting as consecutive esotropia after successful lateral rectus muscle recession for infantile exotropia.

Comitant infantile exotropia is a rare entity and is far less common than infantile esotropia. It has been shown to be highly correlated with intracranial pathology. We report a case of a medulloblastoma in a child in whom it presented as an incomitant esotropia after surgical correction for comitant infantile exotropia.

Coats' disease, Turner syndrome, and von Willebrand disease in a patient with Wildtype Norrie disease pseudoglioma.

The authors describe a girl diagnosed as having Coats' disease, Turner syndrome (45X karyotype), and type 1 von Willebrand disease. She tested negative for the Norrie disease pseudoglioma (NDP) gene located on the X-chromosome, which has been suspected of contributing to Coats' disease.

Endogenous fungal endophthalmitis after cardiac surgery.

The authors present a single-patient interventional case report of endogenous fungal endophthalmitis (EFE) in a patient with an implanted prosthetic device. A 74-year-old man underwent a double coronary artery bypass graft with a bioprosthetic pericardial valve and transvenous dual chamber pacemaker. Four months later, he presented with EFE. Despite aggressive local and systemic antifungal therapy, the patient died of septic shock due to a fungally infected pacemaker. Successful management of post-cardiac surgery EFE requires aggressive local and systemic antifungal therapy, but without surgical removal of implanted thoracic hardware these modalities alone may be insufficient for a cure.

Argon Laser Photocystotomy of a Vitreous Cyst.

Free-floating idiopathic vitreous cysts are rare entities. For symptomatic patients, treatment options include laser photocystotomy or pars plana vitrectomy (PPV) with cyst excision. Historically, size of the cyst impacted treatment approaches. Laser treatments have been used for smaller cysts ranging 3 to 5 mm in diameter. PPV has been utilized for cysts larger than 4 mm. Successful laser photocystotomy has been reported, utilizing both argon and Nd: YAG lasers. Herein, we present a case of successful argon laser photocystotomy of a 7.1 mm diameter vitreous cyst, the largest documented cyst ever treated. Treatment should be individualized based on patient age, ability to cooperate in an office setting, phakic status, status of the posterior hyaloid, cyst morphology, and location. In our case, because of pigment on the anterior cyst surface, and the presence of an attached posterior hyaloid, the argon green laser was used.

Baylisascaris Procyonis Neural Larva Migrans in an Infant in New York City.

Neural larva migrans (NLM) with eosinophilic meningoencephalitis secondary to raccoon roundworm (Baylisascaris procyonis) infection has been reported in rural and suburban areas of North America and Europe with extant raccoon populations. Most cases have occurred in infants less than two years of age exposed to areas of raccoon fecal contamination. Here, we present a case of Baylisascaris-induced NLM from the densely populated borough of Brooklyn in New York City and alert urban pediatricians to consider this cause of clinical neurologic disease even in areas not typically thought to be associated with endemic risk factors. Infected raccoons also occur in urban settings, and urban children may be exposed to environmental areas or materials contaminated with their feces and the parasite's eggs.

Baylisascaris Procyonis Induced Diffuse Unilateral Subacute Neuroretinitis in New York City.

Diffuse unilateral subacute neuroretinitis (DUSN) secondary to raccoon roundworm (Baylisascaris procyonis) infection has been reported in rural and suburban areas of North America and Europe with extant raccoon populations. Here, we present a case of Baylisascaris-induced DUSN from the densely populated borough of Brooklyn in New York City and alert urban ophthalmologists to consider this etiology even in areas not typically thought to be associated with endemic risk factors. Infected raccoons also occur in urban settings, and urban patients may be exposed in surrounding areas. Most patients with Baylisascaris ocular larva migrans-DUSN will not have concomitant neurologic disease; this fact and larval neurotropism are both misconceptions regarding this infection.

Endocapsular cellulomonas as a cause of persistent postoperative endophthalmitis.

Sequestration of bacteria within the capsular fornices after cataract extraction with intraocular lens implantation can cause both acute and chronic inflammation. A case of persistent postoperative endophthalmitis caused by capsular sequestration of Cellulomonas is described. The patient underwent uncomplicated cataract extraction with intraocular lens implantation and subsequently developed acute postoperative endophthalmitis. Inflammation persisted despite several vitreous taps and the injection of intravitreal antibiotics. Definitive treatment required pars plana vitrectomy, intraocular lens explantation, capsular bag removal, and intravitreal and parenteral antibiotics. In patients with postoperative endophthalmitis, one must consider atypical organisms as the source and should consider explantation of the intraocular lens with capsular bag removal.