TRACTION RETINAL DETACHMENT UNDER SILICONE OIL TREATED WITH TWO-PORT, 25-GAUGE PARS PLANA VITRECTOMY AND SCISSOR SEGMENTATION IN A CHILD WITH FAMILIAL EXUDATIVE VITREORETINOPATHY Eric J. Sigler, MD,*† John C. Randolph, MD,*† Jorge I. Calzada, MD*†
Purpose: To describe a method of 25-gauge pars plana vitrectomy (PPV) with posterior hyaloid scissor segmentation in a child with recurrent retinal detachment caused by vitreous traction under silicone oil. Methods: A two-port, 25-gauge PPV was performed under previously instilled silicone oil with retinotomy and internal drainage of subretinal fluid. Results: A 5-year-old monocular girl with familial exudative vitreoretinopathy presented with decreased vision and a recurrent retinal detachment 3 weeks after PPV and air– silicone exchange for rhegmatogenous retinal detachment. Ophthalmoscopy, fundus photography, and optical coherence tomography revealed persistent posterior hyaloid adherent to multiple retinal foci inducing a tractional detachment. The patient underwent two-port PPV revision with scissor segmentation of vitreous traction. Follow-up examination at 3 months revealed the absence of vitreous traction, an attached retina, and markedly improved vision. Conclusion: Posterior vitreous sequestered under silicone oil inducing consecutive retinal detachment may be successfully managed with two-port interface PPV and scissor segmentation of vitreous traction. RETINAL CASES & BRIEF REPORTS 7:183–187, 2013
detachment (RD).2–4 Histologically, FEVR has been reported to demonstrate exudate, vascular thickening, retinal pigment epithelial metaplasia, retinal disorganization and atrophy, and vitreoretinal membranes.5,6 The genetic features of FEVR are heterogeneous and include incomplete penetrance, variable expressivity, and multiple gene mutation associations.7–9 The clinical course is highly variable, ranging from asymptomatic carriers to severe retinal dysplasia with total RD with a poor prognosis.3,10–12 The fluorescein angiographic features include peripheral retinal avascularity, neovacularization, and focal vascular leakage within areas of vitreoretinal traction.13
From the *Charles Retina Institute, Memphis, Tennessee; and †Division of Vitreoretinal Surgery, Hamilton Eye Institute, University of Tennessee at Memphis, Memphis, Tennessee.
F
amilial exudative vitreoretinopathy (FEVR) is an inherited vitreoretinal disorder with variable clinical features and heterogeneous morphology. First described by Criswick and Schepens1 in a series of six patients, the morphologic features of FEVR include tortuous retinal vasculature, incomplete retinal vascularization, retinal folds, subretinal exudate, and vitreoretinal traction occasionally leading to retinal 183
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Retinal detachment occurring in FEVR may result from an exudative, rhegmatogenous, or tractional process11,14–16 and has been reported to have a variable surgical success rate with guarded visual prognosis. Tractional RD may occur as a result of contraction of fibrocellular vitreoretinal membranes composed of vascular elements and glia.16,17 The purpose of the present report was to describe the clinical course, imaging characteristics, and successful surgical technique used in a child with FEVR and vitreous tractioninduced RD under previously placed silicone oil (SO).
