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Rubella Virus Infections
Published in Sunit K. Singh, Daniel Růžek, Neuroviral Infections, 2013
M. Jennifer Best, Susan Reef, Liliane Grangeot-Keros
The typical cataracts and pigmentary retinopathy seen in CRS were described by Gregg in 1941. The cataracts may consist of a central dense pearly white opacity (Figure 17.5e) or may be total with a more uniform density throughout the lens. Bilateral cataracts are found in about 50% of affected infants; they are usually present at birth but may not be visible until several weeks later. Cataracts, which are often accompanied by microphthalmia (Figure 17.6 a,b), are a useful marker for surveillance of CRS (Bloom et al. 2005; WHO 1999; Vijayalakshmi et al. 2007). Retinopathy is found in about 50% of affected infants. Hyperpigmented and hypopigmented areas of the retina give it a “salt and pepper” appearance, which can be a useful diagnostic indicator of CRS; however, because it does not cause any visual defects, it may not be suspected. Retinopathy is due to a defect in pigmentation and usually involves the macular areas. Glaucoma is less frequently observed than cataract. Other symptoms are pupil rigidity, cloudy cornea, corneal opacity, microcornea, iris hypoplasia, optic atrophy, anophthalmos, chronic uveitis, corneal hydrops, choroidal neovascularization, and keratoconus (Arnold et al. 1994; Vijayalakshmi et al. 2007). Some of these ocular abnormalities may occur later in life (see below).
Progressive corneal ectatic disease in pregnancy
Published in Clinical and Experimental Optometry, 2021
Devanshi Jani, James McKelvie, Stuti L Misra
Seventeen patients (n = 30) with new-onset or progressive keratoconus received corneal cross-linking after at least six months postpartum. Of the patients who developed corneal hydrops with or without corneal perforation, two women received penetrating keratoplasty postpartum for residual corneal scarring and one patient received corneal apex excision.20,24,30 One patient with corneal hydrops was simultaneously found to be hypothyroxinaemic and treated with levothyroxine 50 µg/day. Thyroid function tests returned to normal after one week and corneal hydrops improved in six weeks since onset.20 Another patient with corneal hydrops was managed with topical antibiotics and corticosteroids during pregnancy. Within eight days, the patient returned with complete resolution of corneal hydrops on examination.28
Corneal ectasia associated with posterior lamellar opacification
Published in Ophthalmic Genetics, 2021
Madeline Yung, Angela C. Chen, Doug D. Chung, Alice Barrington, Junwei Zhang, Ricardo F. Frausto, Otavio A. Magalhaes, Anthony J. Aldave
This patient was described in a previously published case report (11). In brief, a 5-day-old female infant was evaluated for bilateral cloudy corneas. She was a fraternal twin born by cesarean section at 33 weeks gestational age. The parents identified as Ashkenazi Jews with no known consanguinity and no family history of corneal disease. The patient demonstrated bilateral diffuse posterior stromal opacification and an otherwise normal ocular exam. Cycloplegic retinoscopy at 7 weeks was +6.00 both eyes (OU). Ocular findings remained stable until age 11, when the patient developed progressive corneal thinning, myopia, and irregular astigmatism. The patient progressed to acute corneal hydrops in the left eye at age 16. Corneal tomography demonstrated bilateral corneal thinning and asymmetric steepening with globular corneal protrusion. AS-OCT demonstrated posterior stromal opacification with intervening clear zones. The patient was diagnosed with PACD given the presence of posterior stromal opacification noted clinically and on AS-OCT, with normal IOPs and negative workup for systemic metabolic disease. A concurrent diagnosis of keratoglobus was made based on the clinical presentation of astigmatism, corneal steepening with globular protrusion, corneal thinning greatest at the periphery, and an episode of acute hydrops (1,24).
When to use anterior segment optical coherence tomography
Published in Expert Review of Ophthalmology, 2020
Eray Atalay, Daryle Jason Yu, Monisha Esther Nongpiur
Acute corneal hydrops is a visually debilitating complication of keratoconus that results in loss of corneal transparency ultimately requiring keratoplasty for vision rehabilitation. Several precursory AS-OCT features have been defined that may be associated with the development of this condition. Among the reported AS-OCT features predictive of hydrops were epithelial thickening and stromal thinning at the cone area along with hyperreflectivity at the Bowman’s layer and sites of Vogt’s striae [42]. The same study also revealed that hydrops did not develop in any of the eyes with stromal scarring which was judged to be a protective factor. Another study showed that although baseline values were similar, the annual change in swept-source AS-OCT measured radius of the posterior best fit sphere and minimum corneal thickness was remarkably faster in eyes that developed hydrops compared to ones that did not [34]. AS-OCT may also be instrumental in providing predictions for clinical prognosis in corneal hydrops. In their case series comprising 24 patients with acute corneal hydrops, Basu et al. reported more prolonged corneal edema in eyes with a larger and deeper Descemet’s membrane break [43]. Moreover, the resolution of hydrops and/or response to intracameral gas injection may be observed using AS-OCT.