VISUAL OUTCOME IN EARLY VITRECTOMY FOR POSTERIOR PERSISTENT FETAL VASCULATURE ASSOCIATED WITH TRACTION RETINAL DETACHMENT ANGELA BOSJOLIE, DO,* PHILIP FERRONE, MD† Purpose: This study investigates the anatomical and visual outcome of vitrectomy for the treatment of unilateral posterior persistent fetal vasculature with associated traction retinal detachment in very young patients. Methods: A retrospective case series study from 1998 through 2010 of 11 eyes in 11 patients who underwent pars plana vitrectomy with or without lensectomy for unilateral posterior persistent fetal vasculature with traction retinal detachment affecting the macula. Results: Ten of the 11 patients (91%) received surgical intervention when 13 months old or younger (average age, 4 months). Postoperatively, 6 of 10 eyes (60%) had 20/800 or better vision, 2 of which had 20/60 vision. All 10 patients in this group had their retinas reattached postoperatively with significant reversal of retinal dragging. One of the 11 patients (9%) received surgical intervention for posterior persistent fetal vasculature at 33 months of age. This patient had persistent traction retinal detachment and a postoperative visual acuity of hand motion. Postoperative glaucoma was detected in 4 patients (36%). Conclusion: This study suggests that early intervention in patients with unilateral posterior persistent fetal vasculature with associated macula affecting traction retinal detachment may provide a better visual and anatomical outcome when vitrectomy and retinal reattachment are performed at a very early age. RETINA 35:570–576, 2015
P
unilateral.1 When present, PFV can cause a number of anatomical malformations, including microcornea, microphthalmia, anterior shift of the lens–iris diaphragm, glaucoma, in addition to iris, lens, retinal, macular, and optic nerve abnormalities.2 Traditionally, PFV is divided into three categories based on location of the vascular malformations: anterior, posterior, or combined anterior and posterior.3 In posterior PFV, histologic studies show that the retrolental fibrovascular stalk contains mesodermal (vascular) and neuroectodermal (glial) tissue leading to anteroposterior traction between the retina and lens diaphragm.4 This traction can lead to retinal folds, traction retinal detachment (TRD), macular dragging, and retinal dysplasia.2,5 Bilateral PFV may be associated with systemic conditions such as Trisomy 13,6 Trisomy 15,7 Aicardi syndrome,8 Norrie disease,9 and Walker–Warburg syndrome.8,10 Glaucoma, recurrent vitreous hemorrhage, and phthisis are known as secondary complications of PFV.5
ersistent fetal vasculature (PFV) is the failure of the fetal vascular system to spontaneously regress after birth. It was coined as persistent hyperplastic vitreous by Reese1 in 1955 who described this clinical entity as the postnatal persistence of retrolental blood vessels causing fibrovascular sheath formation. In 1997, Goldberg labeled this term a misnomer, recognizing that fetal vasculature may persist not only in the posterior pole but also in the anterior segment. He renamed this congenital vascular malformation as PFV to incorporate to the full extent of the disease.2 Persistent fetal vasculature is thought to arise from a spontaneous congenital malformation and is usually From the *National Retina Institute, Towson, Maryland; and †Long Island Vitreoretinal Consultants, Great Neck, New York. None of the authors have any financial/conflicting interests to disclose. Reprint requests: Angela Bosjolie, DO, National Retina Institute, 901 Dulaney Valley Road, Suite 200, Towson, MD 21204; e-mail: [email protected]
570
EARLY VITRECTOMY PFV BOSJOLIE AND FERRONE
A clinical spectrum of disease severity and visual potential exists for PFV. Eyes with severe retinal dysplasia historically have little or no visual potential and were considered poor surgical candidates with a very poor visual prognosis.2,11 Eyes that are minimally affected are more common and usually have functional vision with no secondary complications. The natural disease course of severe forms of PFV is progression of fibrovascular dyplasia leading to angle closure glaucoma, corneal clouding, cataract, intraocular hemorrhage, retinal detachment, and phthisis bulbi.1,2 Of patients with severe PFV who did not receive surgical intervention, 37% to 46% eventually progressed to no light perception vision.12–14 Indications for surgical intervention include recurrent or severe intravitreal hemorrhage, retinal detachment, progressive shallowing of the anterior chamber, and uncontrolled intraocular pressure secondary to anterior chamber angle closure.2 Vitrectomy with or without lensectomy is the procedure of choice to treat posterior PFV. We present the anatomical and visual results of a retrospective case series of 11 eyes with posterior PFV and associated TRD affecting the macula that underwent surgical repair. From our case series, we suggest that earlier surgical intervention gives these patients increased opportunity for functional vision and improved anatomical outcomes.
Methods The North Shore Long Island Jewish Health System Institutional Review Board (Manhasset, NY) approved this retrospective case study series. We reviewed charts from our referral-based vitreoretinal practice of all patients diagnosed with primarily posterior PFV with associated TRD affecting the macula who underwent surgical intervention from 1998 to 2010. Our investigation revealed 11 patients fitting the inclusion criteria of unilateral primarily posterior PFV with associated TRD affecting the macula who underwent 20-gauge or 23-gauge pars plana vitrectomy (PPV), 2-port, 20-gauge irrigating end light pipe, with or without lensectomy by the same retinal surgeon. The two-port vitrectomies were performed with the irrigating end light pipe in one sclerotomy and with high-speed vitreous cutter in the other. Sclerotomies were made through the pars plana in all patients, and varied from 1.0 mm to 2.0 mm posterior to the limbus depending on the age of the patient at surgery and the extent of microphthalmia affecting the operative eye. The stalk was divided with the high-speed mechanical vitrector anteriorly taking care not to damage the anteriorly dragged retina in the stalk and the posterior
571
lens capsule. After the stalk was divided, no diathermy was applied to the fibrovascular stalk because bleeding was not an issue. Also, fairly complete vitrectomy was performed around the residual stalk to remove the sheets of the connected vitreous and residual hyalocytes around the posterior aspect of the residual stalk. Age at surgical intervention, length of follow-up, preoperative and postoperative vision and anatomical status, postoperative complications, and postoperative treatment were recorded.
