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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 30  |  Issue : 3  |  Page : 203-205

Persistent hyaloid artery with posterior polar cataract in a young male: A rare presentation


1 Department of Ophthalmology, Adesh Institute of Medical Sciences and Research, Bathinda, Punjab, India
2 Department of Ophthalmology, Adesh Medical College and Hospital, Ambala, Haryana, India
3 Department of Ophthalmology, Government Medical College, Patiala, Punjab, India

Date of Web Publication17-Dec-2018

Correspondence Address:
Rajwinder Kaur
Department of Ophthalmology, Adesh Institute of Medical Sciences and Research, Barnala Road, Bathinda - 151 109, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_64_18

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  Abstract 

Persistent fetal vasculature (PFV) is a rare congenital developmental malformation of the eye which results from a failure of the fetal hyaloid artery to involute. PFV is usually characterized by microphthalmia, a shallow anterior chamber, elongated ciliary processes, a posterior subcapsular cataract, and a fibrovascular stalk that extends from the optic disc to the lens. It is a rare and sporadic disease with an overall prevalence of 0.064%. We report a case of a young male who presented with decreased vision in the right eye. On examination, it was diagnosed with complete persistent hyaloid artery. The patient was advised regular follow-up.

Keywords: Bergmeister's papilla, fibrovascular stalk, Mittendorf's dot


How to cite this article:
Kaur R, Khan B, Syal E, Sidhu H, Kaur M. Persistent hyaloid artery with posterior polar cataract in a young male: A rare presentation. Kerala J Ophthalmol 2018;30:203-5

How to cite this URL:
Kaur R, Khan B, Syal E, Sidhu H, Kaur M. Persistent hyaloid artery with posterior polar cataract in a young male: A rare presentation. Kerala J Ophthalmol [serial online] 2018 [cited 2019 Jan 16];30:203-5. Available from: http://www.kjophthal.com/text.asp?2018/30/3/203/247589


  Introduction Top


The central hyaloid artery supplying nutrition to the fetal lens is seen during approximately 20 weeks' gestational age and undergoes involution in the third trimester. Regression is usually completed at birth. Persistence of the hyaloid vascular system is seen in 3% of full-term infants and 95% of premature infants.[1]

It may be present as Mittendorf's dot located at the posterior lens capsule or as Bergmeister's papilla located at the optic disc.[1] Persistent hyaloid artery extending from the disc up to the posterior lens capsule is uncommon.[1] Here, we present a case of the complete persistent hyaloid artery.


  Case Report Top


A 30-year-old male patient presented to the ophthalmology outpatient department with a complaint of decreased vision right eye. He was nonhypertensive and nondiabetic, with no history of ocular trauma or surgery. He was born full term with uneventful natal and postnatal period and no developmental delay. Family history was unremarkable. Systemic examination revealed both kidneys on the right side on ultrasound abdomen.

On ocular examination, the best-corrected visual acuity was 20/60 in the right eye and 20/20 in the left eye. Intraocular pressure was 11 mmHg in both the eyes. Examination of the left eye was normal. Slit lamp examination of the right eye revealed a thick posterior polar cataract [Figure 1]. Fundus examination depicted white stalk of fibrovascular tissue extending from the optic nerve near its nasal margin to the posterior lens capsule [Figure 1]. The retina around the optic nerve was drawn up in the peripapillary region as a Bergmeister's papilla. A Mittendorf's dot, the anterior attachment was observed at the nasal part of the posterior lens capsule as a significant, circular posterior polar cataract.
Figure 1: Fundus picture showing vascular stalk from back of cataract to posterior in red light (left) and red free light (right)

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Further examination showed the anterior chamber depth of 3.31 and 3.37 mm, lens thickness of 4.38 and 4.31 mm, central corneal thickness as 458 and 474 mm, and axial length of 22.58 and 23.61 mm in the right and left eye, respectively. Thus, the axial length of the right eye was 1.04 mm shorter than the left eye. The horizontal and vertical corneal diameters were 11 and 10.5 mm, respectively, in both eyes.

B-scan ultrasound right eye showed the presence of a moderately echogenic band extending from the optic disc to the back of lens [Figure 2]. Fluorescein angiography of the right eye revealed a partially patent artery within the fibrovascular stalk, documented with partial filling of the vessel near the disc [Figure 3]. Spectral domain optic coherence tomography at the level of the optic disc revealed the presence of a hollow tubule along with perivascular glial tissue with nasal peripapillary traction [Figure 4] and [Figure 5]. With the help of all these investigative modalities, the case was diagnosed with right persistent hyaloid artery in its complete form. The patient was advised to follow-up annually.
Figure 2: B scan ultrasound showing echogenic band from back of lens to optic disc

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Figure 3: Fundus picture showing attachment of stalk at disc (left) and angiography showing partial filing of stump (right)

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Figure 4: Optic coherence tomography showing peripapillary traction

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Figure 5: 3D view of optic disc showing elevation at nasal peripapilary region

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  Discussion Top


The hyaloid artery is a branch of the ophthalmic artery, which is a branch of the internal carotid artery. It is contained within the optic stalk of the eye and extends from the optic disc through the vitreous cavity to the lens during intrauterine period.

