Persistent fetal vasculature with vascularized cataract and lens cyst: A rare and unique presentation

Aafreen Bari; Suman Lata; Rajat S Gwal

doi:10.4103/ijo.IJO_2119_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 317-318

Persistent fetal vasculature with vascularized cataract and lens cyst: A rare and unique presentation

Aafreen Bari, Suman Lata, Rajat S Gwal
Cornea, Cataract and Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Date of Submission	31-Aug-2022
Date of Acceptance	13-Mar-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Suman Lata
Senior Resident, Cornea, Cataract and Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2119_22

Abstract

Persistent fetal vasculature may present as anterior, posterior, or mixed type. Its typical features include microphthalmia, retrolental fibrovascular membrane, stretched ciliary processes, Mittendorf dot, total cataract, and Bergmeister papilla. Atypical features described in literature are axial myopia, platyphakia, lentiglobus, and a normal sized cornea. Platy-phakia (Flat-Lens) is a term commonly used for antero-posteriorly flattened crystalline lens. It is a described but extremely rare finding in Persistent Fetal Vasculature and may represent absorption of the lens with fusion of vestigial lens remnants with retrolental fibrovascular membrane.[1] The spectrum of unusual features may also include unregressed vascular vesicular cataractous lens. The visual prognosis in PFV is generally guarded owing to the developmental anomaly and associated amblyopia, strabismus, glaucoma, and retinal pathologies.

Keywords: Cataract, persistent fetal vasculature, lenticular cyst

How to cite this article:
Bari A, Lata S, Gwal RS. Persistent fetal vasculature with vascularized cataract and lens cyst: A rare and unique presentation. Indian J Ophthalmol Case Rep 2023;3:317-8

How to cite this URL:
Bari A, Lata S, Gwal RS. Persistent fetal vasculature with vascularized cataract and lens cyst: A rare and unique presentation. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:317-8. Available from: https://www.ijoreports.in/text.asp?2023/3/2/317/374937

Persistent fetal vasculature (PFV), formerly known as persistent hyperplastic primary vitreous is a failure of regression of component of fetal vessels within the eye. PFV was coined by Goldberg due to involvement of both hyaloid vessels and tunica vasculosa lentis persisting in the condition.^[1] They nourish the developing lens prior to aqueous production. In 95% premature infants and 3% full-term infants, PFV can result in a spectrum of disorders. The classic presentation of PFV is leukocoria, micro-ophthalmia, and cataract. Management aims to remove any media opacity like cataract and vitreoretinal tractions causing retinal detachments.

Case Report

An infant's parents came with complaints of a white reflex in baby's left eye since birth. On gross examination, the left eye of child appeared to be smaller as compared to the right eye, with a small sluggishly reacting pupil and white cataractous lens. On B-scan ultrasonography (USG), a smaller axial length (i.e., 16 mm in the left eye as compared to 18 mm in the right eye) with stalk-like projection from the optic nerve head was noted. A diagnosis of persistent fetal vasculature (PFV) was made. On examination under anesthesia (EUA), the anterior segment examination revealed prominent hypopigmented iris collarettes and 360 degree plastered iris into the angle and on the anterior lenticular surface, which was extensively vascularized [Figure 1]a and [Figure 1]b. The lens was cataractous and wrapped with vessels all around. Ultrasound biomicroscopy (UBM) showed convex iris configuration with prominent, elongated, and stretched ciliary processes with hypoechoic cyst like cavity within the cataractous lens [Figure 2].

Figure 1: (a) Clinical photograph of left eye with prominent hypopigmented iris collarettes and plastered iris into the angle and on the anterior lenticular surface with extensive vascularization. (b) Ultrasound B-scan with stalk-like protrusion at optic disk suggestive of remnant persistent fetal vasculature in a small eye

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Figure 2: Ultrasound biomicroscopy (UBM) image highlighting convex iris configuration with prominent, elongated, and stretched ciliary processes with hypoechoic cyst like cavity within the cataractous lens

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The patient was planned for lens aspiration without intraocular lens implant, because of the smaller white to white (WTW) and shorter axial length of the left eye. Intraoperatively, the vessels on the lens surface were cauterized, and dissection to separate adherent iris from anterior capsular surface was attempted using viscoelastic devices and a Sinskey hook; however, it could not be achieved due to dense synechiae. Anterior capsule-rhexis was performed using micro-scissors and micro-forceps. The entire lens matter was noted to be vascularized. Coaxial system of irrigation and aspiration failed to aspirate the lens. Hence, diathermy was further used to remove the lens matter. Visual axis was cleared and the patient was left aphakic. Post-operatively, the patient had hyphema (of around 2mm) and the vitreous hemorrhage, which cleared at 3-month follow-up.

Discussion

PFV is a pleomorphic disorder of ocular development due to incomplete regression of fetal hyaloid vasculature and its varied clinical presentations pose diagnostic and surgical challenges.^[2] Cases with stretched ciliary processes predispose to higher adverse effects and poor visual outcomes.^[3] A study by Wang et al.^[4] described histopathological characteristics such as fibrocyte proliferation of capsular and lenticular tissue with hyaline degeneration and calcification, in cases of PFV. Surgical techniques described in these cases include use of endo-diathermy to control bleeding and intraocular scissors to disinsert the ciliary process from an abnormally thickened posterior capsule.^[5] Post-operative complications include hyphema, cataract, corneal opacity, vitreous hemorrhage, and retinal detachment.

Our case exhibits a challenging instance of PFV, where the patient had both anterior and posterior PFV. This case was surgically challenging owing to the extensive vascularization, lenticular cyst (challenging to perform irrigation and aspiration in such a case), and small axial length. Despite the use of diathermy to cauterize all vessels, post-operatively there was hyphema and vitreous hemorrhage. This was due to a completely vascularized lens. The vesicular vascularized cataractous lens suggests a halt in development at embryonic phase. Such cases generally have extremely guarded visual prognosis, and clearing the visual axis does not warrant visual rehabilitation. This case report highlights the unique presentation of unregressed PFV with lens cyst, management strategies, and surgical complications.

Conclusion

Persistent fetal vasculature is now an extensively studied entity. Advanced diagnostic strategies and treatment modalities have made the management of PFV simpler and more effective. Early intervention with apt post-operative therapy gives fair chance for optimal visual rehabilitation.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2.	Goldberg MF. Persistent fetal vasculature (PFV): An integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). LIV Edward Jackson Memorial Lecture. Am J Ophthalmol 1997;124:587-626.
3.	Warren N, Trivedi RH, Wilson ME. Persistent fetal vasculature with elongated ciliary processes in children. Am J Ophthalmol 2019;198:25-9.
4.	Wang J, Yan H, Du Z, Zhang J, Wang W, Guo C. Atypical anterior persistent hyperplastic primary vitreous: Report of a rare case. BMC Ophthalmol 2020;20:290. doi: 10.1186/s12886-020-01539-1.
5.	Zahavi A, Weinberger D, Snir M, Ron Y. Management of severe persistent fetal vasculature: Case series and review of the literature. Int Ophthalmol 2019;39:579-87.