Results Our chart review showed 11 children with the diagnosis of PFV with an associated macula affecting TRD who underwent surgical intervention. Ten of these patients (91%) were 13 months old or younger, 9 of whom were younger than 6 months at the time of surgical intervention. One patient (Patient 11, Table 1) underwent surgery at 33 months of age. All patients underwent lens-sparing vitrectomy (LSV) if the central lens was clear. If a visually significant cataract was present, the patient underwent pars plana lensectomy without intraocular lens placement in addition to PPV. Postoperative aphakic correction and amblyopia therapy were performed on those patients who underwent pars plana lensectomy. The average age at surgery for the entire patient population was 7 months (range, 1–33 months). The average follow-up time for the total patient population was 36 months (range, 6–75 months). Ten patients, 13 months old and younger, received surgical intervention for TRD secondary to fibrovascular hyperplasia of primarily posterior PFV (Patients 1–10, Table 1). All 10 patients were noted to have a peripapillary TRD involving the macula and a fibrovascular stalk on preoperative examination. Five of these patients were also noted to have retinal folds (Patients 1, 2, 5, 6, and 7). All of the 10 patients were noted to have abnormal macula architecture due to retinal dragging and detachment. All had preoperative lenticular opacities because of the anterior stalk attachment to the posterior lens capsule. Two patients (Patients 6 and 7) had detectable elements of anterior PFV. Patient 7 had a vitreous hemorrhage from severe traction. All of the 10 patients receiving surgery in the first 13 months of life had their retinas reattached postoperatively with significant reversal of retinal dragging with all attaining macula reattachment. Three of these patients had a small, residual peripapillary retinal fibrotic fold. The average age at surgical intervention was 4 months (range, 1–13 months). The average postoperative followup time was 32 months (range, 6–72 months). The
M/F
OD/OS
Initial VA
1
M
OS
LP
2
M
OS
LP
3
F
OS
LP
4
F
OS
LP
5
M
OD
LP
6
M
OD
LP
7
M
OS
LP
8
F
OD
LP
9
F
OS
LP
10
M
OD
11
M
OD
Patient 1
Initial Fundus Examination
Other Examination Findings
Age at Surgery, months
TRD with retinal folds, no foveal ,3 mm posterior capsular reflex, distorted macular anatomy opacity, out of visual axis TRD involving peripapillary area and 2 mm posterior capsular opacity, infranasal to fovea, fovea drawn up into vertical visual axis fold, distorted vessels in fold, no foveal reflex TRD with stalk None TRD with stalk, mild peripheral retinal detachment TRD with inferior retinal folds, macula involved, no foveal reflex TRD with stalk peripapillary area, inferior retinal fold, high RD of macula TRD, stalk, temporal folding and dragging macula area, no normal macula architecture with RPE changes, VH TRD involving macula, peripapillary stalk TRD, peripapillary stalk
Central, steady, TRD, preretinal fibrosis, subretinal unmaintained hemorrhage in peripapillary area, no foveal reflex LP
TRD peripapillary area, no foveal reflex, stalk
Subsequent Surgery
Length of Follow-up, months
Age at Last Follow-up, months
None
30
32
Vision at Last Follow-up Central, steady maintained
2
2 1
Mild inferior posterior capsular opacity 3 mm central posterior polar cataract, enlarged cornea and globe Polycoria, trace NS
5.5
3 mm posterior polar cataract None
Slight microophthalmia, 15 PD ET, 1 mm posterior capsular opacity, out of visual axis ,3 mm central after polar cataract, mild ET, mild microophthalmia
LSV, 20 G, end light LSV, 23 G, end light
4
3+ PSC
Type of Surgery 2 port, irrigating pipe 2 port, irrigating pipe
LSV, 20 G, 2 port, irrigating end light pipe PPL, PPV, 20 G, 2 port, irrigating end light pipe LSV, 20 G, 2 port, irrigating end light pipe LSV, 20 G, 2 port, irrigating end light pipe
3 4
PPL, PPV, 20 G, 2 port, irrigating end light pipe
2
PPL, PPV; 20 G, 2 port, irrigating end light pipe LSV, 20 G high-speed mechanical core vitrectomy with 2-port 20 G irrigating and light pipe LSV, 20 G, 2 port, irrigating end light pipe
3.25
13
33.5
LSV, 20 G, 2 port irrigating end light pipe
Last Fundus Examination
Complications
Resolution of TRD, residual preretinal gliosis, no well-defined foveal reflex, macular anatomy otherwise normal
None
Other Comments 60 PD ET, amblyopia therapy
RETINA, THE JOURNAL OF RETINAL AND VITREOUS DISEASES 2015 VOLUME 35 NUMBER 3
Patient
572
Table 1. Study Group Characteristics
Table 1.
