This is a report of rare cases of full-term infants born with persistent tunica vasculosa lentis (TVL) with no retinopathy of prematurity (ROP) and no plus disease. This condition can be mistaken with iris vascular enlargement-associated plus disease, leading to unnecessary laser or intravitreal injections. The cases were treated with close observation, which resulted in complete resolution of the TVL. In conclusion, we encourage the diagnosis of TVL and careful monitoring of such cases before the intervention, as the condition may revert completely.

暂无摘要。

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) is a rare congenital developmental ocular disorder caused by incomplete regression of the embryonic hyaloid vasculature. Here we report a case of nonsurgical unilateral anterior PHPV that was managed by amblyopia treatment and resulted in an improvement of visual acuity and regression of the fetal vasculature.
METHODS: A three-year-old girl was diagnosed with unilateral anterior PHPV in the left eye, manifested with posterior pole cataract, posterior capsule opacification, tunica vasculosa lentis, and a floating hyaloid artery connected to the retrolental mass. The plaque was not large enough to fill the pupil, and conservative management along with amblyopia treatment was conducted. Nineteen months later, the visual acuity in the affected eye improved from 20/100 to 20/50 with correction, and the fetal vasculature regressed gradually and finally into a nonperfusion ghost vessel.
CONCLUSIONS: In PHPV-affected children, regression of the fetal vasculature may be observed, and conservative management and amblyopia treatment may be helpful for visual improvement.

BACKGROUND: Laser therapy has been the gold standard treatment for retinopathy of prematurity (ROP), while intravitreal bevacizumab (IVB) is reported to be of significant benefit for zone I ROP. A problem with laser therapy is that it is difficult to administer in ROP patients with severely dilated tunica vasculosa lentis and poor mydriasis. However, although IVB treatment has been performed in such severe ROP cases, only 1 report has discussed its usefulness.
UNASSIGNED: A male infant was born with a birth weight of 382 g at 23 weeks\' gestation. As visualization was poor and laser therapy could not be performed due to dilated tunica vasculosa lentis and poor mydriasis, IVB (0.625 mg/0.025 mL) was administered to both eyes. Following treatment, the ROP gradually improved, with regression of the dilated tunica vasculosa lentis and improvement of the mydriasis in both eyes.
UNASSIGNED: A male infant was born with a birth weight of 698 g at 25 weeks\' gestation. As laser therapy could not be performed due to severely dilated tunica vasculosa lentis and poor mydriasis, IVB (0.625 mg/0.025 mL) was administered to both eyes. Following treatment, the ROP gradually improved, with regression of the dilated tunica vasculosa lentis and improvement of the mydriasis in both eyes.
CONCLUSIONS: IVB is potentially more useful than laser therapy for the treatment of severe ROP with dilated tunica vasculosa lentis and poor mydriasis.

OBJECTIVE: Fibrillin-2 (Fbn2) is the dominant fibrillin isoform expressed during development of the mouse eye. To test its role in morphogenesis, we examined the ocular phenotype of Fbn2(-/-) mice.
METHODS: Ocular morphology was assessed by confocal microscopy using antibodies against microfibril components.
RESULTS: Fbn2(-/-) mice had a high incidence of anterior segment dysgenesis. The iris was the most commonly affected tissue. Complete iridal coloboma was present in 37% of eyes. Dyscoria, corectopia and pseudopolycoria were also common (43% combined incidence). In wild-type (WT) mice, fibrillin-2-rich microfibrils are prominent in the pupillary membrane (PM) during development. In Fbn2-null mice, the absence of Fbn2 was partially compensated for by increased expression of fibrillin-1, although the resulting PM microfibrils were disorganized, compared with WTs. In colobomatous adult Fbn2(-/-) eyes, the PM failed to regress normally, especially beneath the notched region of the iris. Segments of the ciliary body were hypoplastic, and zonular fibers, although relatively plentiful, were unevenly distributed around the lens equator. In regions where the zonular fibers were particularly disturbed, the synchronous differentiation of the underlying lens fiber cells was affected.
CONCLUSIONS: Fbn2 has an indispensable role in ocular morphogenesis in mice. The high incidence of iris coloboma in Fbn2-null animals implies a previously unsuspected role in optic fissure closure. The observation that fiber cell differentiation was disturbed in Fbn2(-/-) mice raises the possibility that the attachment of zonular fibers to the lens surface may help specify the equatorial margin of the lens epithelium.