Case Report A 5-year-old white child with a history of FEVR presented with decreased vision in her left eye. The child was the product of a 41-week gestation and weighed 2.6 kg at birth. The patient’s mother had a history of high myopia and asymptomatic FEVR, confirmed by recent ophthalmoscopy, and with stable visual acuity of 20/40 in both eyes. There was no history of prematurity, perinatal oxygen supplementation, or intensive care unit admission for either the patient or her mother. Best-corrected Snellen visual acuity (BCVA) was hand motion in her right eye and 20/100 in her left eye. Ophthalmoscopy revealed a total RD in the right eye with a large temporal retinal break through the fovea and absent peripheral retina in this meridian and was considered a poor candidate for operative repair because of multiple previous surgeries at an outside institution. The left eye demonstrated atrophic peripheral retina with incomplete vascularization of the superior and temporal retina. Examination under anesthesia was performed including laser retinopexy to several regions of atrophic holes and overlying gliosis within the superior and temporal retina of the left eye. Three weeks later, the patient presented with decreased vision in her left eye. Examination revealed hand motion only BCVA and a total RD with multiple new retinal breaks in the peripheral temporal retina, adjacent to but not involving the recent laser retinopexy. The patient underwent standard 3-port 25-gauge pars plana vitrectomy (PPV) with internal drainage of subretinal fluid, endophotocoagulation, and air–SO exchange. Induction of complete posterior vitreous detachment was attempted intraoperatively but adherence of the posterior hyaloid precluded safe separation from the retinal surface in the nasal and inferior quadrants. Posterior hyaloid separation was achieved in the region of the temporal retina and culprit retinal breaks. The patient achieved complete retinal reattachment intraoperatively and the vitreous cavity was filled with SO for postoperative endotamponade. On the first postoperative day, BCVA was 20/200 and ophthalmoscopy revealed an attached retina with .90% SO fill. Three-week follow-up examination revealed decrease in BCVA to light perception only and the findings are presented in Figure 1. The patient was treated with PPV revision using a two-port approach. Interface vitrectomy was performed under the previously placed SO and the persistent posterior hyaloid strands were severed with 25-gauge curved scissors. This maneuver was performed using conformal, curved 25-gauge scissors (Alcon, Fort Worth, TX) to segment posterior vitreous at a preretinal location throughout the The authors have no financial or conflicts of interests to disclose. Reprint requests: Eric J. Sigler, MD, Charles Retina Institute, 6401 Poplar Avenue, Suite 190, Memphis, TN 38119; e-mail: [email protected]
postequatorial nasal and inferior region. This resulted in complete retinal reattachment. Macular reattachment was confirmed by intraoperative optical coherence tomography. Removal of small amounts of additional peripheral vitreous was performed with the microvitrector positioned beneath the vitreous–silicone interface. No additional retinopexy or SO infusion was necessary. On the first postoperative day, BCVA was improved to 20/400 and ophthalmoscopy revealed a completely reattached retina with absence of posterior hyaloid traction and SO-filled vitreous cavity. Three-month follow-up examination revealed BCVA of 20/100 and no evidence of RD or posterior hyaloid traction. Spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany) throughout the clinical course is presented in Figure 2. Imaging at 3-month follow-up is demonstrated in Figure 3.
Discussion The present case illustrates the inherent difficulties involved in RD repair in pediatric patients with FEVR and provides a potentially useful surgical technique for repair of consecutive tractional RD in FEVR. To date, a least five reports involving 111 eyes have described the challenges present in RD repair in FEVR.4,10,16,18,19 Most of these have been treated with scleral buckling, with a minority treated with combined PPV/scleral buckling or consecutive PPV after failed scleral buckling. To our knowledge, there are no previous reports of early tractional detachment because of persistent posterior hyaloid under SO without contracture or membrane formation in FEVR. During the initial PPV, care was taken to meticulously remove the posterior hyaloid from regions of RD surrounding all retinal breaks. During induction of the peripapillary and nasal posterior hyaloid separation, firm adhesions were encountered and were left in place to avoid posterior iatrogenic retinal breaks. Ikeda et al18 in perhaps the largest and best documented series of PPV for rhegmatogenous RD in FEVR, reported iatrogenic breaks in 22 of 28 eyes undergoing initial surgical repair. Although no iatrogenic breaks were created in this case, the consecutive detachment occurred in the early postoperative period because of incomplete posterior hyaloid separation. One possible cause of a new retinal break, in this case, was necrosis of retinal tissue caused by laser retinopexy or alteration in vitreoretinal interface adjacent to retinopexy. The former seems unlikely because the areas of previous retinopexy were attached during the consecutive RD. This illustrates the delicate balance between difficult artificial posterior vitreous detachment and the avoidance of surgically induced retinal breaks, in both pediatric vitreoretinal cases and those with potentially thin, atrophic or dysplastic retinal tissue. Interface vitrectomy is a previously described technique for PPV with surgical tasks performed at the
FAMILIAL EXUDATIVE VITREORETINOPATHY
185
Fig. 1. Preoperative fundus photography and optical coherence tomography of the patient with FEVR and RD under SO. A. Fundus photograph of the right eye demonstrating tortuous retinal vasculature with a large temporal retinal break extending to the foveal region (arrow) and a complete RD. B. Composite fundus photograph of the left eye 2 weeks after vitrectomy and SO with persistent posterior hyaloid (arrow) visible at the vitreous–oil interface, and tractional RD involving the central macula (asterisk). C. Fundus photograph of left eye demonstrating posterior hyaloid attached through the nasal peripapillary region, green arrow denoting raster position for spectral domain optical coherence tomography. D. Tractional RD due to posterior hyaloid adherence (arrow) with SO meniscus visible (arrowhead).