(Continued )
Subsequent Surgery
Length of Follow-up, months
Age at Last Follow-up, months
2
None
25
28
3
None
72
74
Teller VA of 4.8 cycles/cm at 38 cm; conversion to Snellen 20/180 20/800
4
None
35
36
2/400
Resolution of TRD, mild optic nerve pallor, inferior preretinal gliosis Resolution of TRD
5
None
36
41
20/60
Resolution of TRD
At 4 months: lensectomy for Elschnig’s pearls. At 12 months: Baerveldt tube None
29
31
Central, steady, unmaintained
Resolution of TRD
Aphakic glaucoma
6
10
LP
Resolution of TRD, few retinal folds
—
8 months: glaucoma tube shunt CE/IOL
30
32
20/200
Resolution of TRD, nasal fold
Aphakic glaucoma, IOP controlled on two glaucoma medications Aphakic glaucoma
36
39
20/60
Resolution of TRD
—
20
33
20/150
Resolution of TRD, atrophic macular changes
75
108
Postoperative cataract Persistent ET, continued amblyopia therapy —
Patient
7
8 9 10 11
16 months: strabismus surgery
Hand motion
Last Fundus Examination Resolution of TRD, improved retinal folds, present foveal reflex, mild peripapillary pigment changes
Improved but significant and persistent TRD, peripapilary fibrosis, good laser
Complications Elevated IOP, controlled on two glaucoma medications
Other Comments —
None
—
—
60 PD ET, aphakic correction, amblyopia therapy 30 PD ET, amblyopia therapy Noncompliant with amblyopia therapy
—
—
—
EARLY VITRECTOMY PFV BOSJOLIE AND FERRONE
6
Vision at Last Follow-up
—
CE, cataract extraction; ET, esotropia; F, female; G, gauge; IOL, intraocular lens implant; IOP, intraocular pressure; LP, light perception; M, male; OD, right eye; OS, left eye; PD, prism diopters; PPL, pars plana lensectomy; PSC, posterior subcapsular cataract; RPE, retinal pigment epithelium; VA, visual acuity; VH, vitreous hemorrhage.
573
574
RETINA, THE JOURNAL OF RETINAL AND VITREOUS DISEASES 2015 VOLUME 35 NUMBER 3
average age at the last follow-up was 40 months (range, 10–74 months; median, 40 months). Seven eyes underwent LSV, whereas three eyes underwent PPV with lensectomy for a preoperative visually significant cataract. Six of the 10 eyes (60%) had functional visual acuity (20/800 or better). Two of these 6 eyes had vision that measured 20/60 (Patients 5 and 9). Of the remaining 4 eyes, 1 eye had central, steady maintained vision at 32 months of age; 1 eye had Teller visual acuity of 4.8 cycles per centimeter at 38 cm (approximate Snellen conversion, 20/180; Patient 2, Figure 1, A and B)15; 1 eye had central vision only at 31 months of age; and the remaining 1 eye had light perception vision at 10 months of age (Patient 7). Patient 7 had light perception only vision at the last visit at 10 months of age. This patient presented with a severe form of posterior PFV with a vitreous hemorrhage. Although at the last examination, the retina was noted to be attached with a decreased retinal fold. In addition, this patient’s young age of 10 months posed a communication barrier in postoperative visual
acuity assessment. As this patient ages and gains the ability to participate in subjective vision assessments, the measured visual acuity may improve. One patient in our case series (Patient 11, Table 1) received surgical intervention for posterior PFV at 33 months of age. The postoperative follow-up time for this patient was 75 months. The age at the last followup was 108 months. This patient underwent LSV. Visual acuity at the last examination was hand motion with an improved but significant and persistent TRD with peripapillary fibrosis after surgical intervention. This patient had the most limited reversal of retinal dragging, which we attribute to the later date of intervention for this patient. Postoperative glaucoma was detected in 4 of the 11 patients (36%), 3 of whom were aphakic. Of the four patients with postoperative glaucoma, two (one phakic and the other aphakic) required topical drops for intraocular pressure control. The other two cases required glaucoma tube surgery for adequate intraocular pressure control. Progression of cataract was seen in 1 of the 8 phakic patients (12%). This patient subsequently had cataract extraction with posterior chamber intraocular lens implant at a later date with the last noted visual acuity of 20/60. Of note, all patients who had pars plana lensectomy were referred for appropriate amblyopia therapy. Patient 6 was noted to be noncompliant with amblyopia therapy and remained at a visual acuity of central, unsteady unmaintained vision at 31 months of age.
Discussion
Fig. 1. A. Patient 2 preoperative. B. Patient 2 postoperative LSV, 23 gauge (2 ports).
Early literature indicated that vitrectomy for the treatment of posterior PFV resulted in a poor visual outcome.2,4,16 In some cases, the posterior forms of PFV were considered as exclusion criteria for surgical treatment, especially if associated with TRD or macular dysplasia. More recently, PPV with or without lensectomy has been used in an attempt to treat posterior PFV, improve visual function, and prevent secondary complications of glaucoma, vitreous hemorrhage, and phthisis. Dass and Trese17 reported that 6 of 27 eyes with combined or strictly posterior PFV achieved Snellen visual acuity of 20/60 to 20/800 after PPV. The authors suggested this improved visual potential was attributable to recent advancements in surgical techniques. Interestingly, on review of their data, 4 of the 6 eyes with functional Snellen acuity had unilateral involvement with noted retinal dysplasia, and 3 of
EARLY VITRECTOMY PFV BOSJOLIE AND FERRONE
these 4 eyes received surgical intervention before 6 months of age. Similarly, a study performed by Hunt et al13 on 33 eyes that underwent surgical intervention for PFV concluded that those patients who received surgery before 77 days of age were 13 times more likely to have visual acuity of counting fingers or better than those operated on at an older age. Although Hunt et al did not comment if TRD was present, the study does support early surgical intervention can result in improved visual function. In a study by Walsh et al,18 it was noted that only 3 of 13 patients with bilateral combined PFV who underwent surgical intervention had good functional vision. Review of their data shows that these 3 patients received surgical treatment at 8 months of age or younger. The other 10 patients in this study who received surgical intervention at a later age had no light perception to hand motion vision. Our interpretation of this data supports our notion that earlier intervention, even in the cases of bilateral PFV, potentially yields a better visual prognosis. On histologic review of eyes with posterior PFV, Manschot4 noted that the retrolental fiborvascular stalks contained glial cells. The glial and vascular components of the stalk cause an anteroposterior traction on the peripapillary retina leading to retinal folds, macular dragging, and TRD. If left to further mature in the eye, these stalks can cause irreversible retinal detachment with dysplasia and reduced vision. Our data suggest that a period of retinal “physical plasticity” extends to at least 13 months of age. All 10 patients who received surgical intervention at 13 months of age or younger had reattachment of the retina with reversal of retinal dragging and decreased retinal folds. Of note, 9 of the 10 patients were younger than 6 months of age at the time of surgical intervention. Six of these 10 patients (60%) had functional vision of 20/800 or better, a favorable visual result. This functional visual result may also relate to the development of vision with early intervention allowing for early treatment of amblyopia. From the review of previous studies in combination with our data, we theorize that early PPV surgical intervention may provide a better visual prognosis for the macula affecting posterior PFV even with concurrent retinal dysplasia. We advocate for referral and consideration of early vitrectomy in children with TRD and posterior PFV. We base this on the theory that the retina has a period of “physical plasticity,” as in retinopathy of prematurity, where the retina can revert to a more normal anatomical position and functional role if early intervention is initiated. The one patient in our study who had a persistent TRD received surgical intervention at 33 months of
