James G. Chelnis

Immunoglobulin G4–related sclerosing disease with orbital inflammation in a 12-year-old girl

Immunoglobulin G4-related sclerosing disease is a rare but potentially debilitating cause of orbital inflammation, with a predilection for older males. We report the case of a 12-year-old African girl with immunoglobulin G4-related sclerosing disease, including possible extraorbital involvement. Because of an escalating severity of illness leading to oculomotor nerve palsy and cavernous sinus thrombosis that was resistant to steroids, systemic immunosuppressive therapy with rituximab was used to achieve disease remission. The diagnosis was histologically confirmed with a tissue biopsy.

Urrets-Zavalia syndrome following goniotomy in a child

Urrets-Zavalia syndrome is a surgical complication in which the pupil remains fixed and dilated after a procedure. It is believed to be caused by brief periods of high intraocular pressure. Although the syndrome was originally associated with penetrating keratoplasty, it has subsequently been associated with other procedures. We report the case of a 13-year-old boy with congenital glaucoma who developed Urrets-Zavalia syndrome after goniotomy. After 2 years of follow-up, the pupil remained fixed and dilated in that eye. To our knowledge this is the first case of Urrets-Zavalia syndrome to be reported following goniotomy and the first to occur in a child.

Enhanced Depth Imaging and Fundus Autofluorescence of Toxocara Optic Nerve Granuloma

To the Editor, Ocular toxocariasis often presents with posterior pole granulomas. Peripheral retina and the macula are frequently involved, but optic nerve granulomas are rare (6–19%).1 A comprehensive literature search found no imaging reports of this presentation in the existing literature. To our knowledge, our study is the first to investigate the morphologic features of the optic nerve lesion in a patient with toxocariasis, with images obtained by enhanced-depth imaging optical coherence tomography (EDI-OCT) and fundus autofluorescence (FAF) using a Heidelberg Spectralis SD-OCT (Heidelberg Engineering, Heidelberg, Germany). A 6-year-old boy living with pet dogs presented with reduced vision of the right eye for 3 months. Examination revealed visual acuity of 20/200, right exotropia, normal pupillary response, 2+vitreous cell and haze, a yellowish lesion overlying the optic nerve, and a raised fibrotic lesion in the temporal periphery. The left eye was normal. The patient’s serum was positive for Toxocara Ab ELISA but negative for toxoplasma IgG and IgM, ANA, herpes simplex virus I & II IgG, CMV IgG and IgM, Lyme IgG and IgM, HLA-B27, and rheumatoid factor, along with an unremarkable CBC. The patient was treated with oral and topical corticosteroids and mebendazole. Two weeks later the patient’s vitritis had improved, vision improved modestly to 20/100, and he was referred to a pediatric ophthalmologist for amblyopia treatment. The patient’s vitritis completely resolved and visual acuity improved to 20/70 by 2 months. Over an 8-month interval, the patient reached a visual acuity of 20/30 without recurrence of vitritis and with continued amblyopia treatment. Eight months after the resolution of vitritis, clinical exam and color fundus photography demonstrates a fibrotic lesion at the optic disc. On FAF the lesion has a heterogeneous punctate hyperfluorescent pattern. This modality served as a supplement to fundus photography in defining the boundaries of the lesion over the optic nerve. Previous macular OCT studies of Toxocara granulomas have reported these lesions as round, subretinal masses associated with surrounding fluid.2,3 Histological examinations of Toxocara granulomas in monkeys were also found to be nodular with fibrous capsules.4 The mass in this study has a similarly nodular appearance and is associated with a hyperreflective fibrotic cap on OCT, though it was not associated with fluid. The mass fills the optic cup and remains superficial over the optic disc, while involving all the layers of the adjacent retina and sparing vasculature. EDI-OCT allows imaging of the deeper levels of the optic nerve than standard techniques and, despite shadowing in some views that preclude definitive judgment, certain views support sparing of deeper nerve layers in our case. The optic nerve has been suggested as a route of entry for Toxocara larvae into the eye. The presence of an optic nerve granuloma with adjacent vascular sparing supports perineural invasion of the globe. Good visual recovery and lack of afferent pupillary defect in our patient suggests that a significant portion of optic nerve was spared. In conclusion, EDI-OCT of optic nerve Toxocara granulomas identify possible sparing of the deeper nerve layers despite deep involvement of adjacent retina. These lesions can allow good visual recovery. Ocular Immunology & Inflammation, 21(1), 82–83, 2013

Periorbital Necrotizing Fasciitis Secondary to Candida parapsilosis and Streptococcus pyogenes.

