|Year : 2023 | Volume
| Issue : 2 | Page : 576-577
Grouped congenital pigmented nevi of retinal pigment epithelium in an infant with aggressive retinopathy of prematurity
Megha Gulati, Bhavik Panchal, Avinash Pathengay
Faculty of Vitreoretina and Uveitis Services, L V Prasad Eye Institute, Visakhapatnam, Andhra Pradesh, India
|Date of Submission||25-Jul-2022|
|Date of Acceptance||04-Dec-2022|
|Date of Web Publication||28-Apr-2023|
H. No 43, Sector 14, Sonipat - 131 001, Haryana
Source of Support: None, Conflict of Interest: None
Keywords: Aggressive retinopathy of prematurity, bear track, grouped congenital pigmented nevi of retinal pigment epithelium
|How to cite this article:|
Gulati M, Panchal B, Pathengay A. Grouped congenital pigmented nevi of retinal pigment epithelium in an infant with aggressive retinopathy of prematurity. Indian J Ophthalmol Case Rep 2023;3:576-7
|How to cite this URL:|
Gulati M, Panchal B, Pathengay A. Grouped congenital pigmented nevi of retinal pigment epithelium in an infant with aggressive retinopathy of prematurity. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:576-7. Available from: https://www.ijoreports.in/text.asp?2023/3/2/576/374906
A preterm male with a gestational age of 35 weeks and birth weight of 2.3 kg with a postmenstrual age (PMA) of 38 weeks and 4 days and weighing 1.7 kg was referred for retinopathy of prematurity screening. On examination of both eyes with an indirect ophthalmoscope with 20 D lens as a magnifier, the cornea was clear and the child had good pupillary dilatation with no prominent neovascularization of iris. Lens and posterior segment media were clear. Disk was of medium size and the cup disk ratio was 0.2, with markedly dilated and tortuous vessels at the posterior pole with a normal-appearing fovea. Retina was vascularized till zone 2 with arcading and looping of vessels at the junction of vascularized and nonvascularised retina with a few dot-like retinal hemorrhages. A diagnosis of bilateral aggressive retinopathy of prematurity was made, and intravitreal bevacizumab 0.675 mg/0.025 ml was injected into both eyes, 1 mm from the limbus, taking aseptic precautions with the patient under topical anesthesia. At subsequent follow-up, gradual reduction in dilatation and tortousity of vessels was noted with returning of normal retinal vasculature. At 3 weeks follow-up, arcading and looping of vessels had resolved and normal retinal vascularization had extended to zone 3. At 6 weeks (PMA 44 weeks 5 days), fundus examination showed zone 3 immature retina with multiple flat, small, well-defined, outer retinal pigmented lesions, which were noted to be present in groups and distributed throughout the fundus, more in left eye than the right eye [Figure 1]a and [Figure 1]b. Macula was spared, and the lesions were of varying size and arranged in groups, with smaller lesions located at the apex and directed toward optic disk. These lesions were absent during previous visits. There was no change in vessel caliber or any signs of inflammation noted after appearance of new lesions. Ocular examination of parents was normal with no present or past systemic illness. A diagnosis of bilateral grouped congenital pigmented nevi of retinal pigment epithelium (CPNRPE) in bear track configuration was made.
|Figure 1: Fundus photograph (widefield CLARUS) of the right eye (a) and left eye (b) showing zone 3 immature retina with multiple flat, small, outer retinal pigmented lesions sparing the macula and they were noted to be present in groups, more in the left eye than the right eye|
Click here to view
At 10 weeks (PMA 49 weeks), fundus examination showed mature retina with stable outer retinal pigmented lesions. On subsequent follow-ups at 3 and 6 months, lesions were noted to be stable with no visual dysfunction.
| Discussion|| |
Pigmented fundal lesions in a child can be due to choroidal nevus, CPNRPE, congenital hypertrophy of retinal pigment epithelium, choroidal osteoma and hemangioma, congenital toxoplasmosis, congenital herpes simplex virus 1 infection, and rubella retinopathy. They can be congenital, developmental, neoplastic, or the result of infectious or inflammatory processes.
Multiple grouped, bilateral, pigmented, and flat fundus lesions with normal-appearing overlying retina, suggestive of CPNRPE, are often noted to be stationary and might run in families. Visual function is usually not affected and is reported to be associated with normal electro-oculographic findings. Ocular examination of parents was noted to be normal with no pigmented lesion of fundus.
Such a case of multiple grouped bilateral lesions in an infant with aggressive retinopathy of prematurity has never been reported before. These lesions have been described as congenital and remain probably stable as explained by Gass. Although pigmented lesions of CPNRPE appear, during which phase of antenatal life is not clear, they were not seen during the initial month of life in our preterm infant, but developed progressively and were first documented at around 45 weeks of PMA.
Multiple, grouped, bilateral, pigmented, and flat fundus lesions with normal-appearing overlying retina are suggestive of CPNRPE. CPNRPE are congenital, stable, and are not associated with visual dysfunction. Grouped type CPNRPE are present in bear track configuration, and lesions tend to be larger in peripheral fundus.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Maria Antonietta B, Maria Grazia S, Andrea S, Martina M, Giulia M, Monica Maria P. Pigmented lesions of ocular fundus: Clinical aspects. Biomed J Sci Tech Res 2019;13:MS.ID.002477.
Gass JDM. Grouped-type congenital pigment nevi of the retinal pigment epithelium “bear tracks”. In: Gass JDM, editor. Stereoscopic Atlas of Macular Diseases. Vol 2. St Louis, Mo: CV Mosby Co; 1997. p. 816-7.