Persistent fetal vasculature with microphakia, lacrimal drainage disorder, and congenital ptosis: An unusual presentation

Surbhi Khurana; Parul C Gupta; Jagat Ram

doi:10.4103/ijo.IJO_3254_20

CASE REPORT

Year : 2021 | Volume : 1 | Issue : 3 | Page : 574-576

Persistent fetal vasculature with microphakia, lacrimal drainage disorder, and congenital ptosis: An unusual presentation

Surbhi Khurana, Parul C Gupta, Jagat Ram
Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission	14-Oct-2020
Date of Acceptance	02-Jan-2021
Date of Web Publication	02-Jul-2021

Correspondence Address:
Dr. Jagat Ram
Professor, Advanced Eye Centre, PGIMER, Chandigarh - 160 012
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3254_20

Abstract

Persistent fetal vasculature (PFV) is a rare congenital ocular anomaly, characterized by failure of regression of embryonal hyaloid vasculature and is known to cause a broad range of anomalies. We report a 3-month old female infant with PFV, congenital nasolacrimal duct obstruction, and congenital ptosis in the left eye. At the time of lens aspiration, microphakia was seen after dilatation with a Malyugin ring. The child underwent uneventful lens aspiration, primary posterior capsulotomy, and anterior vitrectomy. All these features point towards role of abnormal embryological development in PFV. The meticulous surgical technique is essential to prevent intraoperative and postoperative complications in these patients.

Keywords: Microphakia, persistent fetal vasculature, persistent hyperplastic primary vitreous

How to cite this article:
Khurana S, Gupta PC, Ram J. Persistent fetal vasculature with microphakia, lacrimal drainage disorder, and congenital ptosis: An unusual presentation. Indian J Ophthalmol Case Rep 2021;1:574-6

How to cite this URL:
Khurana S, Gupta PC, Ram J. Persistent fetal vasculature with microphakia, lacrimal drainage disorder, and congenital ptosis: An unusual presentation. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2023 Jun 1];1:574-6. Available from: https://www.ijoreports.in/text.asp?2021/1/3/574/320093

Persistent fetal vasculature (PFV), a rare congenital ocular anomaly, is characterized by failure of regression of embryonal hyaloid vasculature. It is known to cause a broad range of anomalies and can present as anterior, posterior, or combined PFV; presenting as a diagnostic and surgical challenge. We report the first case, to the best of our knowledge, of microphakia in a patient of unilateral combined PFV, managed by phacoaspiration assisted with a pupil expanding device.

Case Report

A 3-month-old healthy female infant, born at 37 weeks gestation, was brought by her parents to our tertiary care hospital in India with white reflex and watering in her left eye, noticed two months back. The antenatal, perinatal, and systemic history were unremarkable. On ocular examination, the child had central, steady and maintained fixation in the right eye (OD) and uncentral, steady and unmaintained fixation in the left eye (OS), with mild congenital ptosis (1 mm), poor lid crease and exotropia of 30 Prism Diopters in the left eye. Accumulation of mucoid discharge was seen near the medial canthus of the left eye, with positive regurgitation on pressure over the lacrimal sac (ROPLAS) test, suggestive of congenital nasolacrimal duct obstruction (NLDO) OS.^[1] Slit-lamp examination revealed calcified posterior capsular plaque in the left eye [Figure 1]. Right eye anterior segment and fundus examination were unremarkable. B-scan ultrasonography revealed a thin hyperechoic stalk extending from the posterior plaque to the optic disc in the left eye, consistent with persistent remnant of the hyaloid artery [Figure 2]. A-scan ultrasonography revealed axial length OD as 18.32 mm and OS 16.86 mm, consistent with microphthalmia in the left eye. A diagnosis of left eye combined PFV, congenital NLDO, and congenital ptosis was made; the child was advised Crigler massage;^[2] and planned for examination under anesthesia (EUA). After a month, ROPLAS OS was negative OS. Intraocular pressure during EUA was 12 and 14 mm Hg OD and OS respectively using Perkin's handheld tonometer. Horizontal corneal diameters were 10.2 mm OD and 9.5 mm OS. The pupil was mid-dilated after preoperative dilatation with tropicamide 0.8%, cyclopentolate 1% and phenylephrine 5%. After taking informed consent of the parents, the child was planned for left eye lens aspiration without intraocular lens (IOL) implantation under general anesthesia. In view of non-dilatation of the pupil even after instillation of intracameral adrenaline, a pupil-expanding device Malyugin Ring (MicroSurgical Technology Inc., Redmond, Washington, USA) was applied. It was seen that the child had small capsular bag and lens, with horizontal diameter of 5.5 mm, measured intraoperatively using Castroviejo caliper; suggestive of microphakia [[Figure 3] and Video 1]. Intact zonules were stretched circumferentially. Intraoperatively, the stalk was separated from the retrolental membrane using intraocular diathermy after lens aspiration. The child underwent successful phacoaspiration, primary posterior capsulotomy, and anterior vitrectomy, and was left aphakic [Figure 4]. She was kept on a steroid-antibiotic combination with a cycloplegic. After 2 weeks, cycloplegic retinoscopy was done; the child was prescribed aphakic contact lens in the left eye and part-time occlusion OD during waking hours for at least 4 h a day was started. EUA, two months after surgery, revealed intraocular pressure of 14 mm Hg, clear visual axis, with a PFV stump attached to the disc. Oculoplastic consultation for mild ptosis was sought; no surgical intervention was done for mild ptosis, as the visual axis was clear. Pediatric consultation did not reveal any systemic association or disorder. At 6 months follow-up, child has a clear visual axis, with no evidence of glaucoma or traction on the retina by PFV stump.

