Neurofibromatosis type 1 presenting with retinal detachment and laryngeal plexiform neurofibroma in a toddler.

PURPOSE: To present a 22-month-old girl with a complete retinal detachment who was found to have systemic exam findings consistent with neurofibromatosis type 1 during the course of multi-specialty exam under anesthesia. OBSERVATIONS: During examination under anesthesia, ophthalmic exam findings demonstrated retinal detachment with cyst formation, as well as peripheral non-perfusion of the retina in the left eye. Non-ophthalmic findings discovered on difficulty with intubation included a laryngeal plexiform neurofibroma and café-au-lait spots. CONCLUSIONS: Pediatric retinal detachments are uncommon compared to those in adults. Pediatric patients with neurofibromatosis type 1 can present with vision loss as the presenting symptom. Systemic signs and symptoms should be carefully screen and monitored.

Asymmetric retinopathy of prematurity in presumed fungal endophthalmitis.

PURPOSE: To report a case of asymmetric retinopathy of prematurity (ROP) in a neonate with endophthalmitis. OBSERVATIONS: A 25-week old female was born by caesarean section due to preeclampsia. The patient required supplemental oxygen after birth. The neonatal period was complicated by sepsis secondary to necrotizing enterocolitis with intestinal perforation. The patient subsequently developed endophthalmitis in the right eye. A fungal ball was seen overlying the termination of a persistent hyaloid artery. The patient also had ROP, identified at 31 weeks postconceptional age, which progressed asymmetrically and demonstrated greater severity in the eye affected by endophthalmitis. The endophthalmitis resolved with intravitreal antifungal treatment and systemic therapy. The right eye was also treated with intravitreal bevacizumab, demonstrating regression of ROP severity on follow up. CONCLUSIONS AND IMPORTANCE: The present case describes the first reported case of asymmetric ROP associated with endophthalmitis. The more severe ROP occurred in the eye with endophthalmitis suggesting that, outside of systemic factors, the local ocular inflammatory environment is important in determining the progression of ROP. Additionally, the fungal ball present in the eye affected by endophthalmitis was seen at the termination of the hyaloid artery, suggesting the hyaloid artery as the route of entry of the fungus into the vitreous.

Circumferential retinal hemorrhages after ophthalmic examination with scleral depression in an infant with anti-VEGF treated retinopathy of prematurity.

We report a case of retinal hemorrhages in a baby with retinopathy of prematurity (ROP) following examination with indirect ophthalmoscopy and scleral depression. There have been rare reports of examination-induced retinal hemorrhages during ROP screening, although those hemorrhages were diffusely scattered in the posterior pole. In this report the hemorrhages were found on the surface of the neovascular ridge. Changes in intraocular pressure caused by scleral depression may result in rupture of the fragile and immature retinal vessels, which have poor autoregulation in these premature babies. Ophthalmologists performing ROP screening examinations should be aware of the possibility of causing retinal hemorrhages with scleral depression, although the hemorrhages will resolve spontaneously.

Revascularization After Intravitreal Bevacizumab and Laser Therapy of Bilateral Retinal Vascular Occlusions in Incontinentia Pigmenti (Bloch-Sulzberger Syndrome).

No consensus exists for the treatment of retinopathy in incontinentia pigmenti (IP). Vascular ischemia leads to tractional retinal detachments if untreated. Ultra-widefield fluorescein angiography (FA) is used to follow the vascular status of the retina. A 13-week-old female with IP presented with bilateral retinal vascular occlusions in both eyes. Ultra-widefield FA showed reperfusion after treatment with intravitreal bevacizumab (IVB) and angiography-guided laser to the avascular retina. Anti-vascular endothelial growth factor treatment reduces neovascularization and allows for growth of retinal vessels. IVB and FA-guided laser to the avascular retina is an option in IP. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e33-e37.].

Persistent fetal vasculature presenting with axial elongation and platyphakia.

Leukocoria in children should always raise the concern for retinoblastoma. However, a variety of non-neoplastic conditions can also present with leukocoria, including persistent fetal vasculature (PFV), a nonhereditary, congenital anomaly caused by a failure of the fetal intraocular vasculature to regress during development. Classically PFV presents with features that make it easily distinguishable from retinoblastoma, including microphthalmia, retrolental fibrovascular membrane, central dragging of ciliary processes, and cataract. We present an atypical case of PFV in a 9-month-old boy who presented with the unusual features of axial myopia and platyphakia.

Subretinal Cysticercosis Extraction With Bimanual, 3-D, Heads-Up-Assisted Pars Plana Vitrectomy: Clinicopathological Correlation and Surgical Technique.

A 16-year-old male presented with blurred vision in the right eye after recent travel to Nicaragua. Funduscopic examination revealed subretinal cysticercosis superior to the optic nerve. The cyst was drained and excised using a bimanual, three-dimensional, heads-up-assisted pars plana vitrectomy without complications. Technical maneuvers for cyst extraction along with clinicopathological correlation are described. Postoperatively, the patient exhibits no signs of recurrence and has excellent vision. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:708-711.].

Avascular Retinal Findings in a Child With Achondroplasia.

The authors present clinical and angiographic findings in a 12-year-old girl with achondroplasia who presented with bilateral retinal peripheral nonperfusion and unilateral rhegmatogenous retinal detachment, which has not been previously described in achondroplasia. This report contributes incremental knowledge regarding aberrant retinal vascular phenomena observed in pediatric disease states and implicates the possible role of mutations in the FGFR3 gene in peripheral vascular abnormalities. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:272-274.].

First Travel-Associated Congenital Zika Syndrome in the US: Ocular and Neurological Findings in the Absence of Microcephaly.

A 6-day-old female baby with known diagnosis of congenital Zika infection was referred for ophthalmologic examination. The mother (37 years old) was referred for a pruritic rash, conjunctival hyperemia, and malaise at 12 weeks of gestation while still living in Venezuela. Upon arrival to Miami, Zika virus (ZIKV) exposure was confirmed during prenatal screening. At birth, due to the known exposure, a complete congenital ZIKV workup was performed, including brain ultrasound and MRI, which disclosed calcifications in the frontal lobe. Fundus examination revealed a hypopigmented retinal lesion in the left eye that was documented with retinal imaging. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:952-955.].