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Dr.Surbhi Khurana, Dr.Jagat Ram,Dr.Ramandeep Singh

Introduction                                                                         

Congenital cataract is one of the major causes of childhood blindness in the world, amounting to about 10% of blindness in children worldwide.1,2 Persistent Fetal Vasculature (PFV) [also called as “Persistent Hyperplastic Primary Vitreous (PHPV)”] is a very rare congenital developmental malformation of the eye, which can cause congenital cataract.3,4 PFV is known to be caused due to the failure of regression of the primary vitreous. This disorder is thought to be sporadic in inheritance and is usually not associated with any systemic disorder.5 It is usually unilateral, but less than 10% are bilateral cases.6

PFV is known to have both anterior and posterior chamber anomalies and is clinically characterized by white vascularized retrolental tissue, leucocoria, shallow anterior chamber, cataract, angle closure glaucoma, elongated ciliary processes, microphthalmos and retinal detachment.5,7 Unilateral leucocoria, microophthalmia and cataract are the most common presenting features.8

Persistent Fetal Vasculature can be anterior, posterior or mixed/combined type.9 Anterior PFV (also known as persistent tunica vasculosa lentis) can manifest as cataract, glaucoma, shallow anterior chamber or retrolental vascularized membrane. Posterior PFV usually has vitreous stalk, optic hypoplasia, preretinal membranes or retinal folds.10 Mixed/combined type usually encompasses features of both anterior and posterior PFV. Combined PFV is the most common type and is usually unilateral.10,11

B-scan ultrasonography usually demonstrates a retrolental mass extending from the optic disc to the posterior lens capsule along with microphthalmos.12,13 Doppler studies reveal blood flow in these masses representing persistent hyaloid vasculature and CT scan can reveal retrolental hyperdense masses without any evidence of intralesional calcification, which differentiates this disorder from retinoblastoma.5

The aim of management in this disorder is a clear visual axis to restore vision and to prevent complications, which occur during the natural course of the disease.14 Early surgical intervention is required to prevent the disease progression and for visual rehabilitation.15 Phacoaspiration combined with anterior vitrectomy (AV), with or without IOL implantation, should be considered at an early age for children with PFV.16 The decision to operate depends upon variety of factors such as the age of the patient, visual prognosis and severity of the disease.17 Surgery is usually avoided in advanced cases of posterior PFV, with features like foveal hypoplasia, retinal detachment and severe microophthalmia.17

Anisometric amblyopia caused due to unilateral disease is one of the major challenges in this disorder, leading to a poor visual prognosis.17 Intraoperative hemorrhage from the vascularized stalk and friable hyaloid vasculature is one of the most dangerous intraoperative complications of PFV, leading to postoperative hyphema and vitreous hemorrhage.18 The vascularized PFV stalk can also cause traction on the retina, causing tractional retinal detachment.18 Secondary glaucoma is another cause for poor prognosis in PFV.19,20 We aim to study the risk factors, intraoperative and postoperative complications and outcome of cataract surgery in patients of PFV with cataract after phacoaspiration with primary posterior capsulotomy (PPC) and anterior vitrectomy (AV) with or without intraocular lens (IOL) implantation.

Methods

This study was approved by Institutional review board in accordance with the tenets of the Declaration of Helsinki. This prospective study included children, of either sex, with Persistent Fetal Vasculature (PFV) attending the Lens clinic, Retina clinic or Pediatric Ophthalmology clinic of Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh’.

This study included children of age less than 12 years suffering from anterior or combined PFV. The patients were enrolled till December, 2017 and had a minimum follow up period of 6 months after cataract surgery. This study included the following patients: age <12 years, documentation of PFV on slit lamp examination or on B-scan USG, unilateral/bilateral cataract along with PFV and with minimum follow up of 6 months. This study excluded the children with traumatic cataract, subluxated cataract, chorioretinal coloboma, complex microphthalmos or parents not willing for follow up. 

Preoperative evaluation

Detailed ocular history and complaints of the children were taken from the parents or guardian. Any genetic or infectious cause was ruled out by a detailed antenatal and perinatal history. Systemic associations were ruled out by general physical and systemic examination of the child. Ocular examination, including recording the best corrected preoperative visual acuity and fixation preference [using Central Steady Maintained (CSM) method], was performed. Visual axis and pupillary reactions were noted. Intraocular pressure was recorded with non-contact tonometer, depending upon the cooperation of the child.