Necrotizing fasciitis is most often caused by either polymicrobial bacterial infections or by Gram-positive organisms, such as Streptococcus or Staphylococcus; however, rare cases of fungal necrotizing fasciitis have been reported. Candida parapsilosis is an emerging fungal pathogen. This fungus grows in either a yeast or pseudohyphal form. C. parapsilosis has been reported to cause keratitis, intraocular infection, and seeding of frontalis slings. C. parapsilosis is a commensal of human skin and can be acquired by nosocomial spread. Necrotizing fasciitis due to Candida has rarely been reported, but to date C. parapsilosis has not been identified as the causative organism in necrotizing fasciitis. This is the first documented case of human periocular soft tissue infection by C. parapsilosis, and also the first report providing evidence of mycotic infection in a necrotizing fasciitis concurrently infected by Streptococcus pyogenes.

Eyelid desmoplastic trichilemmoma: 2 case reports and review.

Desmoplastic trichilemmomas (DTs) are a rare variant of trichilemmomas first described in 1985. Since then, 11 cases involving the eyelid have been reported. Two cases of this study raise this total to 13. DTs are often clinically confused with basal cell carcinoma and must be proven by biopsy to obtain the correct diagnosis. These lesions are more commonly present in patients older than 50 years. Despite the fact that they are benign, they are often associated with basal cell carcinomas and as such should be managed carefully, requiring excisional biopsy with frozen borders or Mohs controlled margins.

Hybrid Neurofibroma/Schwannoma of the Supraorbital Nerve: Clinicopathologic Correlation of a Rare Tumor.

Benign peripheral nerve sheath tumors such as schwannoma and neurofibroma have long been considered distinct entities. Recently, hybrid tumors demonstrating combined morphological features of neurofibroma and schwannoma have been described, primarily in dermal locations. Only 1 case of hybrid peripheral nerve sheath tumor of the orbit has been reported in the literature. Hybrid morphology is important to recognize because of its association with the neurofibromatoses, including schwannomatosis; however, the paucity of literature on orbital hybrid peripheral nerve sheath tumor poses a diagnostic challenge. This article describes a case of hybrid neruofibroma/schwannoma of the orbit arising from the supraorbital nerve with clinicopathologic correlation.

Reply: To PMID 22681954.

In the case of the child reported by Chelnis and colleagues the IOP also was not measured, although it is described to be “increased by palpation” on the first postoperative day already associated with the dilated and nonreactive pupil. It is my belief that Urrets-Zavalia syndrome is, as speculated by the authors, the result of acute elevation of the IOP to significant enough levels to cause iris ischemia, secondary visible pupillary sphincter atrophy, resultant permanent mydriasis, and often later occurrence of isolated anterior lens opacities (Glaukon-Flecken). Although until now unreported after goniotomy surgery, it is a development that I have seen many times in children referred for care after successful or failed glaucoma procedures, including goniosurgery performed with the use of a viscoelastic. It is also seen after traumatic ocular injuries, with excessive reflux of blood into the anterior chamber after goniosurgery and with delayed or untreated acute angle-closure glaucoma. The report by Chelnis and colleagues is an important contribution to alerting clinicians to the risk of ischemic ocular abnormalities secondary to acute glaucoma and to advance the understanding of Urrets-Zavalia syndrome. This narrative should include consideration of the potential window of opportunity to relieve the pressure and the necessary pressure level for it to develop. I would offer that when the postoperative IOP is greater than the preoperative IOP the child may be at risk. Clearly the therapeutic window of opportunity is less than 24 hours and may be much less. I would suggest that children must be monitored carefully for the occurrence of this acute glaucoma and would offer that its development secondary to blood or a viscoelastic represents a pediatric ocular emergency dictating immediate surgical anterior chamber washout. Because of the suspected short therapeutic window of opportunity, prevention of this syndrome may be a more realistic and valuable concern. The risk of viscoelastics must be appreciated. The historical uncertainty explaining Urrets-Zavalia syndrome should no longer be misunderstood and impede prevention, recognition, and prompt care of this important childhood glaucoma.

Neuroepithelial Cyst of the Optic Nerve in a Newborn.

Cystic lesions of the optic nerve are uncommon findings that have been associated with neoplasms, systemic disorders, or described as idiopathic. They have been described in a wide range of ages, with few congenital cysts having been described in infants. Surgical excision of these lesions may lead to significant morbidity as previously reported cases often involved transection of the optic nerve. The authors describe a unique case of a newborn with a rare neuroepithelial cyst of the intraorbital optic nerve that was successfully excised while sparing the optic nerve, ultimately resulting in the resolution of an afferent pupillary defect. This case demonstrates that careful surgical excision of a benign optic nerve cyst can be performed without significant morbidity to potentially preserve vision.

Office-based needle aspiration of idiopathic acute subperiosteal hematoma

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