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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 414-415

Unilateral incomplete retinal vascularization due to congenital heart disease in a full-term newborn

Ophthalmology Service, SAMIC Pediatric Hospital, Buenos Aires, Argentina

Date of Submission27-Sep-2020
Date of Acceptance22-Feb-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Franco Benvenuto
Ophthalmology Service at Hospital Nacional de Pediatria Prof. Dr. Juan P. Garrahan, Buenos Aires
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3077_20

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Keywords: Pediatric retina, retina, ROP

How to cite this article:
Benvenuto F, Diaz Gonzalez L, Mansilla MC, Fandiño A. Unilateral incomplete retinal vascularization due to congenital heart disease in a full-term newborn. Indian J Ophthalmol Case Rep 2021;1:414-5

How to cite this URL:
Benvenuto F, Diaz Gonzalez L, Mansilla MC, Fandiño A. Unilateral incomplete retinal vascularization due to congenital heart disease in a full-term newborn. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 28];1:414-5. Available from: https://www.ijoreports.in/text.asp?2021/1/3/414/320102

A 6-day-old full-term baby girl was hospitalized in the intensive care unit and had a history of tetralogy of Fallot (TOF), diaphragmatic hernia, and polydactyly in one of her hands. The external eye examination was normal. The fundus examination of the right eye (OD) was difficult due to poor pupillary dilation. Macular edema, preretinal hemorrhages, vascular tortuosity, arteriovenous shunts, and avascular retina were observed [Figure 1]a. The left eye fundus was normal [Figure 1]b.

The condition was interpreted as ischemic retinopathy with vascular thrombotic cause related to the cardiological congenital disease and retinal vascularization interruption. Laboratory tests for congenital infectious diseases and molecular genetic testing were negative.
Figure 1: (a) Right eye fundus photograph at initial exam showing retinal hemorrhages, macular edema, arteriovenous shunts, and avascular peripheric retina. (b) Left eye fundus with normal retinal vasculature

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It was decided to perform treatment with intravitreous bevacizumab 0.625 mg in 0.025 mL. One week after the injection, pupillary dilation improved, the vitreous became clearer, there were fewer hemorrhages and vascular tortuosity. An extensive retinal without vessels was seen [Figure 2].
Figure 2: Right eye fundus photograph 1 week after intravitreal bevacizumab injection, showing improvement of the macular edema and retinal hemorrhages. An extensive avascular retina can be seen

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  Discussion Top

Retinal vascular dysgenesis can occasionally occur in full-term infants. The risk factors for the development of such retinopathy changes in full-term newborns are oxygen administration, low-birth weight, multiple births, hypoxia, or sepsis. Some of them are considered Retinopathy of Prematurity-Like retinopathies.

Cyanotic heart disease, such as tetralogy of Fallot (TOF), is known to cause retinal vascular tortuosity, retinal hemorrhages, retinal venous occlusions, and macular edema.[1],[2] However, it is unlikely to find retina vascularization interrupted.[3]

There are a few reports of retinal arteriovenous anastomosis in conjunction with congenital cytomegalovirus (CMV) infection.[4] Nevertheless, our patient was negative for CMV and other congenital infections.

To our knowledge, there are no previous reports of retinal vascularization interruption due to a complication of congenital heart disease in a newborn. A prompt screening of neonates and early treatment should prevent visual impairment in these cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Vinekar A, Dogra MR, Sangtam T, Narang A, Gupta A. Retinopathy of prematurity in Asian Indian babies weighing greater than 1250 grams at birth: Ten year data from a tertiary care center in a developing country. Indian J Ophthalmol 2007;55:331-6.  Back to cited text no. 1
[PUBMED]  [Full text]  
Mansour AM, Bitar FF, Traboulsi EI, Kassak KM, Obeid MY, Megarbane A, et al. Ocular pathology in congenital heart disease. Eye (Lond) 2005;19:29-34.  Back to cited text no. 2
P Vilela MA, Colossi CG, Freitas HP, Valle GD, Pellanda LC. Ocular Alterations Associated with Primary Congenital Heart Disease - A Cross-sectional Study. Middle East Afr J Ophthalmol 2020;27:28-33.  Back to cited text no. 3
Tagami M, Honda S, Morioka I, Iijima K, Yamada H, Nakamura M. An unusual case of congenital cytomegalovirus infection-related retinopathy. BMC Ophthalmol 2016;16:81.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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