Familial exudative vitreoretinopathy (FEVR) can often present with retinal falciform folds, and rarely with retrolenticular adhesive radial retinal folds. Management of advanced FEVR-associated tractional falciform folds with retrolenticular adhesion to the peripheral retina in the literature has been limited to vitrectomy with or without lensectomy. The authors describe a unique surgical management of a case of bilateral FEVR-associated tractional radial folds with nonaxial retrolenticular adhesion treated with scleral buckling with deferred laser, avoiding the complications associated with vitrectomy and lensectomy on ocular development.
[Ophthalmic Surg Lasers Imaging Retina. 2019;50:594–596.]
Familial exudative vitreoretinopathy (FEVR) is a vitreoretinal dystrophy that most commonly presents in childhood. FEVR has a variety of inheritance patterns and phenotypic expressions, including varying radial retinal folds. These folds most commonly run temporally; however, they have been described in all quadrants.1 Radial falciform folds can be macula-sparing or macula-involving, and range from dry, knife-like, to broad-shaped with subretinal exudation. Rarely, these tractional folds can be adherent to the posterior lens capsule. In one of the largest reviews of clinical presentations of FEVR, Ranchod et al. found vitreoretinal-lens adhesions occurring in 6% of cases.1 Management of advanced FEVR-associated tractional falciform folds with retrolenticular adhesion to the peripheral retina in the literature has been limited to vitrectomy with or without lensectomy.1–4 We describe a unique surgical management of a case of bilateral FEVR-associated tractional radial folds with retrolenticular adhesion treated with scleral buckling with deferred laser.
A 12-day-old microcephalic male newborn born at 39 weeks gestational age presented for an ophthalmic examination. Birth weight was 2.80 kg, with an otherwise unremarkable gestational course. Examination under anesthesia showed bilateral foveal sparing temporal exudative-tractional radial folds with bilateral nonaxial peripheral retrolenticular adhesions consistent with a diagnosis of FEVR (Figure 1). Since the traction was foveal-sparing and located in the far periphery, and the lenticular adhesion was clear of the visual axis, surgical management consisted of bilateral scleral buckling (41 band in the right eye, 240 band in the left eye) with bilateral deferred laser 2 months postoperatively. Genetic testing revealed a mutation in KIF11a. Postoperatively, the tractional retinal detachment near-completely resolved, with bilateral release of the retrolenticular adhesions (Figure 2) The patient underwent bilateral scleral buckle severing 1.5 years later to allow unrestricted ocular growth. At the time of last exam, no cataractous changes were seen in both eyes.
Temporal retinal exudative tractional folds in familial exudative vitreoretinopathy in the right (A) and left (C, D) eyes associated with retrolenticular adhesion (B, E).
Released retrolenticular adhesion with near-resolution of the temporal exudative tractional retinal detachments in the right (A) and left (B) eyes status post-bilateral scleral buckling.
FEVR is a retinal vascular disease with a hallmark of avascular peripheral retina, with likely dysregulation of TGF-β and vascular endothelial growth factor levels.5 There is a paucity of literature guiding surgical management of pediatric FEVR complications. Pendergast and Trese outlined a commonly used a five-stage grading scheme designed around management and treatment of FEVR complications.6 Based on these guidelines and various other published series, in general, FEVR-associated exudative retinal detachments are commonly repaired with scleral buckling in combination with laser or deferred laser, whereas tractional detachments and tractional rhegmatogenous detachments are repaired with vitrectomy alone with or without lensectomy or, less commonly, in combination with scleral buckling.6 For this reason, ablative therapy to peripheral avascular retina is recommended. Contrary to published surgical reports on management of tractional folds with lenticular adhesion in FEVR standardized to treatment with vitrectomy with or without lensectomy, we present a unique case of bilateral release of retrolenticular traction and near-resolution of tractional retinal detachment with scleral buckling. Scleral buckling for FEVR, in addition to avoiding complications of vitrectomy and lensectomy, suppresses progression of fibrovascular proliferation when combined with ablative laser or cryotherapy and releases traction.4,7 Also, lens preservation benefits visual development and avoidance of amblyopia.
Retinal-fibrous proliferation to the posterior lens often promotes the need for vitrectomy with or without lensectomy to separate the adhesion and reduce anterior-posterior traction. However, in cases of nonaxial, mild lenticular adhesion, the present case exemplifies the role in which primary scleral buckling can offer a surgical treatment of FEVR-associated tractional retinal detachments with lenticular adhesion that avoids the complications associated with vitrectomy and lensectomy on ocular development.
- Ranchod TM, Ho LY, Drenser KA, Capone A Jr., Trese MT. Clinical presentation of familial exudative vitreoretinopathy. Ophthalmology. 2011;118(10):2070–2075. doi:10.1016/j.ophtha.2011.06.020 [CrossRef]21868098
- Ma J, Hu Y, Lu L, Ding X. 25-gauge lens-sparing vitrectomy with dissection of retrolental adhesions on the peripheral retina for familial exudative vitreoretinopathy in infants. Graefes Arch Clin Exp Ophthalmol. 2018;256(11):2233–2240. doi:10.1007/s00417-018-4035-4 [CrossRef]29907944
- Fei P, Yang W, Zhang Q, Jin H, Li J, Zhao P. Surgical management of advanced familial exudative vitreoretinopathy with complications. Retina. 2016;36(8):1480–1485. doi:10.1097/IAE.0000000000000961 [CrossRef]26807630
- Yamane T, Yokoi T, Nakayama Y, Nishina S, Azuma N. Surgical outcomes of progressive tractional retinal detachment associated with familial exudative vitreoretinopathy. Am J Ophthalmol. 2014;158(5):1049–1055. doi:10.1016/j.ajo.2014.08.009 [CrossRef]25127701
- Zhao S, Overbeek PA. Elevated TGF beta signaling inhibited ocular vascular vascular development. Dev Biol. 2001;237(1):45–53. doi:10.1006/dbio.2001.0360 [CrossRef]11518504
- Pendergast SD, Trese MT. Familial exudative vitreoretinopathy. Results of surgical management. Ophthalmology. 1998;105(6):1015–1023. doi:10.1016/S0161-6420(98)96002-X [CrossRef]9627651
- Yokoi T, Yokoi T, Kobayashi Y, Hiraoka M, Nishina S, Azuma N. Evaluation of scleral buckling for stage 4A retinopathy of prematurity by fluorescein angiography. Am J Ophthalmol. 2009;148(4):544–550. doi:10.1016/j.ajo.2009.05.027 [CrossRef]19589496