During the 7th month of intrauterine life, the artery becomes impervious to blood in its middle part and ruptures near the center and undergoes regression during the 1st postnatal weeks.

The two ends retract and the anterior part remains faintly attached to the back of the lens throughout the life. The posterior part is thought to disappear, but according to Mann et al.,[2] it is difficult to examine the posterior part of the vitreous, and hence, minimal remnants attached to the disc may be more common than is generally supposed. Three types of the persistent hyaloid artery may occur as follows:

  1. Whole vessel – this is rare and probably arises as a result of some influence retarding development in the 7th month. The vessel is seen to pass from the optic disc across the vitreous to its attachment at the back of the lens
  2. Anterior portion (Mittendorf's dot) – a faint remnant or fibrous cord with the free posterior end is commonly seen attached to the rear of the lens. It does not interfere with vision except in for rare occasions when it induces focal, posterior cataracts
  3. Posterior portion (Bergmeister's papilla) – this is rarely found after birth, appear ophthalmoscopically as red to white tufts originating from optic disc and extending anteriorly a variable distance into vitreous.


In our case, the hyaloid vessel extended from the optic disc to lens in its complete form which is a rare presentation, particularly in young age group. Failure of regression of the hyaloid artery is a sign of abnormal eye development and is usually associated with a persistent hyperplastic primary vitreous (PHPV).[3] Despite the absence of PHPV in our case, the persistence of the hyaloid artery belongs to the same spectrum of ocular malformations, and examination showed a unilateral right cataract, which has been described as a frequent association.[4] Surprisingly, association of a duplex kidney and persistent hyaloid artery has not been reported before. In our case, the combination of duplex kidneys suggested a syndromic association of unknown etiology.

The patient requires regular follow-up to look for the early signs of complications such as retinal folds, tractional or rhegmatogenous retinal detachment, or any other unforeseen complications. A case showing connections between the branches of the persistent hyaloid artery and peripheral retinal vessels was reported in 1977.[5] Another rare case showing association of the persistent hyaloid artery with an aberrant peripheral retinal attachment was reported in 2013.[6] Another case of functional artery in a 68-year-old male patient was reported in 1999.[7]

Treatment of persistent fetal vasculature (PFV) depends on the pathogenesis of vision loss and on the anticipated visual outcome. Simple observation is the accepted treatment particularly those with good visual acuity. Cataract extraction with IOL implantation in bag/sulcus, there are high chance of bleeding introperatively, so surgery should be planned where posterior segment surgeon is available. Those at the opposite extreme with complex tractional detachment and disorganized retina are managed accordingly with lensectomy and vitrectomy with or without silicon oil. High chances of Amblyopia should be explained to the patient.


  Conclusion Top


The persistent hyaloid artery is an uncommon faulty primary vitreous regression, often unilateral and sporadic, associated with microphthalmos. Fundus fluorescein angiography should be done in all such cases to know the patency of hyaloid artery as it may be supplying some part of the retina. PFV is of considerable importance when evaluating congenital anomalies in infants and young children.

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 Top

1.
Jones HE. Hyaloid Remnants in the Eyes of Premature Babies. Br J Ophthalmol 1963;47:39-44.  Back to cited text no. 1
    
2.
Mann I. “Developmental Abnormalities of the Eye”. 2nd ed. London: British Medical Association; 1957. p. 127.  Back to cited text no. 2
    
3.
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.  Back to cited text no. 3
    
4.
Haddad R, Font RL, Reeser F. Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature. Surv Ophthalmol 1978;23:123-34.  Back to cited text no. 4
    
5.
Schwab S, Schriever D. Connections between the branches of a persistent hyaloid artery and the peripheral retinal vessels. Ber Zusammenkunft Dtsch Ophthalmol Ges 1977;74:793-4.  Back to cited text no. 5
    
6.
Sheth JU, Sharma A, Chakraborty S. Persistent hyaloid artery with an aberrant peripheral retinal attachment: A unique presentation. Oman J Ophthalmol 2013;6:58-60.  Back to cited text no. 6
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7.
Le Rouic JF, Castelli P, Chapard J, Salabert D, Rodallec Y. Persistence of a functional hyaloid artery in an adult. Apropos of a case. J Fr Ophtalmol 1999;22:70-2.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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