Yumi Suzuki

Clinical Features of Congenital Retinal Folds

PURPOSE To investigate the clinical features and prognosis of congenital retinal folds without systemic associations. DESIGN Retrospective observational case series. METHODS The characteristics, clinical course, ocular complications, and best-corrected visual acuity (BCVA) of eyes with congenital retinal folds were studied during the follow-up periods. The affected and fellow eyes were examined by slit-lamp biomicroscopy, binocular indirect ophthalmoscopy, and fundus fluorescein angiography. The parents and siblings of each patient also underwent ophthalmoscopic examinations. The BCVA was measured using a Landolt ring VA chart. RESULTS One hundred forty-seven eyes of 121 patients with congenital retinal folds were examined. Fifty-five patients (45.5%) were female. The fold was unilateral in 95 patients (78.5%), and 69 of those patients (72.6%) had retinal abnormalities in the fellow eye. The meridional distribution of folds was temporal in 136 eyes (92.5%). The family history was positive in 32 patients (26.4%). Secondary fundus complications, including fibrovascular proliferation and tractional, rhegmatogenous, and exudative retinal detachments, developed in 44 eyes (29.9%). The BCVAs could be measured in 119 eyes and ranged from 20/100 to 20/20 in 5 eyes (4.2%), 2/100 to 20/200 in 45 eyes (37.8%), and 2/200 or worse in 69 eyes (58.0%). The follow-up periods ranged from 4 to 243 months (mean, 79.7 ± 58.9 months). CONCLUSIONS These clinical features suggested that most congenital retinal folds may result from insufficient retinal vascular development, as in familial exudative vitreoretinopathy, rather than persistent fetal vasculature. Adequate management of active retinopathy and late-onset complications, especially retinal detachment, is required.

Scleral window surgery and topical mitomycin C for nanophthalmic uveal effusion complicated by renal failure: case report

PurposeTo describe the case of a 16-year-old patient with nanophthalmic uveal effusion associated with renal failure that was treated with scleral window surgery and topical administration of mitomycin C (MMC).MethodsCase report.ResultsTotal uveal effusion was restored in the nanophthalmic eyes. Partial-thickness scleral flap with deep sclerostomy was performed and topical MMC was administered to one quadrant of the equatorial sclera. The subretinal fluid resorbed gradually.ConclusionsTopical MMC might relieve the blocked transscleral outflow of intraocular fluid in the small area of a sclerostomy in young patients with nanophthalmos or in patients with renal failure in whom uveal effusion occurs repeatedly.

Glial extrusion from regressed retinoblastoma after conservative treatment.

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Congenital Rotated Macula with Good Vision and Binocular Function

BackgroundTwo patients presented with congenital rotated macula with good vision and binocular function.CasesTwo patients had retinal folds with an extremely rotated macula OD as the result of peripheral fibrous proliferation on the retina. Each macula was substantially rotated to the nasal border of the disc.ObservationsA 3-year-old girl (case 1) with best-corrected visual acuity (VA) of 0.5 OD and 1.0 OS was treated for amblyopia, after which she successfully achieved 1.0 bilaterally as well as good stereopsis of 120 seconds of arc measured with the TNO test. A 6-year-old girl (case 2) obtained a VA of 0.7 OD and 1.0 OS, and her stereopsis was of 240 seconds of arc.ConclusionsGood VA and stereopsis may be achieved by adaptation in the brain and an extremely large fusional potential at an early infantile age or a gradual shift of the macula. Appropriate treatment of amblyopia should be performed in patients with rotated macula if the macula appears normal.

Two patients with different features of congenital optic disc anomalies in the two eyes.

PurposeDescription of two patients, each with different features of congenital optic disc anomalies in the two eyes.MethodsCase reportResultsPatient 1, a 3-month-old girl, showed retinochoroidal coloboma involving the optic nerve in the right eye and optic nerve hypoplasia in the left eye. Patient 2, a 5-month-old boy, showed retinal fold extending inferiorly in the right eye and optic disc coloboma in the left eye.ConclusionsSince in both cases coloboma was seen in one eye, the optic nerve hypoplasia or retinal fold in the fellow eye of these two patients may have been related to the timing of embryonic fissure opening or closing.

Eyelash line resection for cilial entropion in patients with Down's syndrome

Aim To describe a surgical procedure for excising the eyelash line to treat cilial entropion in patients with Downs syndrome. Methods 24 Downs patients with cilial entropion and medial epiblepharon underwent eyelash line resection bilaterally. The area and density of fluorescein staining of the superficial punctate keratopathy (SPK; 0=none to 3=wide or severe) and the frequency of spectacle wear (0=no wear to 2=all day wear) were evaluated prospectively. Results The postoperative area and density of the SPK improved significantly in both eyes (p<0.001, p<0.001). The score of spectacle wear was 1.3±0.8 preoperatively which improved significantly to 1.7±0.5 after surgery (p=0.018). Five patients had a partial recurrence of misdirected eyelashes adjacent to the site of the excision. Two patients had extended excisions of the misdirected eyelashes 3 years after the initial surgery, and they did not have any further recurrences. Three other patients were followed without additional treatment because lacrimation and eye discharge were mild even with some eyelashes misdirected toward cornea. Conclusions Eyelash line resection is a simple and effective technique for cilial entropion and medial epiblepharon in patients with Downs syndrome. The surgery improves the SPK and frequency of spectacle wear. Trial registration number NCT01758718.