Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant genetic disease. It is clinically characterized by four major features; blepharophimosis, ptosis, epicanthus inversus, and telecanthus. We report a case of a 1-year-old female with BPES with unilateral anterior persistent fetal vasculature (PFV). On examination, she was found to have all the clinical features of BPES, along with calcified and partially absorbed cataract with elongated ciliary processes in her left eye. B-scan of left eye showed attached retina with no evidence of posterior PFV. Systemic examination was normal. She underwent cataract surgery with primary posterior capsulotomy with intraocular lens implantation under general anesthesia. Literature search did not reveal any previous reports of unilateral anterior PFV and BPES. The clinical features, other associations, and the difficulties in the surgical management of this condition are discussed

Management of idiopathic intracranial hypertension in an infant with bilateral congenital cataract and associated comitant sensory esotropia

In this report, we describe the management of a child with bilateral cataract, nystagmus, and comitant sensory esotropia. Routine ultrasonography done before cataract surgery revealed bilateral disc edema confirmed as idiopathic intracranial hypertension by a pediatric neurologist. The primary intervention for cataract surgery was followed by nonresolving papilledema, despite maximum medical therapy. To salvage the optic nerve function in a nonverbal child, bilateral optic nerve sheath decompression was planned with simultaneous medial rectus recessions for the persistent esotropia with the satisfactory postoperative outcome