Congenital cataract with persistent fetal vasculature without anterior segment pathology associated with best postoperative visual acuity: Study
Congenital cataract with persistent fetal vasculature (PFV)
represents a complex and challenging condition in paediatric ophthalmology.
PFV, formerly known as persistent hyperplastic primary vitreous, is a rare
developmental anomaly characterized by incomplete regression of the primary
vitreous and embryonal hyaloid vasculature, leading to a spectrum of ocular
abnormalities, including cataract and retinal detachment. In the worst case,
the persistent hyaloidal stalk is integrated anteriorly into the posterior lens
capsule and posteriorly to the vascular bundle of the optic nerve head (ONH)
and exerts tractional forces radially in all directions. Ultimately, tractional
retinal detachment (TRD) may occur due to retrolental fibrovascular tissue
proliferation and contraction. In PFV eyes, concomitant retrolental stalk,
avascular peripheral retina, and regional capillary dropout have been observed
with fluorescein angiography examination.
The management of unilateral congenital cataract with PFV
poses unique therapeutic challenges. Surgical intervention is often required to
address the congenital cataract and associated PFV-related anterior and/or
posterior segment complications, with techniques such as lensectomy,
vitrectomy, membranectomy, and retinal detachment repair. And in many cases
multiple surgical interventions are needed. However, in cases with advanced
pathology, including ONH hypoplasia, severe tractional retinal detachment, or
microphthalmia, surgery is generally not a preferred choice since post-operative
vision is often modest. Postoperatively, achieving optimal visual outcomes in
the PFV cases can be hindered by factors including amblyopia, nystagmus,
glaucoma, proliferative vitreoretinopathy (PVR), and refractive errors. Case
selection for surgical treatment is of great importance, since long-standing
complications such as sympathetic ophthalmia, ie, bilateral granulomatous
panuveitis, may occur postoperatively and need to be considered.
Authors carried out a real-world single-centre study with
the aim to provide a comprehensive overview of the clinical features,
management strategies and final visual outcomes for eyes with unilateral
congenital cataract and PFV. By elucidating the complexity of this rare
paediatric condition, clinicians could enhance their understanding and improve
the individually tailored management of affected children, ultimately
optimizing visual outcomes and quality of life
Retrospective observational single-center study was
conducted between January 1, 2009, and December 31, 2019, at Helsinki
University Hospital. The national cohort encompassed 82 children aged from
birth to 15 years who underwent lensectomy, 3-port vitrectomy, or a combined
procedure, with the objective of achieving visual rehabilitation. Among the
surgical cohort, paediatric cases with International Classification of Disease
(ICD-10) codes Q14.0 for PFV and Q12.0 for congenital cataract were identified
and analyzed. Data were collected through a comprehensive review of medical
records, encompassing clinical history (birth weight), gender distribution,
ocular parameters (laterality, intraocular pressure [IOP], visual acuity [VA]),
details of cataract and vitreoretinal surgical interventions, indications for
surgery, postoperative ophthalmic complications, as well as evaluations of
functional and anatomical outcomes.
The cohort consisted of 11 children, ranging in age from 6
months to 12 years. Surgical intervention resulted in the attainment of at
least light perception vision in nine of the operated eyes, representing 81.8%
of cases. Among these, two eyes (18.2%) achieved hand motion vision, while 5
eyes (55.6%) achieved vision of finger counting or better. Additionally, two
eyes (18.2%) achieved visual acuity measurable on the Snellen chart. However,
one eye (9.1%) experienced complete vision loss, while the contralateral eye
developed sympathetic ophthalmia.
Congenital cataract with PFV devoid of anterior segment
pathology tends to correlate with the most favorable postoperative visual
outcomes. Conversely, eyes presenting with anterior segment pathology, such as
microphthalmia, or exhibiting complex posterior segment pathologies like macular
TRD, optic nerve hypoplasia, or foveal hypoplasia, are associated with a less
promising visual prognosis. Most children undergoing surgical intervention
achieve only modest improvements in vision. Given the potential occurrence of
sympathetic ophthalmia following surgery, meticulous case selection for
surgical management is paramount. Furthermore, delving deeper into the
pathogenesis of PFV is imperative to enhance our understanding of this
condition.
Source: Loukovaara; Clinical Ophthalmology 2024:18
https://doi.org/10.2147/OPTH.S472028