Slit lamp biomicroscopic examination of the anterior segment was done to document the type of cataract and other clinical features of PFV, after dilatation of the pupil. Simultaneously, posterior segment examination was carried out with an indirect ophthalmoscope in eyes with relatively clear media. B-scan ultrasonography was carried out in all the cases for posterior segment evaluation and to evaluate the type of PFV. Axial length was measured using A-scan ultrasonography. The type of PFV was decided according to the clinical findings of the anterior segment and USG findings. The power of the IOL to be implanted in the eye was decided according to the Dahan guidelines/ SRK-T formula. Written informed consent from parents or guardians of the children was taken prior to the surgery. Surgery was performed in all children under general anaesthesia with strict vitals and ECG monitoring. Patients were admitted one day prior to surgery.

 Operative steps

All surgeries were performed by a single experienced pediatric cataract surgeon (JR). After sterile draping, two clear corneal side port incisions were made approximately 180 degree apart with a 15 degree disposable knife. A clear corneal incision was made superiorly using 2.2 mm keratome. The anterior chamber was filled with high viscosity 1.4% sodium hyaluronate (Healon GV, AMO Santa Ana, California) through the side port. Anterior capsulorhexis was made by giving nick to the anterior capsule with a needle cystitome and completed with a capsulorhexis forceps. Multiquadrant cortex-cleaving hydrodissection was done in all cases. Bimanual irrigation and aspiration for removal of cortex and thorough capsular polishing was done. The capsular bag was then again filled with sodium hyaluronate. Posterior capsulorhexis was performed similarly as done for anterior capsulorhexis or with the help of vitrectomy cutter followed by anterior vitrectomy.

The size of anterior capsulorhexis was kept around 5 mm and posterior capsulorhexis around 3-3.5mm. Cauterization of the stump was done using endocautery to prevent intraoperative bleeding. En-bloc dissection of the vascularized thick mass was done using a curved retinal scissors followed by its removal. Anterior vitrectomy was performed through two side ports using Infiniti Vision system OZil Intelligent Phaco with cut rate of 600, an aspiration flow rate of 15 cc/min and vacuum of 200 mm Hg. The capsular bag was then refilled with sodium hyaluronate. A foldable hydrophobic acrylic intraocular lens was implanted in the capsular bag/sulcus with or without optic capture or the patients were left aphakic. The residual OVD was aspirated and anterior vitrectomy was performed again if residual vitreous was visualised in the anterior chamber. All the incisions were closed with 10-0 nylon monofilament suture.

Postoperative care

After surgery, all patients received the standard regimen of topical steroids (Betamethasone 0.1%)  6-8 times a day, tapered over 6-8 weeks along with topical antibiotic (Tobramycin 0.3%) six times a day and 2% homatropine twice a day for  2 weeks. Retinoscopy of the child was done 2 weeks after the surgery. Since children with PFV are usually at higher risk of amblyopia, part time occlusion therapy of the dominant eye was started 2 weeks after surgery along with the prescription of glasses.

Post-operatively all patients were followed up on day 1, week 2, 1 month, 3 months and then at 6 months. Young uncooperative children were examined under general anesthesia using hand-held slit lamp and cooperative children with the slit lamp bio-microscope.

The statistical analysis was carried out using Statistical Package for Social Sciences (SPSS Inc., Chicago, IL, version 23.0 for Windows). Chi Square test, ANOVA test and unpaired student t-test were used for statistical comparison.

Results

Preoperative characteristics

17 eyes of 17 children were included in the study and all PFV were unilateral. There were 13 (76.5%) males and 4 (23.5%) female in the study. Right eye was involved in 13 cases (76.5%) and left eye (23.5%) in 4 cases. The minimum age of presentation was 2 weeks and the maximum age of presentation was 3 years. The mean age of presentation in our study was 1.19±1.38 years. All the 17 patients had chief complaint of white reflex in the involved eye (100%). Four patients had history of premature birth and 13 had full term birth. The fixation preference was seen at the time of presentation through CSM method. Four patients had central, steady and maintained fixation (CSM). Six patients had uncentral, unsteady, unmaintained fixation (UCUSUM) in the affected eye. Five patients had uncentral, steady and maintained fixation (UCSM). Two patients had uncentral, unsteady and maintained fixation (UCUSM). The mean IOP at the time of first examination under anaesthesia (preoperative) was 10.92±1.78 mm of Hg. The minimum IOP recorded was 8 mm of Hg and maximum IOP recorded was 14 mm of Hg

Intraoperative variables

12 patients had a vascularized plaque on posterior lens capsule. 8 patients had elongated ciliary processes. 1 patient had microspherophakia. The mean ACCC of all the patients was 5.04 mm, with minimum ACCC as 4.5 mm and maximum ACCC as 6 mm. The mean PCCC of all the patients was 4.08 mm, with minimum PCCC as 3 mm and maximum PCCC as 6 mm.