interface between retina or vitreous and air, SO, or perfluorocarbon liquids.20 The present case illustrates both the appearance of vitreous gel and posterior hyaloid at the interface between vitreous and SO, and the use of a two-port “under oil” interface vitrectomy for successful reoperation on consecutive RD. Rather
than a remove-and-replace approach to this case, we chose a two-port technique20,21 to use the physical properties of the oil–vitreoretinal interface to our advantage. The interface between vitreous and oil allows improved visualization of residual vitreous and stabilization of intraocular tissue through viscous
Fig. 2. Spectral domain optical coherence tomography of the patient with FEVR and tractional RD. A. Scanning laser ophthalmoscopic image showing the raster position (green dashed line) in subsequent images. B. One year before RD showing an attached macula with moderately attenuated retinal elements in the temporal region. C. Retinal detachment under SO after initial vitrectomy involving the foveal region (asterisk). D. Three months after two-port vitrectomy under SO with scissor segmentation of vitreous traction, the macula is reattached.
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Fig. 3. Postoperative fundus photography and optical coherence tomography of the patient with RD and FEVR. A. Fundus photograph of the right eye 3 months after two-port PPV with scissor segmentation of posterior hyaloid demonstrating attached macula and absence of previously present vitreous. B. Fundus photograph of the nasal retina demonstrating attached retina and absence of vitreous traction. C. Composite fundus photograph demonstrating attached retina and pigment epithelial alterations in the region of previously adherent vitreous and tractional RD; green arrow indicates position of raster for optical coherence tomography. D. The macula is attached with no residual vitreous traction.
dampening of retinal motion.20,21 The vitreous cutter can be used under oil at the interface between vitreous and oil, and scissor segmentation may be used to sever focally adherent vitreous strands. Theoretically, with residual vitreous traction after initial PPV, a remove-andreplace technique for SO may have induced longer operating time,21 diminished visibility of the culprit vitreous traction, and increased risk of iatrogenic retinal breaks. Additional methods of providing retinal stabilization, such as perfluorocarbon heavy liquids, may additionally be used for interface vitrectomy.20 In the context of retinal breaks, however, the very low viscosity and relatively lower surface tension of perfluorocarbons probably reduce the efficacy of these substances compared with SO for interface vitrectomy. Air provides spring dampening of retinal motion and high surface tension and may be used to perform interface vitrectomy.20,21 However, in an oil-filled eye, both these options are impractical because of the need for a removeand-replace approach for SO, and the absence of viscous dampening of retinal motion achieved when operating under oil may make these options less efficacious.20,21 Triamcinolone acetate particulate staining may be used to better visualize vitreous, but it is difficult to use under SO because of sequestration beneath the oil–vitreous interface, and we have found that this is unnecessary for two-port reoperations.21 In conclusion, we have presented a challenging surgical case highlighting the particular difficulties present with RD repair in FEVR and in pediatric
