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Year : 2018  |  Volume : 56  |  Issue : 2  |  Page : 132-133

Persistent fetal vasculature


Department of Strabismus and Pediatric Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam, India

Date of Web Publication6-Aug-2018

Correspondence Address:
Dr. Saurabh Deshmukh
Sri Sankaradeva Nethralaya, 96, Basistha Road, Beltola, Guwahati - 781 028, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_43_18

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How to cite this article:
Deshmukh S, Magdalene D, Gupta K. Persistent fetal vasculature. TNOA J Ophthalmic Sci Res 2018;56:132-3

How to cite this URL:
Deshmukh S, Magdalene D, Gupta K. Persistent fetal vasculature. TNOA J Ophthalmic Sci Res [serial online] 2018 [cited 2019 Oct 18];56:132-3. Available from: http://www.tnoajosr.com/text.asp?2018/56/2/132/238491



Reese first described persistent hyperplastic primary vitreous in 1955 as the abnormal persistence of fetal hyaloid system.[1],[2] Persistent fetal vasculature (PFV), a more anatomically and pathologically accurate term, was given by Goldberg.[2],[3]

Pathophysiology — PFV is the congenital abnormality of the eye which results from failure of regression of the embryonic primary vitreous and hyaloid vasculature. It is characterized by the persistence of various structures of the embryonic hyaloid vascular system along with other abnormalities such as microphthalmia, cataract, and glaucoma. PFV is unilateral in 90% of the patients and is responsible for poor vision in that eye.[4],[5]

Clinical types — PFV is classified into three presentations on the basis of anatomical extent: a purely anterior presentation, a purely posterior presentation, or a combination of the two. The combined presentation being the most commonly encountered.[6]

The purely anterior presentation has retrolental opacity, elongated ciliary processes, or a cataractous lens. The purely posterior presentation has an elevated vitreous membrane or stalk from the optic nerve, a retinal fold or retinal dysplasia, retinal detachment, or optic nerve hypoplasia. The combined presentation has features of both anterior and posterior PFV.[7],[8]

Differential diagnosis — The most common presentation of PFV is leukocoria. Hence, the differential diagnosis includes all the causes of leukocoria. The most important being the retinoblastoma, the most common intraocular tumor of childhood. Other causes could be a congenital cataract, Coats' disease, toxocariasis, and retinopathy of prematurity. Many genetic syndromes may present with PFV such as Norrie's disease, trisomy 13, and Walker—Warburg syndrome. The posterior PFV should be considered when ophthalmoscopy or ultrasonography (USG) B-scan shows total retinal detachment or retrolental fibrous tissues. Other differentials include familial exudative vitreoretinopathy, and incontinentia pigmenti.[9]

Case report — A 7-year-old male child presented with congential leucokoria in the right eye. Slit-lamp examination of the right eye (OD) showed dense membranous cataract with lenticular neovascularization and elongated ciliary processes [Figure 1]a and [Figure 1]b. USG B-scan OD showed an echogenic membrane extending from the retrolental region to the optic disc [Figure 1]c. The patient was thus advised to undergo cataract extraction, primary posterior capsulectomy, and anterior vitrectomy. Thus, we present this unique photographic documentation of unilateral PFV and emphasize the fact that even though PFV is a rare entity, it should be considered as a differential diagnosis in cases of leukocoria.
Figure 1: Slit-lamp diffuse illumination photograph of (a and b) the right eye showing membranous cataract with neovascularization of the lens and elongated ciliary processes. (c) Ultrasonography B-scan OD showing an echogenic membrane-like structure extending from the lens through the vitreous cavity to the optic disc suggestive of persistent fetal vasculature

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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.

Acknowledgment

We would like to thank Sri Kanchi Sankara Health and Educational Foundation, Guwahati, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Reese AB. Persistent hyperplastic primary vitreous. Am J Ophthalmol 1955;40:317-31.  Back to cited text no. 1
    
2.
Zhao Y, Chen D, Li J. Bilateral persistent fetal vasculature in an adult: Clinical manifestations and surgical outcomes. J Cataract Refract Surg 2010;36:1421-6.  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.
Sisk RA, Berrocal AM, Feuer WJ, Murray TG. Visual and anatomic outcomes with or without surgery in persistent fetal vasculature. Ophthalmology 2010;117:2178-83.  Back to cited text no. 4
    
5.
Jain TP. Bilateral persistent hyperplastic primary vitreous. Indian J Ophthalmol 2009;57:53-4.  Back to cited text no. 5
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6.
Quinn GE, Young TL. Congenital malformations evident in the first year of life. In: Isenberg SJ, editor. The Eye in Infancy. 2nd ed. St. Louis: Mosby; 1993. p. 401.  Back to cited text no. 6
    
7.
Pollard ZF. Persistent hyperplastic primary vitreous: Diagnosis, treatment and results. Trans Am Ophthalmol Soc 1997;95:487-549.  Back to cited text no. 7
    
8.
Pruett RC. The pleomorphism and complications of posterior hyperplastic primary vitreous. Am J Ophthalmol 1975;80:625-9.  Back to cited text no. 8
    
9.
Apushkin MA, Apushkin MA, Shapiro MJ, Mafee MF. Retinoblastoma and simulating lesions: Role of imaging. Neuroimaging Clin N Am 2005;15:49-67.  Back to cited text no. 9
    


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