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Year : 1982  |  Volume : 30  |  Issue : 1  |  Page : 43-45

Persistent hyperplastic primary vitreous

Consul Eye Clinic Mahavir Marg, Jaipur, India

Correspondence Address:
Sidharth Consul
Consul Eye Clinic, Mahavir Marg, Jaipur, 302 001
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Source of Support: None, Conflict of Interest: None

PMID: 7141585

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How to cite this article:
Consul S, Khuteja A, Mathur P. Persistent hyperplastic primary vitreous. Indian J Ophthalmol 1982;30:43-5

How to cite this URL:
Consul S, Khuteja A, Mathur P. Persistent hyperplastic primary vitreous. Indian J Ophthalmol [serial online] 1982 [cited 2023 Dec 11];30:43-5. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1982/30/1/43/27940

White pupillary reflex is always a cause for concern more so in a child or in a young adult. Among the more sinister diagnosis of retinoblastoma, congenital cataract, retinal dysplasia and intraocular inflammation-the diagnosis of persistent primary vitreous must also be kept in mind.

Although the condition of manifest Persi­stent hyperplastic primary vitreous in adult is not common[1]. -The pathological material is abundant because of enucleation mistakenly done under the diagnosis of retinoblastoma, development of intractable glaucoma, corneal opacities, shrunken globe and spontaneous vitreous haemorrhage.

We report here a case of Persistent hyper­plastic primary vitreous (circumlental type) running into adulthood without any other anomaly.

  Case report Top

A young adult of 18 yrs. presented with eso-deviation of his left eye and complaining of whitish reflex in the pupillary area since early childhood.

His was a full term, normal delivery and no history of trauma or infection was forth­coming.

Systemic examination did not reveal any abnormality. The fellow eye was normal.

On ocular examination of his left eye he had a white reflex in the pupillary area with fine vessels visible on the surface. On dilata­tion of the pupil a whitish grey mass was seen with cystic appearance in its lower outer quadrant and elongated ciliary processes at the periphery all around. Vessels on the surface could be seen coursing around the equator [Figure - 1]. Fundus could be visualised from the upper outer quadrant only on indirect

Ophthalmoscopy and the disc was normal.

Patient had finger counting close to face. anterior chamber depth, Intra-ocular pressure and the movements of the eye were normal. Corneal diameters were also normal and equal to that of right eye (corneal diameter-l0 mm & 10.5 mm). Cover test revealed poor fixation of left tye with esotropia about 20°.

On Slit lamp examination a separate lenticular section could not be obtained and a fibrovascular mass with cystoid spaces within it could be seen.

A diagnosis of Persistent hyperplastic primary vitreous (circumlental) with concomit­ant convergent strabismus was made. Squint correction was done postponing vitrectomy for a later date.

  Discussion Top

In literature[2],[3],[4],[5],[6],[7] only a few identifiable cases of persistent hyperplastic primary vitreous in adult or in those approaching adult life are cited. This is because soon after the 10 mm stage of Embryo, the vascular arrange­ment of the Embryonic eye can be divided into:- (a) The extra ocular blood system which includes the vessels of the orbit and primitive choroid. (b) The intraocular system which consists of the hyaloid artery and its branches forming the vascular capsule of the lens[8].

After 50 mm stage of embryo the vessels of posterior capsule cannot keep pace with the lens growth and growth of the eye and start shrinking and by 812 months, there is total atrophy of vasa hyaloidea propria.

However, persistence of the tunica vascu­losa lentis may manifest as a Mittendorf-dot or it may cover the entire posterior surface of lens or extend around the lens equator engul­fing it and giving rise to circumlental type of Persistent hyperplastic primary vitreous.

The clinical course of Persistent hyperplas­tic primary vitreous is varied. Unlike other anomalies of petal vascular system like persi­stent hyaloid remnants and persistent pupillary membrane-Persistent hyperplastic primary vitreous of the circumlental type is a progressive condition.

The lens initially shows a posterior polar cataract which increases till the whole lens is engulfed. Subsequently the lens may shrink or it may swell to fill the anterior chamber caus­ing intractable glaucoma. Sometimes, spontaneous recurrent haemorrhages may occur causing haemorrhagic glaucoma. Only when changes are minimal or shrinkage of lens occurs does the eye survive upto early life.

In view of the foregoing Persistent hyper­plastic primary vitreous is usually associated with other anomalies like micro-ophthalmus, small lens diameter, polar cat., glaucoma and corneal scarring. Consequently although many a case of Persistent hyperplastic primary vitreous have been reported in premature children associated with other congenital ano­malies, our case of young adult without any anomaly merits reporting.

  Summary Top

A case of Persistent hyperplastic primary vitreous (circumlental type) with concomitant convergent squint in a young adult is reported. It is unusual in that the eye was normal in all other respects developmentally. Squint correction was done postponing vitrectomy for a later date.

  References Top

Duke-Elder, S., 1964 system of Ophthalmology, Vol 3. Pt. 2 P. 772. 773  Back to cited text no. 1
Haddad, R.. Font R.L. and Reeser F. 1978. Survey Ophthalmol. 23 : 123  Back to cited text no. 2
Reese, A.B., 1955., Trans. Am. Acad. Ophthal­mol. Otolaryngol. 59: 271 (Jackson Memorial Lecture)  Back to cited text no. 3
Ford, J.C. and Irvine, A.R., 1961, Arch Ophthalmol 66 :467  Back to cited text no. 4
Spaulding, AG., 1967, Arch. Ophthalmol.77 : 666.  Back to cited text no. 5
Manschat, WA., 1958, Arch. Ophthalmol. 59 :188.  Back to cited text no. 6
Walter, JR, and Flaharty, N.W. 1959, Amer J. Ophthalmol, 47 : 491.  Back to cited text no. 7
Mann, 1., 1937, Development abnormalities of the eye, Ed. I London, Cambridge University press, P. 36.  Back to cited text no. 8


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