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PurposeTo examine retinal structure and changes in photoreceptor intensity after dark adaptation in patients with complete congenital stationary night blindness and Oguchi disease.DesignProspective, observational case series.MethodsWe recruited 3 patients with complete congenital stationary night blindness caused by mutations in GRM6, 2 brothers with Oguchi disease caused by mutations in GRK1, and 1 normal control. Retinal thickness was measured from optical coherence tomography images. Integrity of the rod and cone mosaic was assessed using adaptive optics scanning light ophthalmoscopy. We imaged 5 of the patients after a period of dark adaptation and examined layer reflectivity on optical coherence tomography in a patient with Oguchi disease under light- and dark-adapted conditions.ResultsRetinal thickness was reduced in the parafoveal region in patients with GRM6 mutations as a result of decreased thickness of the inner retinal layers. All patients had normal photoreceptor density at all locations analyzed. On removal from dark adaptation, the intensity of the rods (but not cones) in the patients with Oguchi disease gradually and significantly increased. In 1 Oguchi disease patient, the outer segment layer contrast on optical coherence tomography was 4-fold higher under dark-adapted versus light-adapted conditions.ConclusionsThe selective thinning of the inner retinal layers in patients with GRM6 mutations suggests either reduced bipolar or ganglion cell numbers or altered synaptic structure in the inner retina. Our finding that rods, but not cones, change intensity after dark adaptation suggests that fundus changes in Oguchi disease are the result of changes within the rods as opposed to changes at a different retinal locus.

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Purpose: To describe spectral-domain optical coherence tomography (SD-OCT) and adaptive optics (AO) imaging in hydroxychloroquine retinal toxicity., Methods: Two patients with long-term hydroxychloroquine use, subtle perifoveal ophthalmoscopic pigmentary changes, and bilateral perifoveal defects on automated Humphrey visual field (HVF) 10-2 perimetry were imaged using SD-OCT and AO., Results: SD-OCT images demonstrated loss of photoreceptor inner segment/outer segment (IS/OS) junction and a downward "sink-hole" displacement of inner retinal structures in areas of hydroxychloroquine toxicity corresponding to HVF 10-2 defects and ophthalmoscopic clinical examination findings. SD-OCT irregularities in the IS/OS junction were also seen in areas not detected on HVF 10-2. AO images showed disruption of the cone photoreceptor mosaic in areas corresponding to HVF 10-2 defects and SD-OCT IS/OS junction abnormalities. Additionally, irregularities in the cone photoreceptor density and mosaic were seen in areas with normal HVF 10-2 and SD-OCT findings., Conclusions: SD-OCT and AO detected abnormalities that correlate topographically with visual field loss from hydroxychloroquine toxicity as demonstrated by HVF 10-2 and may be useful in the detection of subclinical abnormalities that precede symptoms or objective visual field loss.

Purpose: To identify preoperative, perioperative, and postoperative risk factors for scleral buckle (SB) removal., Methods: A retrospective, consecutive, matched, case-control study. Cases included all patients undergoing SB removal between 1988 and 2007 at a single academic center. Case patients were matched against 4 randomly selected control patients who underwent SB implantation during the same year as the case patients. Odds ratios (ORs) were calculated for each factor investigated., Results: Forty cases of SB removal and 148 matched control cases were identified. Three cases of SB removal were omitted from analysis because of incomplete records. Factors associated with SB removal for any reason, according to univariate analysis, included concurrent globe-penetrating injury at the time of SB placement (OR, 24; 95% confidence interval [CI], 2.9-200), concurrent pars plana vitrectomy (PPV) (OR, 17.3; CI, 4.9-61), diabetes mellitus (DM) (OR, 7.3; CI, 1.8-30), prior long-term topical ocular therapy (OR, 4.3; CI, 1.7-11), and subsequent ocular procedures (OR, 3.4; CI, 1.5-7.5). Factors independently associated with SB removal according to multivariate analysis included concurrent globe-penetrating injury (OR, 27.3; CI, 1.7-426), concurrent PPV (OR, 11.3; CI, 2.9-45), DM (OR, 8.9; CI, 1.3-58), and subsequent ocular procedures (OR, 3.9; CI, 1.4-11). Factors that did not alter SB removal risk included patient age; sex; and type, size, or location of buckling elements used., Conclusions: Awareness of these risk factors may be valuable for the surgical planning of retinal detachment repair in patients at higher risk for subsequent SB removal and for risk stratification subsequent to SB implantation.

