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Year : 2009  |  Volume : 57  |  Issue : 1  |  Page : 53-54

Bilateral persistent hyperplastic primary vitreous

Department of Radiology, Getwell Polyclinic and Hospital, Jaipur - 302 004, India

Date of Submission04-Sep-2007
Date of Acceptance16-Mar-2008
Date of Web Publication12-Dec-2008

Correspondence Address:
Tarun P Jain
Department of Radiology, Getwell Polyclinic and Hospital, Jaipur - 302 004
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.44487

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A case of bilateral persistent hyperplastic primary vitreous (PHPV) in a 3-month-old male infant, who had bilateral leukokoria, is presented. The child was referred for imaging with a clinical suspicion of retinoblastoma. Gray-scale ultrasound evaluation revealed an echogenic band in the posterior segment of both globes, extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of arterial flow in the band in both globes. Associated echogenic hemorrhage was also seen, which was confirmed by computed tomography. Most cases of PHPV are sporadic and unilateral, and bilateral PHPV is rare. The imaging features in this case suggest the diagnosis of bilateral PHPV and differentiate it from retinoblastoma. This entity, although infrequent, should be considered in the differential diagnosis while evaluating bilateral leukokoria.

Keywords: Computed tomography, Doppler, orbit, ultrasound, vitreous

How to cite this article:
Jain TP. Bilateral persistent hyperplastic primary vitreous. Indian J Ophthalmol 2009;57:53-4

How to cite this URL:
Jain TP. Bilateral persistent hyperplastic primary vitreous. Indian J Ophthalmol [serial online] 2009 [cited 2022 Jul 1];57:53-4. Available from: https://www.ijo.in/text.asp?2009/57/1/53/44487

Persistent hyperplastic primary vitreous (PHPV) is an uncommon condition, presenting clinically as leukokoria (white pupillary reflex), micro-ophthalmia, and cataract. Bilateral PHPV is rare. Most important differential diagnosis is retinoblastoma, which can be differentiated by imaging features.

  Case Report Top

A 3-month-old male infant presented with bilateral leukokoria. Birth history of the child was unremarkable. On initial examination, the size of the cornea looked smaller. The child was referred for ocular ultrasound examination, with a clinical suspicion of retinoblastoma. An ultrasound study was performed with a high-frequency transducer operating at 10 MHz (L5, GE Medical Systems, USA). Gray scale evaluation revealed an echogenic band in the posterior segment of both globes extending from the posterior surface of the lens capsule to the optic disc. The axial length of the globes was 16 mm, which was suggestive of micro-ophthalmia. Color doppler examination revealed the presence of blood flow in the band [Figure 1A]. Spectral analysis of this blood vessel showed arterial waveforms [Figure 1B]. Hyperechoic foci were seen in both the posterior segments adjacent to the artery, suggestive of associated vitreous hemorrhage [Figure 1B]. No calcification was seen.

Computed tomography (CT) of the orbits (Lightspeed, GE Medical Systems, USA) revealed diffusely hyperdense vitreous in both globes, suggestive of hemorrhage [Figure 2]. No solid mass or calcification was seen on either side. Subtle linear densities were seen in the vitreous of both globes, representing hyaloid artery in Cloquet's canal [Figure 2].

Based on the above findings, the patient was diagnosed with bilateral PHPV. Further investigation failed to reveal any history of similar disorder in the family of the patient. The parents were advised to get him examined under general anesthesia, so that appropriate management could be decided. However, the parents were unwilling for a procedure under anesthesia and refused for the examination. Leukokoria was thought to be due to vitreous hemorrhage.

