Dr. Parth Amardeeep Patil, Dr. Prema K. V. Subramaniam, Dr. Parag K Shah,
Dr. Narendran V
Running title
Visual outcome after surgery in cases of persistent fetal vasculature.
Abstract
Aim
To evaluate the surgical results of patients with persistent fetal vasculature (PFV).
Methods
This is a retrospective study from period of 2006-2016 conducted at our institute.Fifteen eyes of 13 patients with PFV were included in the study. In each case the mode of presentation, type of PFV (anterior or posterior), associated ocular findings, preoperative findings, type of surgery performed, visual outcome were noted from the records. Mean follow up was20 months (range two weeks to six years).
Results
Fifteen eyes of 13 patients were involved, with six (46.2%) male and seven (53.8%) females. Two (15.4%) patients were diagnosed with bilateral PFV. Eight (53.3%) right eyes and seven (46.7%) left eyes were included in the study. Twelve (92.3%) patients presented with leukocoria and one (7.7%) with defective vision and one (7.7%) with squint. Thirteen (83.3%) eyes had both anterior and posterior PFV, two (17.7%) had posterior PFV. Associated finding- two patient had disc coloboma, two patient had vitreous hemorrhage. Preoperative vision not possible to assess in nine patients, one patient vision was 3/60 and in one was counting fingers close to face (CFCF). Initial lensectomy was done in eight (53.3%) eyes, lensectomy with vitrectomy in five (33.3%) eyes and only vitrectomy in two eyes (13.3%). Final best corrected visual acuity ranged from fixing and following light to 6/18.
Conclusion
Early surgical intervention with ambylopic therapy with regular follow up provide good visual outcome in selected patients with PFV.
Keywords
Persistent fetal vasculature, lensectomy, vitrectomy, surgical outcome, amblyopia, visual acuity.
Introduction
Persistent fetal vasculature (PFV)is an ocular assembly disorder in which fetal vasculature (hyaloid vessels and tunica vascular lentis) does not regress.[1]
It usually presents unilaterally without associated systemic findings, in normal full-term infants.[2] Majority of PFV cases are sporadic, but there has been evidence of autosomal dominant or recessive inheritance.[3,4]
PFV causesconsiderable visual morbidity in affected eyes.In this study 15 eyes of 13 patients diagnosed with PFV managed surgically over a period from 2006 to 2016 at our institute. Data was analyzed to determine visual outcome in eyes receiving surgical intervention aimed at visual rehabilitation.
Methods
The medical records of patients with PFV between February 2006 and January 2016 were reviewed retrospectively.Potential cases of PFV were screened from departmental and hospital records, including cases diagnosed as congenital cataract, microphthalmia, and leukocoria.
In this study PFV was categorized as anterior if retrolental opacity, elongated ciliary processes, or cataract was present on clinical examination. PHPV was categorized as posterior when one or more of the following features was associated, with an elevated vitreous membrane or stalk from the optic nerve: a retinal fold or retinal dysplasia, retinal detachment, or optic nerve hypoplasia. [1],[5],[12],[13] Posterior cases were further divided into mild and severe involvement.[12] Mild cases included only elevated vitreous membrane with a stalk from the optic nerve. All other cases were considered as severe involvement. Inclusion criteria for this study was cases with anterior and/or mild posterior involvement. Only these cases were chosen for surgery as prognosis for cases with severe involvement was grave even with the surgery.[12]
Diagnosis was made based on a combination of clinical examinationunder general anesthesia, and ultrasound findings.Thedifficult distinction between bilateral PFV and Norriedisease was not defined by molecular genetic analysis. Although Norrie disease has more severe hemorrhagic and dysplastic retinal detachment.[14]
Preoperative ancillary tests including B-scan ultrasound with Doppler, computed tomography (CT), magnetic resonanceimaging (MRI), and visual evoked potentials (VEP) wereperformed as directed by the treating ophthalmologist.
Three surgeons performed all procedures.
Surgical methods included lensectomy with or without anterior or core vitrectomy. All patients were treated withaphakic correction (spectacles) and occlusion therapy.