Visual outcomes and its affecting parameters

At the end of 6 months postoperatively, 12 patients (70.5%) had central, steady and maintained fixation.  4 patients (23.5%) had uncentral, steady and maintained fixation. 15 patients underwent phacoaspiration with primary posterior capsulotomy with PCIOL implantation and 2 patients underwent phacoaspiration without PCIOL. At 6 months follow up, 8 patients (100%) with anterior PFV and 8 patients with combined PFV  had central, steady and maintained fixation. The difference was not statistically significant.

Postoperative complications and management

Five patients had visual axis obscuration (VAO) and 4 out of these 5 patients developed VAO within 1 month follow up, for which membranectomy was done. Visual axis obscuration was seen in 1 patient with anterior PFV and 4 patients with combined PFV.

8 patients had pigment deposition over the IOL at six months follow up. At the follow up of 6 months, 3 patients had posterior synechiae, out of which 2 developed posterior synechiae within 2 weeks after surgery. None of our patients developed glaucoma, endophthalmitis or retinal detachment at six months follow up.

Retinoscopy

The mean retinoscopy was 5.58±5.44 Diopters 2 weeks postoperatively. Part time occlusion of the uninvolved eye was started at 2 weeks follow up post-surgery in all the patients. At 1 month follow up, mean retinoscopy decreased to 5.38 Diopters and at 3 months follow up, it decreased to 4.90 Diopters. Mean retinoscopy was 4.52 Diopters at 6 months follow up.

Histopathology

Samples of vascularised mass and stalk were sent for histopathological examination in 6 cases. Histopathological examination showed features consistent with PFV. Haematoxylin and eosin staining was used to evaluate the microscopic sections for histopathological examination of the vascularised membrane and vitreous stalk. All the sections were examined by light microscopy and were recorded by photomicrographs.

Low magnification of the slides showed collection of inflammatory cells predominantly, which were seen as mononuclear cells with precursors of eosinophils in medium and high magnifications. Vascularisation in the form of loose mesenchymal tissue and multiple thin walled blood vessels was seen in medium and high magnifications. Most of the slides had loose fibrovascular connective tissue. Congested vascular channels, which were diffusely infiltrated with erythroid and eosinophilic precursors, were seen and were suggestive of extramedullary hematopoeisis. Enlarged ciliary processes were seen as pigmented cells. Eosinophilic material from lens was also noted in all the slides. This eosinophilic lens fragment was strongly Periodic acid Schiff positive.

Discussion

Persistent fetal vasculature, also known as persistent hyperplastic primary vitreous, is characterized by the presence of portion of primary vitreous i.e. persistent hyaloid vasculature. It can be seen as a retrolental fibrovascular membrane. It is most commonly associated with cataract, glaucoma, microphthalmos and anteriorly displaced, elongated ciliary process.21 Majority of them are unilateral and usually have poor vision in the affected eye.22 A study from United States on blindness in children and visual loss showed that PFV accounts for about 5% of all cases of blindness, although exact prevalence of PFV is not known.23 Various studies have shown their approach of treating PFV and their outcomes; however such studies were largely retrospective in nature. Ours is one of the very few prospective studies on PFV with such number of patients till date.

We studied 17 children who presented to us with various features of PFV. Out of 17, 13 were males and 4 were females. This is not consistent with various studies.21 High incidence in males is most probably related to higher male to female ratio in Northern India. All the patients had unilateral presentation which is consistent with various studies. Few studies21 and case reports22 have shown to have bilateral involvement as well. In our study, one patient had anophthalmic socket which may be due to intrauterine insult to fetus since the other eye too had PFV. Our study had the minimum age of presentation as 2 weeks which has not been reported in literature till date.

We studied the fixation preference using CSM method in all patients. Out of 17 patients, 4 patients had CSM fixation preoperatively. At the end of six months, 12 patients developed CSM fixation postoperatively. This can be attributed to early surgical intervention and proper surgical techniques followed by strict amblyopia therapy in all our cases. This is the first study in PFV patients using CSM method for fixation preference/vision assessment and was employed considering the younger age of patients in our study.