  Discussion Top

PHPV occurs because of an incomplete regression of the embryonic vitreous and hyaloid vasculature. The primary vitreous is formed during the first month of development and contains branches of the hyaloid artery. This hyaloid artery begins to regress during the formation of the avascular secondary vitreous at 9 weeks. By the third month, the secondary vitreous, which ultimately forms the adult vitreous, fills most of the developing vitreous cavity. The primary vitreous becomes condensed into a narrow band (Cloquet's canal), running from the optic disc to the posterior aspect of the lens. [1],[2] PHPV is classified into three types: anterior, posterior, or a combination of the two. [2]

Bilateral leukokoria in a child may be due to a number of causes, of which retinoblastoma, congenital cataract, retinopathy of prematurity (ROP), severe intermediate uveitis, and retinal dysplasias are the most frequent. [2] PHPV usually presents with unilateral leukokoria. [1],[2] Bilateral PHPV is rare. While analyzing patients presenting with PHPV, Pollard found two of 83 (2.4%) cases to be bilateral, [3] while Haddad et al. found seven of 62 (11%) cases to be bilateral. [4] It is important to exclude retinoblastoma in all cases of leukokoria.

Persistent hyperplastic primary vitreous has a typical imaging appearance, which allows reliable differentiation from retinoblastoma. In PHPV, gray-scale ultrasonography shows an echogenic band extending from the posterior surface of the lens capsule to the optic disc. Doppler may show arterial flow within this band representing a persistent hyaloid artery [2] [Figure 1].

CT findings of PHPV are absence of calcification, increased density of the entire vitreous, tubular intravitreal density (Cloquet's canal or nonattached retina) [Figure 2], decubitus positioning showing a gravitational effect on fluid-fluid level, micro-ophthalmia, enhancement of abnormal intravitreal tissue, and small or irregular lens. [1]

Magnetic resonance imaging (MRI) findings of PHPV consist of a tubular structure, representing the hyaloid vessel; a funnel-shaped retinal detachment, with the subretinal fluid hyperintense on both T1- and T2-weighted images; fluid-fluid level due to the presence of hemorrhage in the subretinal space; a retrolental mass; micro-ophthalmia, and vitreous hemorrhage. [5]

PHPV can be differentiated from retinoblastoma by the absence of a calcified mass, artery running through Cloquet's canal, and typical signal characteristics of retinoblastoma on MRI, i.e., hyperintense on T1-weighted images and hypointense on T2-weighted images. [1] A patent hyaloid artery, as noted in this case, is not a feature of vitreoretinal dysplasias. [2] Differentiation from advanced retinopathy of prematurity (ROP) can be difficult on imaging alone. History of a premature, low birth weight infant undergoing prolonged supplemental oxygen therapy helps to distinguish it from bilateral PHPV. [1]

Complications of PHPV include rupture of the lens capsule, cataract formation, intraocular hemorrhage, secondary glaucoma, tractional retinal folds, and subsequent phthisis bulbi. The friability of the hyaloid vasculature predisposes to vitreous hemorrhage, which was noted in both globes in the present case. Majority of patients with posterior PHPV never obtain useful vision. [2],[3]

The imaging features in this case point toward the diagnosis of bilateral PHPV. This entity, although rare, should be considered in the differential diagnosis of retinoblastoma while evaluating bilateral leukokoria.

  References Top

Mafee MF. The Eye. In: Som PM, Curtin HD, editors. Head and Neck Imaging. 4th ed. St. Louis: Mosby; 2003. p. 441-527.  Back to cited text no. 1
Sanghvi DA, Sanghvi CA, Purandare NC. Bilateral persistent hyperplastic primary vitreous. Australas Radiol 2005;49:72-4.  Back to cited text no. 2
Pollard ZF. Persistent hyperplastic primary vitreous: diagnosis, treatment and results. Trans Am Ophthalmol Soc 1997;95:487-549.  Back to cited text no. 3
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
Sun MH, Kao LY, Kuo YH. Persistent hyperplastic primary vitreous: Magnetic resonance imaging and clinical findings. Chang Gung Med J 2003;26:269-76.  Back to cited text no. 5


  [Figure 1], [Figure 1A], [Figure 1B], [Figure 2]

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