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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 416-417

A curious case of macular edema in persistent fetal vasculature

Disha Eye Hospitals, Department of Vitreoretina Services, Kolkata, West Bengal, India

Date of Submission10-Oct-2020
Date of Acceptance16-Jan-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Debdulal Chakraborty
Disha Eye Hospitals, 88 Ghoshpara Road, Barrackpore, Kolkata - 700 120, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3223_20

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Keywords: Macular edema, persistent fetal vasculature, persistent hyperplastic primary vitreous, persistent pupillary membrane

How to cite this article:
Chakraborty D, Saha A, Pradhan A. A curious case of macular edema in persistent fetal vasculature. Indian J Ophthalmol Case Rep 2021;1:416-7

How to cite this URL:
Chakraborty D, Saha A, Pradhan A. A curious case of macular edema in persistent fetal vasculature. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Sep 24];1:416-7. Available from: https://www.ijoreports.in/text.asp?2021/1/3/416/320114

A 13-year-old boy presented with a 2-week history of decreased vision (20/200) in left eye. There was no history of trauma and family history was unremarkable. Slit-lamp examination revealed a quiet eye with persistent pupillary membrane (PPM) adherent to the anterior lens capsule, sparing the visual axis, translucent on retro-illumination. Higher magnification of the iris revealed mammilations [Figure 1]a, [Figure 1]b, [Figure 1]c. Fundus revealed a congenital falciform fold, with overlying fibrous tissue, dragging of the disc and adjoining retinal tissue upward, extending to the superior retinal periphery [Figure 2] suggestive of persistent fetal vasculature (PFV). Macular edema was noted on 90D examination and confirmed with spectral-domain optical coherence tomography (SD-OCT) along with thickened retinal tissue adjacent to the disc [Figure 3]a. Intraocular pressure was 18mmHg and axial-length was 22.5 mm. Other eye was within normal limits with 20/20 vision (supplementary Optos image)[Additional file 1]. Nepafenac drops were prescribed 3 times a day for 30 days. Follow-up examination after 1 month revealed improvement of vision (20/32) and partial separation of the fibrous tissue from the falciform fold, with some fibrovascular attachments still visible [Figure 4]. Resolution of macular edema was noted on SD-OCT [Figure 3]b. At 3 and 6months follow-up no recurrence of the macular edema was noted (supplementary OCT image).
Figure 1: (a): Slit lamp photograph showing PPM adherent to anterior lens capsule (white star), with a fibrous stalk of PHPV visible behind the lens (white arrow). (b): Magnified slit lamp image showing the PPM (White star) and showing iris mammilations white circle, attachment to iris (white arrow). (c): Retro illumination slit lamp photograph with PPM appearing transluscent (White star) and shadow of the fibrous stalk of PHPV (white arrow)

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Figure 2: Optos widefield image showing congenital falciform fold with overlying fibrous tissue white arrow

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Figure 3: (a) SD OCT image showing neurosensory detachment and spongiform edema along with thickened retinal tissue adjacent to the disc. (b) Follow up SD OCT showing resolution of macular oedema

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Figure 4: Optos widefield image showing fibrous tissue (white arrow) partly separated from the falciform fold (red arrow), with fibrovascular attachments still visible (yellow circles)

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  Discussion Top

Persistent fetal vasculature (PFV) is a common developmental anomaly present in 95% of newborn babies.[1] While PPM usually regress by the 1st year of life, hyaloid system remnants are known to regress by the 8th month of intrauterine life itself.[2] PFV developing macular edema in a child has never been reported. The cause of macular edema is likely to have been due to vitreo-papillary traction. Nepafenac eye drop has been widely used to treat macular edema of various aetiologies.[3] In this patient, however, the natural course of PFV leads to separation of the fibrous tissue from the falciform fold, relieving the vitreo-papillary traction and resolution of the macular edema. A careful periodic follow-up of patients with PFV is essential to keep track of any complications such as retinal detachment vitreous hemorrhage and raised IOP which often occur.[4],[5]

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

Duke-Elder S. Persistent pupillary membrane. Congenital Deformities. Part 2, Vol. 3. London, UK: Henry Kimpton; 1964. p. 775.  Back to cited text no. 1
Cook CS, Ozanics V, Jakobiec FA. Prenatal development of the eye and its adnexa. Chapter – 2. In: Tasman W, Jaeger EA, editors. Duane's Ophthalmology on CD-ROM. Philadelphia, PA: JB Lippincott-Raven Publishers; 1996. p. 40-1.  Back to cited text no. 2
Lim BX, Lim CH, Lim DK, Evans JR, Bunce C, Wormald R. Prophylactic non-steroidal anti-inflammatory drugs for the prevention of macular oedema after cataract surgery. Cochrane Database Syst Rev. 2016 Nov 1;11(11):CD006683. doi: 10.1002/14651858.CD006683.pub3. PMID: 27801522; PMCID: PMC6464900.  Back to cited text no. 3
Goldberg MF. Persistent fetal vasculature (PFV): An integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV).Edward Jackson Memorial Lecture. Am J Ophthalmol 1997;124:587-626.  Back to cited text no. 4
Cerón O, Lou PL, Kroll AJ, Walton DS. The vitreo-retinal manifestations of persistent hyperplasic primary vitreous (PHPV) and their management. Int Ophthalmol Clin 2008;48:53-62.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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