Patients with PFV have shallow anterior chamber and are at risk for developing glaucoma. We analysed the trend of IOP in our study over 6 months follow up and we found significant reduction of IOP at 6 months follow up. The difference in IOP preoperatively and at 6 months was significant. None of the patients developed glaucoma in our study. These results are similar to the results reported by Sinha et al.24 and Li et al.21

Two eyes had microcornea associated with PFV. We did not implant IOL after phacoaspiration in these two cases due to risk of developing glaucoma and were left aphakic. Both of these cases had anterior PFV and their association is not known till date. One of these eyes had presence of microspherophakia. None of our eyes had microphthalmia, as was seen in 2 cases in the study by Sinha et al.24 50% of patients had elongated anteriorly displaced ciliary process which is a pathognomonic sign of PFV. Such high percentage has not been reported in previous studies.24,21

Combined PFV was diagnosed more commonly on USG B-scan while anterior PFV was diagnosed more commonly clinically. Thus, features of combined PFV were missed clinically due to thick vascularised mass or due to the dense nature of cataract. Our study recommends doing USG B-scan in all cases of PFV, whether anterior or posterior.

We found higher incidence of combined PFV as compared to anterior PFV in our study. At 3 months and 6 months follow up, combined PFV had unfavourable visual outcome, higher rates of visual axis obscuration and need for further surgical intervention, as compared to anterior PFV. Our study’s outcomes were in agreement with the outcomes of Pollard19 and Anteby et al25 who found that anterior PFV is associated with good visual outcomes with precise surgical techniques. Fixation preference as assessed by CSM method in our study showed improvement at 3 and 6 months follow up in both types of PFV, although relatively better outcomes in anterior PFV (p> 0.5) were observed. We also started the amblyopia therapy from postoperative week 2, as it also improves the visual acuity in both the groups.

Sinha et al.24 studied only the anterior PFV and their results showed good outcomes in all cases; 2 were left aphakic due to microophthalmia and had poor prognosis as compared to others. No complications were noted in their study. In our study, 2 patients were left aphakic due to presence of microcornea. We also did not find any complications in anterior PFV. Cheng et al.26 studied the outcome of combined PFV and its complications. They had poorer outcomes due to the presence of preoperative retinal detachments and no IOL implantation. However, in our study, we excluded any patients of combined PFV with retinal detachment or macular involvement.

We also studied whether placement of IOL has better outcomes in our study. Phacoaspiration with IOL implantation with primary posterior capsulotomy had better visual outcomes as compared to phacoaspiration with primary posterior capsulotomy without IOL implantation at 6 months follow up. VAO was also more common in the IOL group as compared to no IOL group, although non-significant. Similarly, surgical intervention was more common in IOL group in the form of membranectomy and had improved visual outcomes, accompanied with patching therapy.

None of our patients developed intraoperative bleeding during surgery. Sinha et al.24 in his prospective study reported similar findings. We used endocautery to cauterise the vascularised mass and stalk. We also used endoscopy assisted cauterisation of stalk which has not been reported for management of PFV. None of our patients developed intraocular haemorrhage. None of our patients developed retinal detachment or endophthalmitis during the six months follow up. This is in contrast to the study by Li et al.21 in which one eye developed retinal detachment out of 33 eyes. Since we had cut the stalk during surgery, it released traction on the retina and thus reduced the incidence of subsequent retinal detachment. Thus, meticulous surgical techniques as described in our study can drastically reduce the rates of complications.

Second surgery was done for small pupil in one eye in the form of pupilloplasty at 2 weeks and the patient had good visual outcome. Membranectomy was done for visual axis obscuration twice and pupilloplasty in one patient with recurrent inflammation in the form of PCO and posterior synechiae. One eye had insignificant VAO for which no intervention was done. Thus, patients of PFV develop recurrent inflammation, requiring multiple membranectomies; however, timely intervention gives satisfactory outcomes with clear visual axis. We also observed that motivation of the parents, compliance of the patients, aggressive amblyopia therapy and frequent follow ups over a period of time significantly improve the visual outcomes. Studies have shown role of amblyopia therapy in achieving satisfactory outcomes in patients with PFV.18,19,25

Repeated VAO was the only major complication seen postoperatively. The limitations of this study were small sample size, exclusion of posterior PFV and shorter duration of follow up. Visual acuity assessment may not be accurate in since these children were too young. Future studies with long term follow up and larger number of patients are required.

To conclude, this is the first prospective study including both anterior and combined PFV, where cauterisation of the PFV stalk was done. We have studied largest number of parameters related to various aspects of PFV affecting its outcomes and risk factors. None of our patients had any major complications during this study. We used endocautery to cut the stalk which further reduced our complications. We are the first to use endoscope assisted endocautery in PFV, which had a very good outcome as well. Risk factors identified in our study were the type of PFV, pseudophakia/aphakia and presence of ocular anomalies such as microcornea. Early surgical intervention followed by strict postoperative follow up and amblyopia therapy regime can lead to excellent outcomes, as seen in our study. Thus, we recommend early surgical intervention in cases of PFV, followed by intensive amblyopia therapy. 

References

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