575
age. This later intervention may contribute to the limited retinal reattachment and poor postoperative vision. Preoperatively, this patient had more fibrotic retinal changes that we believe limited the postoperative reversal of retinal dragging. Of course, this single patient does not serve as an adequate control group for this retrospective study. However, it does support our observation that earlier intervention makes for better visual and anatomical outcomes in an already visually devastating condition. Postoperative complications included the development of glaucoma. Thirty-six percent (four patients) developed glaucoma and required treatment (two controlled with topical antiglaucoma drops and two required glaucoma tube surgery). This rate is similar to that reported by other studies of patients with similar anatomical abnormalities undergoing similar surgical procedures. Vasavada et al19 found the rate of aphakic glaucoma in microphthalmic eyes after cataract surgery to be 30.9%. Johnson and Keech20 reported a rate of aphakic glaucoma after cataract surgery in patients with PFV as 32%. Glaucoma was seen in 33% of individuals receiving LSV for retinopathy of prematurity in a study by Choi et al.21 Additionally, it is important to note that the risk of glaucoma in patients with PFV is 23% to 32% with surgical intervention,13,20 and also in many patients with PFV without surgical intervention.3,5 Limitations to our study include its retrospective design, small patient population, lack of control group, variability in follow-up, and compliance with amblyopia therapy. Although PFV is a common congenital malformation, it usually presents in very mild forms, such as a persistent pupillary membrane or a Mittendorf dot, which typically do not affect vision or require surgical intervention. It is very rare to see severe forms of PFV, especially cases with associated TRD. Given this, conducting a prospective study with a large patient population is not practical. In addition, visual acuity assessment of the infant and pediatric populations offers unique challenges. Children have limited verbal communication, and often visual acuity is based on objective measurements. In conclusion, our data support consideration of early surgical intervention for patients with posterior PFV with the macula affecting TRD. Our study suggests that surgical intervention at younger than or equal to 13 months of age offers improved visual potential. Complications observed in this study include the development of glaucoma, the rate of which was similar to that noted for lensectomy for microphthalmia, and LSV for retinopathy of prematurity. Only 2 of the 11 patients included in this study required a surgical glaucoma procedure to achieve adequate postoperative intraocular pressure control.
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RETINA, THE JOURNAL OF RETINAL AND VITREOUS DISEASES 2015 VOLUME 35 NUMBER 3
Key words: persistent fetal vasculature, traction retinal detachment, pars plana vitrectomy, cataract, glaucoma, persistent hyperplastic vitreous. References 1. Reese A. Persistent hyperplastic primary vitreous. Am J Ophthalmol 1955;40:317–331. 2. Goldberg M. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV) LIV Edward Jackson Memorial Lecture. Am J Ophthalmol 1997;124:587–626. 3. Pollard ZF. Persistent hyperplastic primary vitreous: diagnosis, treatment, and results. Trans Am Ophthalmol Soc 1997;95: 487–549. 4. Manschot WA. Persistent hyperplastic primary vitreous; special reference to preretinal glial tissues as pathological characteristic and to the development of the primary vitreous. AMA Arch Ophthalmol 1958;59:188–203. 5. Reese A. Persistent hyperplastic primary vitreous. Trans Am Acad Ophthalmol Otolaryngol 1955;59:271–295. 6. Haddad R, Font RL, Reeser F. Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature. Surv Ophthalmol 1978;23:123–134. 7. Kremer I, Nissenkorn I, Ben-Sira I, Steinherz R. Unusual ophthalmological findings in a case of partial trisomy 15. Metab Pediatr Syst Ophthalmol 1986;9:597–598. 8. Marshman WE, Jan JE, Lyons CJ. Neurologic abnormalities associated with persistent hyperplastic primary vitreous. Can J Ophthalmol 1999;34:17–22. 9. Shastry BS. Persistent hyperplastic primary vitreous: congenital malformation of the eye. Clin Experiment Ophthalmol 2009;37:884–890.
10. Gerding H, Gullotta F, Kuchelmeister K, Busse H. Ocular findings in Walker-Warburg syndrome. Childs Nerv Syst 1993;9:418–420. 11. Soheilian M, Vistamehr S, Rahmani B, et al. Outcomes of surgical and nonsurgical management of persistent fetal vasculature. Ann Ophthalmol 2003;35:28–37. 12. Anteby I, Cohen E, Karshai I, BenEzra D. Unilateral persistent hyperplastic primary vitreous: course and outcome. J AAPOS 2002;6:92–99. 13. Hunt A, Rowe N, Lam A, Martin F. Outcomes in persistent hyperplastic primary vitreous. Br J Ophthalmol 2005;89:859–863. 14. Sisk RA, Berrocal AM, Feuer WJ, Murray TG. Visual and anatomic outcomes with or without surgery in persistent fetal vasculature. Ophthalmology 2010;117:2178–2183.e1–e2. 15. Teller DY, Dobson V, Mayer DL. Teller Acuity Cards II (TAC II), Reference, Instruction Manual. Chicago, IL: Stereo Optical Co, Inc; 2005. 16. Ceron O, Lou PL, Kroll AJ, Walton DS. The vitreoretinal manifestations of persistent hyperplastic primary vitreous (PHPV) and their management. Int Ophthalmol Clin 2008;48:53–62. 17. Dass AB, Trese MT. Surgical results of persistent hyperplastic primary vitreous. Ophthalmology 1999;106:280–284. 18. Walsh MK, Drenser KA, Capone A, Trese MT. Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vasculature syndrome. Retina 2010;30:S2–S8. 19. Vasavada VA, Dixit NV, Ravat FA, et al. Intraoperative performance and postoperative outcomes of cataract surgery in infant eyes with microphthalmos. J Cataract Refract Surg 2009;35:519–528. 20. Johnson CP, Keech RV. Prevalence of glaucoma after surgery for PHPV and infantile cataracts. J Pediatr Ophthalmol Strabismus 1996;33:14–17. 21. Choi J, Kim JH, Kim SJ, Yu YS. Long-term results of lenssparing vitrectomy for stages 4B and 5 retinopathy of prematurity. Korean J Ophthalmol 2011;25:305–310.