Visual acuity was assessed with ability to follow light in the age group of less than one year, cake decoration up to two years, Cardiff cards for two to three years and Kay picture charts for three to four years.
Results
Fifteen eyes of 13 patients was involved in the study, with six (46.2%) male and seven (53.8%) females. Two (15.4%) patients were diagnosed with bilateral PFV and was assigned a single case number. Eight (53.3%) right eyes and seven (46.7%) left eyes were included in the study. Twelve (92.3%) patients presented with leukocoria and one (7.7%) presented with defective vision one (7.7%) also presented with squinting of the left eye. Thirteen(87%) eyes had both anterior and posterior PFV two(13%) had posterior PFV. Associated finding- two patient had disc coloboma, two patient had vitreous hemorrhage in association with PFV. Preoperative vision not possible to assess in nine patients, one patient vision was 3/60 and in one patient vision was CFCF. Initial lensectomy was done in eight (53.3%) eyes,lensectomy with vitrectomy was done in five (33.3%) eyes and only vitrectomy was done in two patients (13.3%),the reoperation was done in one (6.7%)eye, final best corrected visual acuity ranged from fixing and following light to 6/18. Median age at the diagnosis was seven months. Median age at the surgery was seven months. Median age at the final follow up was 24 months.
Discussion
Persistent hyperplastic primary vitreous (PHPV) was first described by Reese in 1955.[13] The term persistent fetal vasculature (PFV) was introduced[1] and is a better name because it addresses the fact that persistent hyaloid vessels and tunica vascular lentis can persist and can lead to a spectrum of structural changes within the eye.
Embryologically, vitreous has three components- primary, secondary and tertiary vitreous.[15]
The primary vitreous forms during the first month of fetal life in the space between lens and retina. It consists of mesodermally derived tissue,including the hyaloid vessels and its branches and a fibrillar meshwork.Remnants of the primitive hyaloid system often persist in small infants, Anteriorly (Mittendorf’s dot behind the lens posterior pole), posteriorly(Bergmeister’s papilla at the optic disc or more extensively(hyaloid vessels).
The secondary vitreous or definitive adult vitreous forms during the second month of embryonic development it composed of 99% of water bound with collagen and hyaluronic acid.
The tertiary vitreous, developed during the fourth month of gestation,forms the zonules of Zinn which suspend the lens.
PFV can predominantly present with tunica vasculosalentis without posterior hyaloid component when termed as anterior PFV. PFV with predominant hyaloids component is termed as posterior PFV.
PFV can be associated with variable amount of retinal dysplasia.
Majority of the cases of PFV are sporadic. No single gene is isolated with PFV. Association with PAX6 gene is recently documented in optic nerve head anomalies which include PFV.[16] Linkage to 10q11-q21 has also been reported.[17]
Systemically PFV can be associated with oculo-palatal-cerebral syndrome, intrauterine herpes simplex infection, Intrauterine exposure to clomiphene, oral-facial-digital syndrome, MPPC (microcornea,posterior lenticonus,persistent fetal vasculature and coloboma).[18],[19]
Bilateral PFV is very rare.[4],[20]-[22]Norries disease is to be excluded before its diagnosis.
PHPV management is based on the type (anterior/ posterior) and extent of anterior and posterior segment involvement which in turn determines the visual outcome. Posterior PHPV has been associated with a poor visual outcome in previous studies.[1],[5],[8]-[12] In all patients in our study, visual acuity ranged from counting finger to 6/18which predicted the absence of severe posterior involvement. As per the previous studies, earlier surgical intervention resulted in better visual outcome.[5],[6] Also the patients with unilateral PFV and gradually deteriorating visual acuity have better prognosis.[7] However in our study surgical intervention as late as at the age of 108 months resulted in better visual outcome. These contradictory findings were may be due to small patient numbers, short follow up time, and/or a selection for surgery of patients with only minimal posterior segment disease.
