Search

Background: The change of visual function in Chediak-Higashi syndrome has not been well described. Cases: The visual function of a 12-year-old Japanese girl with ocular albinism due to Chediak-Higashi syndrome was followed by periodic ophthalmological examinations. Observations: A lack of pigmentation in the iris and ocular fundus, and pigmentary degeneration of the peripheral retina were observed. The visual loss and the constriction of visual field progressed with increasing age. The electroretinogram was extinguished at 12 years of age. Conclusions: The constriction of the visual field may be due to the pigmentary degeneration of the ocular fundus. Ophthalmologists should be aware that progressive visual loss and the constriction of visual field can occur in patients with Chediak-Higashi syndrome as they grow older.

Myopic foveoschisis (MF), a major cause of visual loss in highly myopic patients, shows varied foveal anatomic characteristics. We determined how the foveal status is related to surgical results in MF. Forty-four eyes underwent vitrectomy for MF, including internal limiting membrane (ILM) peeling and gas tamponade. The eyes were divided into three groups depending on the preoperative foveal anatomy: foveal detachment (FD, n = 17), retinoschisis (RS, n = 16), and macular hole (MH, n = 11). Best-corrected visual acuity (BCVA) and optical coherence tomographic findings preoperatively and 3, 6, and 12 months postoperatively were obtained and compared. BCVA improved two lines or more in 81% of the FD group, 50% of the RS group, and 45% of the MH group 12 months postoperatively. The FD group had significantly improved vision (P < 0.01). Visual improvement was borderline in the RS group (P = 0.057) and not significant in the MH group. Visual improvement was significantly better in FD eyes than in RS (P < 0.05) or MH (P < 0.01) eyes. In FD and RS eyes, the postoperative BCVA was significantly correlated with age (P < 0.05) and preoperative BCVA (P < 0.01), whereas visual improvement was correlated with symptom duration (P < 0.05) and preoperative BCVA (P < 0.01). Vitrectomy including ILM peeling and gas tamponade is safe and effective for MF. Patients with FD showed the most visual improvement postoperatively and therefore can obtain the most benefit from the surgery. Surgery also benefits RS and MH patients by preserving vision. The foveal status, age, duration of symptoms, and preoperative BCVA are key factors determining postoperative visual outcome in MF.