P
unilateral.1 When present, PFV can cause a number of anatomical malformations, including microcornea, microphthalmia, anterior shift of the lens–iris diaphragm, glaucoma, in addition to iris, lens, retinal, macular, and optic nerve abnormalities.2 Traditionally, PFV is divided into three categories based on location of the vascular malformations: anterior, posterior, or combined anterior and posterior.3 In posterior PFV, histologic studies show that the retrolental fibrovascular stalk contains mesodermal (vascular) and neuroectodermal (glial) tissue leading to anteroposterior traction between the retina and lens diaphragm.4 This traction can lead to retinal folds, traction retinal detachment (TRD), macular dragging, and retinal dysplasia.2,5 Bilateral PFV may be associated with systemic conditions such as Trisomy 13,6 Trisomy 15,7 Aicardi syndrome,8 Norrie disease,9 and Walker–Warburg syndrome.8,10 Glaucoma, recurrent vitreous hemorrhage, and phthisis are known as secondary complications of PFV.5
ersistent fetal vasculature (PFV) is the failure of the fetal vascular system to spontaneously regress after birth. It was coined as persistent hyperplastic vitreous by Reese1 in 1955 who described this clinical entity as the postnatal persistence of retrolental blood vessels causing fibrovascular sheath formation. In 1997, Goldberg labeled this term a misnomer, recognizing that fetal vasculature may persist not only in the posterior pole but also in the anterior segment. He renamed this congenital vascular malformation as PFV to incorporate to the full extent of the disease.2 Persistent fetal vasculature is thought to arise from a spontaneous congenital malformation and is usually From the *National Retina Institute, Towson, Maryland; and †Long Island Vitreoretinal Consultants, Great Neck, New York. None of the authors have any financial/conflicting interests to disclose. Reprint requests: Angela Bosjolie, DO, National Retina Institute, 901 Dulaney Valley Road, Suite 200, Towson, MD 21204; e-mail: [email protected]
570
EARLY VITRECTOMY PFV BOSJOLIE AND FERRONE
A clinical spectrum of disease severity and visual potential exists for PFV. Eyes with severe retinal dysplasia historically have little or no visual potential and were considered poor surgical candidates with a very poor visual prognosis.2,11 Eyes that are minimally affected are more common and usually have functional vision with no secondary complications. The natural disease course of severe forms of PFV is progression of fibrovascular dyplasia leading to angle closure glaucoma, corneal clouding, cataract, intraocular hemorrhage, retinal detachment, and phthisis bulbi.1,2 Of patients with severe PFV who did not receive surgical intervention, 37% to 46% eventually progressed to no light perception vision.12–14 Indications for surgical intervention include recurrent or severe intravitreal hemorrhage, retinal detachment, progressive shallowing of the anterior chamber, and uncontrolled intraocular pressure secondary to anterior chamber angle closure.2 Vitrectomy with or without lensectomy is the procedure of choice to treat posterior PFV. We present the anatomical and visual results of a retrospective case series of 11 eyes with posterior PFV and associated TRD affecting the macula that underwent surgical repair. From our case series, we suggest that earlier surgical intervention gives these patients increased opportunity for functional vision and improved anatomical outcomes.
Methods The North Shore Long Island Jewish Health System Institutional Review Board (Manhasset, NY) approved this retrospective case study series. We reviewed charts from our referral-based vitreoretinal practice of all patients diagnosed with primarily posterior PFV with associated TRD affecting the macula who underwent surgical intervention from 1998 to 2010. Our investigation revealed 11 patients fitting the inclusion criteria of unilateral primarily posterior PFV with associated TRD affecting the macula who underwent 20-gauge or 23-gauge pars plana vitrectomy (PPV), 2-port, 20-gauge irrigating end light pipe, with or without lensectomy by the same retinal surgeon. The two-port vitrectomies were performed with the irrigating end light pipe in one sclerotomy and with high-speed vitreous cutter in the other. Sclerotomies were made through the pars plana in all patients, and varied from 1.0 mm to 2.0 mm posterior to the limbus depending on the age of the patient at surgery and the extent of microphthalmia affecting the operative eye. The stalk was divided with the high-speed mechanical vitrector anteriorly taking care not to damage the anteriorly dragged retina in the stalk and the posterior
571
lens capsule. After the stalk was divided, no diathermy was applied to the fibrovascular stalk because bleeding was not an issue. Also, fairly complete vitrectomy was performed around the residual stalk to remove the sheets of the connected vitreous and residual hyalocytes around the posterior aspect of the residual stalk. Age at surgical intervention, length of follow-up, preoperative and postoperative vision and anatomical status, postoperative complications, and postoperative treatment were recorded.