Other factor analyzed in the study (sex and laterality) were not found to be significantly associated with visual outcome. Other factors such as compliance with occlusion and correction of refractive errors may influence outcome, but precise measurement of all potential confounders was limited by the retrospective nature of the study
Optimal timing of surgery aimed at visual rehabilitation is presumably governed by the early critical periods of visual development.[23],[24]The study findings support the value of surgical intervention to maximize visual potential in selected patients.
References
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| Case no | sex | Eye | Age
At diagnosis |
Pre-op
BCVA |
PFV
type |
Indication for surgery | Type of surgery | Age
at surgery |
Date
of surgery |
Age
at final follow up |
Date at final follow up | Visual acuity at final follow up | Final follow up in months | |
| 1 | F | RE | 5 | NR | A/P | C, RP | RE – SICS with IOL+ PPC + Anterior Vitrectomy
|
5 | 15/7/13 | 24 | 19/3/15 | RE- 6/76
LE – 6/12
|
19 | |
| 2 | M | LE | 72 | RE- 6/6 LE- 3/60
|
A/P | C | LE – SICS with IOL+ PPC
|
72 | 18/4/11 | 84 | 20/4/12 | RE – 6 / 6, LE – 6 / 36
|
12 | |
| 3 | F | LE | 108 | RE- 6/6 LE- CFCF
|
P | ODS | LE – TPPV
|
108 | 25/11 /14 | 120 | 30/11/15 | RE – 6 / 6, LE – 6/12
|
12 | |
| 4 | M | RE | 1 | A/P | C, RP | RE- TPPV + TPPL
|
1 | 9/6/09 | 60 | 26/1/16 | RE- 6/60 LE- 6/6
|
60 | ||
| 5 | F | RE | 8 | A/P | C, RP | RE – SICS with IOL+ PPC + Anterior Vitrectomy
|
9 | 9/1/15 | 10 | 10/2/15 | 1 | |||
| 6 | F | RE | 0 | A/P | C, RP | RE- TPPV + TPPL
|
2 | 25/2/14 | 24 | 15/12/15 | Child fix and follow light
|
22 | ||
| 7 | F | RE | 7 | A/P | C, RP | RE- TPPL+ membrane removal + core vitrectomy
|
8 | 9/7/13 | 24 | 12/11/14 | RE- Child fix and follow light
|
16 | ||
| 8 | F | LE | 24 | RE-6/9.5 LE- not able to pick card
|
A/P | C, RP | LE-SICS and core vitrectomy
|
24 | 6/12/12 | 48 | 10/8/15 | RE-6/6 LE-HM+
|
24 | |
| 9 | M | BE | 2 | A/P | C, RP | LE-TPPL and membrenectomy RE-SICS + PPC + anterior vitrectomy
|
2 | LE- 30-07-2015 RE- 18-08-2015
|
5 | 23/10/15 | child fix and follow light
|
3 | ||
| 10 | M | LE | 11 | P | ODS | LE-TPPV
|
11 | 17/11/15 | 13 | 28/1/16 | Child fix and follow light
|
2 | ||
| 11 | F | LE | 7 | A/P | RP | LE-ECCE with Primary posterior capsulotomywith Anterior vitrectomy
|
7 | 18/2/06 | 13 | 17/8/06 | Child fix and follow light
|
6 | ||
| 12 | M | RE | 0 | A/P | C, RP | RE- SICS with membrane removal
|
0 | 14/12/10 | 62 | 6/2/16 | RE- 2/60 LE- 6/6
|
62 | ||
| 13 | M | BE | 2 | A/P | C, RP | RE- SICS+PPC+ Ant vitrectomy LE- SICS+PPC+ Ant vitrectomy with membranectomy
|
3 | LE- 25-06-2013 RE- 16-07-2013
|
39 | 8/1/16 | BE- fix and follow light
|
36 |
C- cataract, RP- retrolental plaque, ODS- optic disc stalk, A- anterior, P- posterior, SICS- small incision cataract surgery, ECCE- extracapsular cataract excision, PPC- primary posterior capsulotomy, TPPV- trans pars planavitrectomy, TPPL- trans pars planalensectomy,