Figure 1: Anterior segment photograph of the left eye showing cataract, with mid-dilated pupil

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Figure 2: B-scan ultrasonography showing a hyperechoeic stalk originating from the optic disc and attached to the posterior lens capsule, suggestive of Persistent Fetal Vasculature

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Figure 3: Intraoperative photograph showing a small capsular bag with cataract and stretched zonules, suggestive of microphakia

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Figure 4: Intraoperative photograph after uneventful lens aspiration, primary posterior capsulotomy, and anterior vitrectomy

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Discussion

Persistent hyperplastic primary vitreous, now known as PFV, is characterized by postnatal persistence of one or more components of fetal hyaloid vasculature. Anterior PFV usually has cataract, retrolental membrane, and enlarged ciliary processes. Combined PFV, the most common type, has features of anterior PFV with posterior PFV like tractional retinal detachment, retinal hypoplasia, and stalk from the optic disc.^[3] Various unusual lens abnormalities like subluxation, platyphakia, posterior lenticonus, or spontaneous posterior capsule rupture have been reported with PFV.^[4],[5],[6] To the best of our knowledge, no case has been reported suggesting association of PFV with microphakia; successfully managed using a Malyugin ring during phacoaspiration. Though intraocular lens implantation (IOL) in PFV is considered controversial, it is believed that IOL implantation decreases anisometropia and the risk of glaucoma.^[7] We decided to leave the child aphakic given the presence of microcornea, microphthalmia, and bag diameter smaller than the diameter of optic of IOL.

Role of abnormal embryological development has been hypothesized in PFV;^[8] the presence of microphakia, small capsular bag, ipsilateral NLDO, and congenital ptosis in our patient along with PFV also supports this belief. Nasolacrimal duct begins to form at fifth week, lens vesicle at sixth week; and primary lens fibers, secondary vitreous and optic stalk at seventh week of gestation.^[9] Association of PFV with NLDO has been reported previously as well. Hence, hypothesizing the role of antenatal insult in microphakia with NLDO in this patient of PFV.^[9]

Surgery in PFV can be challenging, with intraoperative hemorrhage from the membrane as the most common complication. Circumferential cauterization of the vascularised membrane and distal end of the fibrovascular stalk has helped us to avoid bleeding in all our patients in a previous study.^[8] This technique enabled us to avoid any complication in this patient as well. Visual rehabilitation of unilateral aphakia should be preferred with contact lens, as such patient can't tolerate glasses because of heavy-weight, prismatic distortion, and anisokeinia.^[10] We believe meticulous surgical techniques can drastically improve outcomes in such patients.

Conclusion

All eyes with PFV should be thoroughly examined for lens abnormalities, as they can have varied lens abnormalities.

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.

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