Results Our chart review showed 11 children with the diagnosis of PFV with an associated macula affecting TRD who underwent surgical intervention. Ten of these patients (91%) were 13 months old or younger, 9 of whom were younger than 6 months at the time of surgical intervention. One patient (Patient 11, Table 1) underwent surgery at 33 months of age. All patients underwent lens-sparing vitrectomy (LSV) if the central lens was clear. If a visually significant cataract was present, the patient underwent pars plana lensectomy without intraocular lens placement in addition to PPV. Postoperative aphakic correction and amblyopia therapy were performed on those patients who underwent pars plana lensectomy. The average age at surgery for the entire patient population was 7 months (range, 1–33 months). The average follow-up time for the total patient population was 36 months (range, 6–75 months). Ten patients, 13 months old and younger, received surgical intervention for TRD secondary to fibrovascular hyperplasia of primarily posterior PFV (Patients 1–10, Table 1). All 10 patients were noted to have a peripapillary TRD involving the macula and a fibrovascular stalk on preoperative examination. Five of these patients were also noted to have retinal folds (Patients 1, 2, 5, 6, and 7). All of the 10 patients were noted to have abnormal macula architecture due to retinal dragging and detachment. All had preoperative lenticular opacities because of the anterior stalk attachment to the posterior lens capsule. Two patients (Patients 6 and 7) had detectable elements of anterior PFV. Patient 7 had a vitreous hemorrhage from severe traction. All of the 10 patients receiving surgery in the first 13 months of life had their retinas reattached postoperatively with significant reversal of retinal dragging with all attaining macula reattachment. Three of these patients had a small, residual peripapillary retinal fibrotic fold. The average age at surgical intervention was 4 months (range, 1–13 months). The average postoperative followup time was 32 months (range, 6–72 months). The
M/F
OD/OS
Initial VA
1
M
OS
LP
2
M
OS
LP
3
F
OS
LP
4
F
OS
LP
5
M
OD
LP
6
M
OD
LP
7
M
OS
LP
8
F
OD
LP
9
F
OS
LP
10
M
OD
11
M
OD
Patient 1
Initial Fundus Examination
Other Examination Findings
Age at Surgery, months
TRD with retinal folds, no foveal ,3 mm posterior capsular reflex, distorted macular anatomy opacity, out of visual axis TRD involving peripapillary area and 2 mm posterior capsular opacity, infranasal to fovea, fovea drawn up into vertical visual axis fold, distorted vessels in fold, no foveal reflex TRD with stalk None TRD with stalk, mild peripheral retinal detachment TRD with inferior retinal folds, macula involved, no foveal reflex TRD with stalk peripapillary area, inferior retinal fold, high RD of macula TRD, stalk, temporal folding and dragging macula area, no normal macula architecture with RPE changes, VH TRD involving macula, peripapillary stalk TRD, peripapillary stalk
Central, steady, TRD, preretinal fibrosis, subretinal unmaintained hemorrhage in peripapillary area, no foveal reflex LP
TRD peripapillary area, no foveal reflex, stalk
Subsequent Surgery
Length of Follow-up, months
Age at Last Follow-up, months
None
30
32
Vision at Last Follow-up Central, steady maintained
2
2 1
Mild inferior posterior capsular opacity 3 mm central posterior polar cataract, enlarged cornea and globe Polycoria, trace NS
5.5
3 mm posterior polar cataract None
Slight microophthalmia, 15 PD ET, 1 mm posterior capsular opacity, out of visual axis ,3 mm central after polar cataract, mild ET, mild microophthalmia
LSV, 20 G, end light LSV, 23 G, end light
4
3+ PSC
Type of Surgery 2 port, irrigating pipe 2 port, irrigating pipe
LSV, 20 G, 2 port, irrigating end light pipe PPL, PPV, 20 G, 2 port, irrigating end light pipe LSV, 20 G, 2 port, irrigating end light pipe LSV, 20 G, 2 port, irrigating end light pipe
3 4
PPL, PPV, 20 G, 2 port, irrigating end light pipe
2
PPL, PPV; 20 G, 2 port, irrigating end light pipe LSV, 20 G high-speed mechanical core vitrectomy with 2-port 20 G irrigating and light pipe LSV, 20 G, 2 port, irrigating end light pipe
3.25
13
33.5
LSV, 20 G, 2 port irrigating end light pipe
Last Fundus Examination
Complications
Resolution of TRD, residual preretinal gliosis, no well-defined foveal reflex, macular anatomy otherwise normal
None
Other Comments 60 PD ET, amblyopia therapy
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Patient
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Table 1. Study Group Characteristics
Table 1.
(Continued )
Subsequent Surgery
Length of Follow-up, months
Age at Last Follow-up, months
2
None
25
28
3
None
72
74
Teller VA of 4.8 cycles/cm at 38 cm; conversion to Snellen 20/180 20/800
4
None
35
36
2/400
Resolution of TRD, mild optic nerve pallor, inferior preretinal gliosis Resolution of TRD
5
None
36
41
20/60
Resolution of TRD
At 4 months: lensectomy for Elschnig’s pearls. At 12 months: Baerveldt tube None
29
31
Central, steady, unmaintained
Resolution of TRD
Aphakic glaucoma
6
10
LP
Resolution of TRD, few retinal folds
—
8 months: glaucoma tube shunt CE/IOL
30
32
20/200
Resolution of TRD, nasal fold
Aphakic glaucoma, IOP controlled on two glaucoma medications Aphakic glaucoma
36
39
20/60
Resolution of TRD
—
20
33
20/150
Resolution of TRD, atrophic macular changes
75
108
Postoperative cataract Persistent ET, continued amblyopia therapy —
Patient
7
8 9 10 11
16 months: strabismus surgery
Hand motion
Last Fundus Examination Resolution of TRD, improved retinal folds, present foveal reflex, mild peripapillary pigment changes
Improved but significant and persistent TRD, peripapilary fibrosis, good laser
Complications Elevated IOP, controlled on two glaucoma medications
Other Comments —
None
—
—
60 PD ET, aphakic correction, amblyopia therapy 30 PD ET, amblyopia therapy Noncompliant with amblyopia therapy
—
—
—
EARLY VITRECTOMY PFV BOSJOLIE AND FERRONE
6
Vision at Last Follow-up
—
CE, cataract extraction; ET, esotropia; F, female; G, gauge; IOL, intraocular lens implant; IOP, intraocular pressure; LP, light perception; M, male; OD, right eye; OS, left eye; PD, prism diopters; PPL, pars plana lensectomy; PSC, posterior subcapsular cataract; RPE, retinal pigment epithelium; VA, visual acuity; VH, vitreous hemorrhage.
