Functional and anatomical outcomes following surgical management
of persistent fetal vasculature: a single-center experience of 44 cases
Isil Sayman Muslubas
Mehmet Giray Ersoz
Received: 4 October 2017 /Revised: 17 December 2017 /Accepted: 20 December 2017 /Published online: 3 January 2018
Springer-Verlag GmbH Germany, part of Springer Nature 2018
Purpose To investigate the functional and anatomical outcomes following surgical management of persistent fetal vasculature
Methods Single-center, retrospective, interventional consecutive case series of 41 patients (44 eyes) who underwent vitrectomy
with or without lensectomy through a limbal or pars plana/plicata approach with diagnosis of anterior, posterior, or combined
anterior and posterior segment PFV were included.
Results The median age at the time of surgery was 3 months (range, 1–36 months), and the mean follow-up period was 37.2 ±
38.1 months (range, 12–164 months). Of the 44 eyes, 5 (11%) had clinical and ultrasonographic findings of anterior PFV, 5 (11%)
had posterior PFV, and 34 (78%) had components of both anterior and posterior disease. At the last visit, 38 eyes (87%) were
aphakic, 5 eyes (11%) were phakic, and 1 eye (2%) was pseudophakic. The mean Snellen equivalent VA at the final visit for the
14 (32%) eyes with measurable VAwas 20/283. Nine (20%) eyes had final VA of only light perception or no light perception. The
remaining 21 (48%) eyes behaved consistently with form vision, but the patients were not able to report VA.
Conclusions Most of the eyes achieved at least form vision, with acceptable postoperative complication rates. Functional and
anatomical outcomes are not strictly dependent on axial length, and microphthalmic eyes with mild posterior segment involve-
ment have the potential for good visual results. Outcomes after surgery for posterior PFV associated with tractional retinal
detachment are limited. Anatomical success does not always equate to functional improvement.
Keywords Pediatric vitrectomy
Persistent fetal vasculature
Persistent hyperplastic primary vitreous
Persistent hyperplastic primary vitreous (PHPV) was renamed
as persistent fetal vasculature (PFV) by Goldberg in 1997 [1,
2]. The exact etiology of PFV is not known and typical cases
are sporadic and unilateral . This entity is a complex con-
dition and has a highly variable clinical presentation [4–7].
The mechanism of regression of the hyaloid vascular system
is not fully understood; however, fetal vasculature structures
may persist abnormally after birth, affecting the anterior and/
or posterior chambers of the eye . The natural history of
untreated PFV is characterized by corneal opacification, sec-
ondary glaucoma, cataract, vitreous hemorrhage, retinal de-
tachment, macular dragging, retinal dysplasia, optic nerve ab-
normalities, and subsequently phthisis bulbi [1, 2]. A signifi-
cant number of untreated patients with severe PFV have a final
visual acuity (VA) of no light perception (NLP) [8–10].
Indication for surgical treatment depends on the severity of
the disease, the patient’s age, and the visual prognosis.
Management is based on the extent of anterior or posterior
segment involvement, or both. The removal of amblyogenic
media opacities and relief of tractional forces are the aims of
surgical intervention . Eyes with advanced posterior PFV
have poor visual prognosis despite surgical treatment, but eyes
with mild PFV usually achieve functional VA .
Part of the data presented in this study was presented as a poster at the
American Academy of Ophthalmology Annual Meeting, 2016.
* Murat Karacorlu
Istanbul Retina Institute, Hakki Yeten Cad. Unimed Center No: 19/7,
Fulya – Şişli, 34349 Istanbul, Turkey
School of Medicine, Deparment of Biostatistics, Bezmialem Vakif
University, Istanbul, Turkey
Graefe's Archive for Clinical and Experimental Ophthalmology (2018) 256:495–501