Persistence of primary vitreous in premature babies

Authors

Abstract

Persistence of the primary vitreous is a congenital anomaly linked to the persistence of the fetal hyaloid system and the hyperplastic primary vitreous. It constitutes a group of complex ocular malformations whose different clinical forms have heterogeneous functional prognoses. It is an isolated, unilateral condition that usually occurs in full-term infants. We report a case of bilateral primary vitreous persistence in 35-week premature amenorrhea, which manifested itself by bilateral leukocoria and early respiratory distress (Silverman 1/10). On clinical examination, there was no malformation syndrome. The radiological assessment showed discreet hydrocephalus on transfontanellar ultrasound and colpocephaly associated with the persistence of the bilateral primary vitreous on a brain scan. This justified treatment in neonatal intensive care during the first week of life. Persistence of the primary vitreous is rare and difficult to manage. Treatment is adapted on a case-by-case basis for early and specific surgical and medical treatment. The prognosis of this malformation is both anatomical and functional; despite good rehabilitation, the functional prognosis remains disappointing, probably due to an overall malformation ocular state.

Keywords

Introduction

Persistent primary vitreous is a congenital anomaly related to the persistence of the fetal hyaloid system and hyperplastic primary vitreous ¹. Remnants of the fetal hyaloid system can induce the growth of a fibrovascular retrolental mass of variable size that can impede proper retinal development and lead to varying degrees of microphthalmia accompanied by lens opacification.
It is an isolated and unilateral condition that usually occurs in full-term infants; it is most often revealed by leukocoria. The diagnosis is based on a precise and complete examination of the child’s eye, often under general anesthesia, supplemented by ocular ultrasound. Although the etiology is assumed to be identical in each of its three variants, the persistent hyperplastic vitreous is further subdivided into three clinical forms; this classification depends on the ocular structures affected; we thus distinguish the anterior form, which corresponds to the classic description and the posterior form which is rarer, their association at all possible degrees being the most frequent form clinically. The treatment is adapted to the case by case; amblyopia must be managed throughout the sensitive period. The prognosis of this malformation is both anatomical and functional, in fact, today, the treatment of children with persistent hyperplastic vitreous is focused on the recovery of useful vision as well as obtaining an acceptable aesthetic result.
In this work, we report a case of bilateral persistence of the primary vitreous in a premature baby of 35 weeks of amenorrhea, which manifested at birth by bilateral leukocoria.

Case Presentation

It was a premature baby of 35 weeks of the female sex, 2nd of a family of 2 of a mother aged 27, born to 1st-degree consanguineous parents, by vaginal delivery at the end of pregnancy followed complicated by a threat of premature delivery with the notion of premature rupture of membranes of 3 days, clear amniotic fluid, the mother was immune to toxoplasmosis and rubella and had in the history three spontaneous abortions. The Apgar score was 9 to 1,10 to 5,10 at 10 min of life. From birth, we noticed early respiratory distress (Silverman 1/10) and bilateral leukocoria. On clinical examination, there was no malformation syndrome.
The radiological assessment revealed slight hydrocephalus on transfontanellar ultrasound and colpocephaly associated with the persistence of the bilateral primary vitreous on a brain scan (Figure 1).
Ophthalmologic examination showed persistence of the posterior hyaloid system in the vitreous cavity (Figure 2).
Surgical treatment is discussed for our patient.

Discussion

Persistent primary vitreous is a rare condition; its incidence is estimated at 1/30,000 births, and its frequency is underestimated because a certain number of them are probably considered a simple cataract. It usually occurs in children without family or personal history, born at term after a normal pregnancy. Our work concerns a case of prematurity.
In most cases, hyperplastic persistence of the primary vitreous is most often unilateral (90%) ¹, persistence of the bilateral primary vitreous is rare and occurs only in 10%
As reported in the literature, diagnosis is usually early. Immediately after birth or in the first weeks of life ².
Three main forms of clinical expression can occur: anterior, posterior (this is the case of our patient), and mixed or combined, 25% of hyperplastic persistences of the primary vitreous are purely anterior, in this form, the posterior pole is entirely normal. The posterior form represents 12% of hyperplastic persistences of the primary vitreous in the literature, and in contrast to the clinical presentation of the anterior form, posterior persistence of the primary vitreous involves relative involvement of the posterior pole. The mixed or combined form is the most frequent, with 77% in the Alexandrakis et al. series and 63% in others.
For many authors, the spontaneous evolution of typical forms of persistent primary vitreous inevitably ends in severe complications (glaucoma, hemorrhages, retinal detachment, uveitis) requiring evisceration or enucleation ³.
On the contrary, other publications have shown that this condition is not always progressive and that some unoperated eyes would reach adulthood in a better condition than if they had undergone surgery with all its iatrogenic risks ³.
Therapeutic management of primary vitreous aims to restore vision by clearing the visual axis and to avoid complications that can lead to enucleation (or evisceration) ⁴.

Conclusion

Persistent vitreous is a rare ocular abnormality whose origin remains unknown.
All studies support the value of early diagnosis and early intervention to maximize visual potential.
Management varies according to studies, and indications are not codified.

Declarations

References

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About This Article

Received:

October 3, 2024

Accepted:

January 13, 2025

Published Online:

February 10, 2025