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BACKGROUND: Posterior microphthalmos (PM) is a rare condition with poor visual prognosis even after amblyopia treatment. We report a case of PM with achievement of good visual acuity and disappearance of papillomacular retinal folds (PFs) over a period of 7 years. CASE PRESENTATION: A girl aged 3 years and 5 months was referred to our hospital, after poor visual acuity was identified at a medical checkup for 3-year-olds. She had severe spherical hyperopia: + 17.25 D in the right eye (RE) and + 18 D in the left eye (LE). Her corrected visual acuity was 20/200 in the RE and 20/250 in the LE. PFs were observed in both eyes on optical coherence tomography (OCT), and the diagnosis of PM was made based on the normal corneal diameter and anterior chamber depth. During the course of the disease, a gradual decrease in the height of the PFs was observed on OCT. The corrected visual acuity at age 10 years was 20/20 in the RE and 20/25 in the LE. CONCLUSIONS: The visual prognosis of PM is poor, and only one case with good visual acuity has been reported in the literature. The patient in the present case not only developed good visual acuity, but also showed improvement in macular morphology, which was not noted in previous reports. Early diagnosis of PM and early amblyopia treatment is important for the visual development in PM.

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BACKGROUND: To investigate the clinical characteristics of children with congenital ptosis, with particular attention given to the incidence of anisometropia, and the difference in axial length (AL) between the right and left eyes. METHODS: The medical charts of 55 patients with congenital ptosis at Niigata University Medical and Dental Hospital were retrospectively analyzed. Clinical characteristics, including age, cycloplegic refraction, AL, and the presence of amblyopia and its causes were analyzed. RESULTS: Age at the initial visit was 16 ± 20 (mean ± standard deviation, the same applies below) months. Of the 49 patients whose cycloplegic refraction was measured, hyperopic anisometropia, defined as ≥ one-diopter difference in spherical equivalent (SE), was observed in 1/11, 9/27 and 5/11 patients with bilateral, right, and left ptosis, respectively. Among 14/38 patients with hyperopic anisometropia involving unilateral ptosis, 13 demonstrated a larger SE in the ptotic eye than in the non-ptotic eye. The inter-eye difference in AL (AL of the ptotic eye minus that of the non-ptotic eye) in six patients with unilateral ptosis and hyperopic anisometropia ipsilateral to the ptotic eye (-0.29 ± 0.40 mm) was significantly smaller than that in three patients with unilateral ptosis and no hyperopic anisometropia (0.38 ± 0.29 mm). CONCLUSIONS: At our institute, children with congenital ptosis had a high incidence of hyperopic anisometropia ipsilateral to the ptotic eye. Furthermore, this condition was associated with a shorter axial length. These results indicate that refractive correction for hyperopic anisometropia is important for proper visual development in children with congenital ptosis.

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The purpose of this study was to investigate the clinical characteristics of Japanese patients with optic nerve hypoplasia (ONH), with particular attention to the prevalence of brain abnormalities. We retrospectively analysed the medical charts of 16 patients who were diagnosed with ONH and who underwent magnetic resonance imaging (MRI) at Niigata University Medical and Dental Hospital. We recorded the age, sex, laterality, initial eye and visual symptoms, best-corrected visual acuity, and brain abnormalities on MRI (excluding ONH). The median age at the first visit to the Ophthalmology Clinic was 2.4 years old. Four patients were male and 12 were female. ONH was bilateral in 11 patients and unilateral in five. Best-corrected visual acuity ranged from no light perception to 20/20. Seven patients (43.8%) had brain abnormalities including agenesis of the septum pellucidum, pituitary gland hypofunction, cerebral dysplasia, and West syndrome. Five of these seven patients had general manifestations since the neonatal or infantile period. Our study revealed the prevalence of brain abnormalities associated with optic nerve hypoplasia in Japanese patients at a single institute. Because two of 11 patients had no general manifestations since the neonatal or infantile period but demonstrated brain abnormalities, MRI should be performed to investigate all patients with ONH.