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average age at the last follow-up was 40 months (range, 10–74 months; median, 40 months). Seven eyes underwent LSV, whereas three eyes underwent PPV with lensectomy for a preoperative visually significant cataract. Six of the 10 eyes (60%) had functional visual acuity (20/800 or better). Two of these 6 eyes had vision that measured 20/60 (Patients 5 and 9). Of the remaining 4 eyes, 1 eye had central, steady maintained vision at 32 months of age; 1 eye had Teller visual acuity of 4.8 cycles per centimeter at 38 cm (approximate Snellen conversion, 20/180; Patient 2, Figure 1, A and B)15; 1 eye had central vision only at 31 months of age; and the remaining 1 eye had light perception vision at 10 months of age (Patient 7). Patient 7 had light perception only vision at the last visit at 10 months of age. This patient presented with a severe form of posterior PFV with a vitreous hemorrhage. Although at the last examination, the retina was noted to be attached with a decreased retinal fold. In addition, this patient’s young age of 10 months posed a communication barrier in postoperative visual
acuity assessment. As this patient ages and gains the ability to participate in subjective vision assessments, the measured visual acuity may improve. One patient in our case series (Patient 11, Table 1) received surgical intervention for posterior PFV at 33 months of age. The postoperative follow-up time for this patient was 75 months. The age at the last followup was 108 months. This patient underwent LSV. Visual acuity at the last examination was hand motion with an improved but significant and persistent TRD with peripapillary fibrosis after surgical intervention. This patient had the most limited reversal of retinal dragging, which we attribute to the later date of intervention for this patient. Postoperative glaucoma was detected in 4 of the 11 patients (36%), 3 of whom were aphakic. Of the four patients with postoperative glaucoma, two (one phakic and the other aphakic) required topical drops for intraocular pressure control. The other two cases required glaucoma tube surgery for adequate intraocular pressure control. Progression of cataract was seen in 1 of the 8 phakic patients (12%). This patient subsequently had cataract extraction with posterior chamber intraocular lens implant at a later date with the last noted visual acuity of 20/60. Of note, all patients who had pars plana lensectomy were referred for appropriate amblyopia therapy. Patient 6 was noted to be noncompliant with amblyopia therapy and remained at a visual acuity of central, unsteady unmaintained vision at 31 months of age.
Discussion
Fig. 1. A. Patient 2 preoperative. B. Patient 2 postoperative LSV, 23 gauge (2 ports).
Early literature indicated that vitrectomy for the treatment of posterior PFV resulted in a poor visual outcome.2,4,16 In some cases, the posterior forms of PFV were considered as exclusion criteria for surgical treatment, especially if associated with TRD or macular dysplasia. More recently, PPV with or without lensectomy has been used in an attempt to treat posterior PFV, improve visual function, and prevent secondary complications of glaucoma, vitreous hemorrhage, and phthisis. Dass and Trese17 reported that 6 of 27 eyes with combined or strictly posterior PFV achieved Snellen visual acuity of 20/60 to 20/800 after PPV. The authors suggested this improved visual potential was attributable to recent advancements in surgical techniques. Interestingly, on review of their data, 4 of the 6 eyes with functional Snellen acuity had unilateral involvement with noted retinal dysplasia, and 3 of
EARLY VITRECTOMY PFV BOSJOLIE AND FERRONE
these 4 eyes received surgical intervention before 6 months of age. Similarly, a study performed by Hunt et al13 on 33 eyes that underwent surgical intervention for PFV concluded that those patients who received surgery before 77 days of age were 13 times more likely to have visual acuity of counting fingers or better than those operated on at an older age. Although Hunt et al did not comment if TRD was present, the study does support early surgical intervention can result in improved visual function. In a study by Walsh et al,18 it was noted that only 3 of 13 patients with bilateral combined PFV who underwent surgical intervention had good functional vision. Review of their data shows that these 3 patients received surgical treatment at 8 months of age or younger. The other 10 patients in this study who received surgical intervention at a later age had no light perception to hand motion vision. Our interpretation of this data supports our notion that earlier intervention, even in the cases of bilateral PFV, potentially yields a better visual prognosis. On histologic review of eyes with posterior PFV, Manschot4 noted that the retrolental fiborvascular stalks contained glial cells. The glial and vascular components of the stalk cause an anteroposterior traction on the peripapillary retina leading to retinal folds, macular dragging, and TRD. If left to further mature in the eye, these stalks can cause irreversible retinal detachment with dysplasia and reduced vision. Our data suggest that a period of retinal “physical plasticity” extends to at least 13 months of age. All 10 patients who received surgical intervention at 13 months of age or younger had reattachment of the retina with reversal of retinal dragging and decreased retinal folds. Of note, 9 of the 10 patients were younger than 6 months of age at the time of surgical intervention. Six of these 10 patients (60%) had functional vision of 20/800 or better, a favorable visual result. This functional visual result may also relate to the development of vision with early intervention allowing for early treatment of amblyopia. From the review of previous studies in combination with our data, we theorize that early PPV surgical intervention may provide a better visual prognosis for the macula affecting posterior PFV even with concurrent retinal dysplasia. We advocate for referral and consideration of early vitrectomy in children with TRD and posterior PFV. We base this on the theory that the retina has a period of “physical plasticity,” as in retinopathy of prematurity, where the retina can revert to a more normal anatomical position and functional role if early intervention is initiated. The one patient in our study who had a persistent TRD received surgical intervention at 33 months of
