Pulse cyclophosphamide therapy in the management of patients with macular serpiginous choroidopathy.

Purpose: To evaluate safety and efficacy of intravenous pulse cyclophosphamide (CyP) in acute macular serpiginous choroiditis (SC). Methods: Patients with acute macular SC with lesions threatening and/ or involving fovea were enrolled. All patients received CyP (1 g/m2) for 3 days followed by high‑dose oral steroids (1.5 mg/kg) tapered over 6 months and monitored for visual acuity, response to treatment and systemic side effects. Results: Eight patients (seven unilateral and one bilateral) with median age of 27 years (range: 13-40 years) were recruited. Mean visual acuity at presentation was 0.71 ± 0.35 logarithm of the minimum angle of resolution while postpulse visual acuity was 0.40 ± 0.32. Final mean visual acuity at 1‑year was 0.31 ± 0.23 (P ≤ 0.05). Three eyes had recurrence and 3 patients developed transient hair loss with no other adverse effect. Conclusion: Intravenous CyP provides rapid resolution of lesion activity and thereby helps in maintaining good functional acuity.

Novel occurrence of Axenfeld: Rieger syndrome in a patient with blepharophimosis ptosis epicanthus inversus syndrome.

is a complex eyelid malformation characterized by the classical tetrad of blepharophimosis, telecanthus, ptosis, and epicanthus inversus. It has been reported to be associated with other ocular anomalies such as euryblepharon, strabismus, nystagmus, amblyopia, microphthalmos, lacrimal drainage apparatus abnormality, extra ocular muscle abnormalities, microcornea, trabecular dysgenesis, optic nerve hypoplasias, and colobomas of the optic disk. We describe a case of BPES with Axenfeld–Rieger syndrome, a neurocristopathy characterized by maldevelopment of the anterior segment with predisposition to development of glaucoma. Interestingly, both syndromes are caused by mutations in the same class of genes, namely the winged‑helix/ forked transcription factors (FOX) involved in a variety of developmental processes.