Purpose: Pars plana vitrectomy (PPV) in children requires that sclerotomy placement be adjusted for changing dimensions of the ciliary body during ocular development. Experience with an aged-based method for sclerotomy placement is described., Methods: Using data from previously reported morphometric studies on ciliary body length by age, an age-based method was used for planning sclerotomy location in children between 1 month and 18 years of age. Sclerotomies were placed 1.5 mm posterior to the limbus in those aged 1 to 6 months, 2.0 mm in those 6 months to 1 year, 2.5 mm in those 1 to 2 years, 3.0 mm in those 2 to 6 years, and 3.5 mm in those 6 to 18 years., Results: Between 1993 and mid-2005, 82 pediatric PPV procedures were performed using this scheme. None of the 82 procedures were complicated by inadvertent lens trauma or retinal perforation during sclerotomy placement., Conclusion: The age-based method for sclerotomy placement may provide a useful guideline for vitrectomy in children with normal ocular growth and development.

Purpose: Macular schisis or detachment is frequently observed in eyes with optic pits or colobomas. Although spontaneous resolution of the maculopathy has been reported, concurrent changes in the optic nerve coloboma have not. We report three cases of atypical optic nerve colobomas in which dynamic optic nerve changes coincide with the development and subsequent resolution of the associated maculopathy., Methods: We reviewed the records of three patients with dynamic optic nerve changes associated with maculopathy. All patients were observed for at least 6 months. Fundus photography and fluorescein angiography were used to document the optic nerve and macular changes., Results: Three patients were noted to have macular detachments without apparent optic nerve excavation. With observation, the maculopathy spontaneously resolved in each case. We documented concurrent optic nerve changes whereby atypical optic nerve colobomas became apparent over several months in all cases. In one case, we noted the simultaneous development of maculopathy in association with obscuration of a prior disc anomaly. None of the eyes had a posterior vitreous detachment. We could not identify any associated systemic conditions or reproduce the findings with external stimulation. Initial Snellen acuity ranged from 20/60 to 20/200. Final Snellen acuity ranged from 20/20 to 20/40., Conclusions: Fluctuating optic nerve changes may occur in the setting of atypical optic nerve coloboma and associated maculopathy. In cases of macular schisis or detachment where an optic nerve coloboma is not readily apparent, and no other causes are identified, consideration of a period of observation prior to therapeutic intervention seems appropriate.

Objectives: To test the feasibility of human immune globulin (IG, Gamimune N, 10%) as a new treatment for endophthalmitis, the ocular tolerance, distribution, and ability of intravitreal IG to attenuate the toxic effects of Staphylococcus aureus culture supernatant were evaluated in a rabbit model., Methods: Effects of intravitreally injected IG were assessed histologically and with Western blot analysis performed 1 to 5 days after injection. IG reactivity to products of S. aureus strain RN4220 was tested by Western blotting, using known toxins (beta hemolysin and toxic shock syndrome toxin-1) and a concentrated culture supernatant containing S. aureus exotoxins (pooled toxin, PT). Endophthalmitis was induced by intravitreal PT injection. For treatment, IG and PT were mixed and injected simultaneously, or IG was injected immediately after, or 6 hours after, PT injection. PT toxicity was graded clinically and histologically over 9 days., Results: IG persisted intravitreally at least 5 days, inducing no clinical inflammation and minimal mononuclear cell infiltration. In the endophthalmitis model, toxicity from PT was significantly reduced when IG was mixed with PT and injected simultaneously, or when IG was delivered immediately after PT. Only minimal clinically detectable reductions were observed when IG delivery was delayed 6 hours., Conclusions: Intravitreal IG is well tolerated in the rabbit eye and attenuates the toxicity of culture supernatant containing S. aureus exotoxins. Because toxin elaboration likely occurs gradually in true infection, reduced effects observed with delayed treatment in this toxin-injected model do not preclude clinical application. IG may represent a novel adjunct in endophthalmitis treatment.