575
age. This later intervention may contribute to the limited retinal reattachment and poor postoperative vision. Preoperatively, this patient had more fibrotic retinal changes that we believe limited the postoperative reversal of retinal dragging. Of course, this single patient does not serve as an adequate control group for this retrospective study. However, it does support our observation that earlier intervention makes for better visual and anatomical outcomes in an already visually devastating condition. Postoperative complications included the development of glaucoma. Thirty-six percent (four patients) developed glaucoma and required treatment (two controlled with topical antiglaucoma drops and two required glaucoma tube surgery). This rate is similar to that reported by other studies of patients with similar anatomical abnormalities undergoing similar surgical procedures. Vasavada et al19 found the rate of aphakic glaucoma in microphthalmic eyes after cataract surgery to be 30.9%. Johnson and Keech20 reported a rate of aphakic glaucoma after cataract surgery in patients with PFV as 32%. Glaucoma was seen in 33% of individuals receiving LSV for retinopathy of prematurity in a study by Choi et al.21 Additionally, it is important to note that the risk of glaucoma in patients with PFV is 23% to 32% with surgical intervention,13,20 and also in many patients with PFV without surgical intervention.3,5 Limitations to our study include its retrospective design, small patient population, lack of control group, variability in follow-up, and compliance with amblyopia therapy. Although PFV is a common congenital malformation, it usually presents in very mild forms, such as a persistent pupillary membrane or a Mittendorf dot, which typically do not affect vision or require surgical intervention. It is very rare to see severe forms of PFV, especially cases with associated TRD. Given this, conducting a prospective study with a large patient population is not practical. In addition, visual acuity assessment of the infant and pediatric populations offers unique challenges. Children have limited verbal communication, and often visual acuity is based on objective measurements. In conclusion, our data support consideration of early surgical intervention for patients with posterior PFV with the macula affecting TRD. Our study suggests that surgical intervention at younger than or equal to 13 months of age offers improved visual potential. Complications observed in this study include the development of glaucoma, the rate of which was similar to that noted for lensectomy for microphthalmia, and LSV for retinopathy of prematurity. Only 2 of the 11 patients included in this study required a surgical glaucoma procedure to achieve adequate postoperative intraocular pressure control.
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Key words: persistent fetal vasculature, traction retinal detachment, pars plana vitrectomy, cataract, glaucoma, persistent hyperplastic vitreous. References 1. Reese A. Persistent hyperplastic primary vitreous. Am J Ophthalmol 1955;40:317–331. 2. Goldberg M. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV) LIV Edward Jackson Memorial Lecture. Am J Ophthalmol 1997;124:587–626. 3. Pollard ZF. Persistent hyperplastic primary vitreous: diagnosis, treatment, and results. Trans Am Ophthalmol Soc 1997;95: 487–549. 4. Manschot WA. Persistent hyperplastic primary vitreous; special reference to preretinal glial tissues as pathological characteristic and to the development of the primary vitreous. AMA Arch Ophthalmol 1958;59:188–203. 5. Reese A. Persistent hyperplastic primary vitreous. Trans Am Acad Ophthalmol Otolaryngol 1955;59:271–295. 6. Haddad R, Font RL, Reeser F. Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature. Surv Ophthalmol 1978;23:123–134. 7. Kremer I, Nissenkorn I, Ben-Sira I, Steinherz R. Unusual ophthalmological findings in a case of partial trisomy 15. Metab Pediatr Syst Ophthalmol 1986;9:597–598. 8. Marshman WE, Jan JE, Lyons CJ. Neurologic abnormalities associated with persistent hyperplastic primary vitreous. Can J Ophthalmol 1999;34:17–22. 9. Shastry BS. Persistent hyperplastic primary vitreous: congenital malformation of the eye. Clin Experiment Ophthalmol 2009;37:884–890.
10. Gerding H, Gullotta F, Kuchelmeister K, Busse H. Ocular findings in Walker-Warburg syndrome. Childs Nerv Syst 1993;9:418–420. 11. Soheilian M, Vistamehr S, Rahmani B, et al. Outcomes of surgical and nonsurgical management of persistent fetal vasculature. Ann Ophthalmol 2003;35:28–37. 12. Anteby I, Cohen E, Karshai I, BenEzra D. Unilateral persistent hyperplastic primary vitreous: course and outcome. J AAPOS 2002;6:92–99. 13. Hunt A, Rowe N, Lam A, Martin F. Outcomes in persistent hyperplastic primary vitreous. Br J Ophthalmol 2005;89:859–863. 14. Sisk RA, Berrocal AM, Feuer WJ, Murray TG. Visual and anatomic outcomes with or without surgery in persistent fetal vasculature. Ophthalmology 2010;117:2178–2183.e1–e2. 15. Teller DY, Dobson V, Mayer DL. Teller Acuity Cards II (TAC II), Reference, Instruction Manual. Chicago, IL: Stereo Optical Co, Inc; 2005. 16. Ceron O, Lou PL, Kroll AJ, Walton DS. The vitreoretinal manifestations of persistent hyperplastic primary vitreous (PHPV) and their management. Int Ophthalmol Clin 2008;48:53–62. 17. Dass AB, Trese MT. Surgical results of persistent hyperplastic primary vitreous. Ophthalmology 1999;106:280–284. 18. Walsh MK, Drenser KA, Capone A, Trese MT. Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vasculature syndrome. Retina 2010;30:S2–S8. 19. Vasavada VA, Dixit NV, Ravat FA, et al. Intraoperative performance and postoperative outcomes of cataract surgery in infant eyes with microphthalmos. J Cataract Refract Surg 2009;35:519–528. 20. Johnson CP, Keech RV. Prevalence of glaucoma after surgery for PHPV and infantile cataracts. J Pediatr Ophthalmol Strabismus 1996;33:14–17. 21. Choi J, Kim JH, Kim SJ, Yu YS. Long-term results of lenssparing vitrectomy for stages 4B and 5 retinopathy of prematurity. Korean J Ophthalmol 2011;25:305–310.