patients. This monocular patient has achieved successful retinal reattachment and improved, functional visual acuity at 3-month follow-up. We suggest that a two-port interface approach to PPV for retinal reattachment may be efficaciously used in consecutive RD because of vitreous traction under previously placed SO. Key words: familial exudative vitreoretinopathy, interface vitrectomy, pars plana vitrectomy, pediatric retinal detachment, retinal detachment, silicone oil. References 1. Criswick VG, Schepens CL. Familial exudative vitreoretinopathy. Am J Ophthalmol 1969;68:578–594. 2. Miyakubo H, Inohara N, Hashimoto K. Retinal involvement in familial exudative vitreoretinopathy. Ophthalmologica 1982;185:125–135. 3. Swanson D, Rush P, Bird AC. Visual loss from retinal oedema in autosomal dominant exudative vitreoretinopathy. Br J Ophthalmol 1982;66:627–629. 4. Chen SN, Hwang JF, Lin CJ. Clinical characteristics and surgical management of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. Retina 2012;32:220–225. 5. Brockhurst RJ, Albert DM, Zakov ZN. Pathologic findings in familial exudative vitreoretinopathy. Arch Ophthalmol 1981;99:2143–2146. 6. Boldrey EE, Egbert P, Gass JD, Friberg T. The histopathology of familial exudative vitreoretinopathy. A report of two cases. Arch Ophthalmol 1985;103:238–241. 7. Shastry BS, Hejtmancik JF, Trese MT. Identification of novel missense mutations in the Norrie disease gene associated with
FAMILIAL EXUDATIVE VITREORETINOPATHY
8.
9.
10.
11.
12.
13. 14.
one X-linked and four sporadic cases of familial exudative vitreoretinopathy. Hum Mutat 1997;9:396–401. Shastry BS, Trese MT. Familial exudative vitreoretinopathy: further evidence for genetic heterogeneity. Am J Med Genet 1997;69:217–218. Toomes C, Bottomley HM, Jackson RM, et al. Mutations in LRP5 or FZD4 underlie the common familial exudative vitreoretinopathy locus on chromosome 11q. Am J Hum Genet 2004;74:721–730. Ranchod TM, Ho LY, Drenser KA, et al. Clinical presentation of familial exudative vitreoretinopathy. Ophthalmology 2011;118:2070–2075. van Nouhuys CE. Signs, complications, and platelet aggregation in familial exudative vitreoretinopathy. Am J Ophthalmol 1991;111:34–41. Machemer R, Williams JM Sr. Pathogenesis and therapy of traction detachment in various retinal vascular diseases. Am J Ophthalmol 1988;105:170–181. Canny CL, Oliver GL. Fluorescein angiographic findings in familial exudative vitreoretinopathy. Arch Ophthalmol 1976;94:1114–1120. van Nouhuys CE. Juvenile retinal detachment as a complication of familial exudative vitreoretinopathy. Fortschr Ophthalmol 1989;86:221–223.
187 15. Pendergast SD, Trese MT. Familial exudative vitreoretinopathy. Results of surgical management. Ophthalmology 1998;105:1015–1023. 16. Glazer LC, Maguire A, Blumenkranz MS, et al. Improved surgical treatment of familial exudative vitreoretinopathy in children. Am J Ophthalmol 1995;120:471–479. 17. Joshi MM, Ciaccia S, Trese MT, Capone A Jr. Posterior hyaloid contracture in pediatric vitreoretinopathies. Retina 2006;26:S38–S41. 18. Ikeda T, Fujikado T, Tano Y, et al. Vitrectomy for rhegmatogenous or tractional retinal detachment with familial exudative vitreoretinopathy. Ophthalmology 1999; 106:1081–1085. 19. Shubert A, Tasman W. Familial exudative vitreoretinopathy: surgical intervention and visual acuity outcomes. Graefes Arch Clin Exp Ophthalmol 1997;235:490–493. 20. Charles S, Calzada J, Wood B. Vitreous Microsurgery. 5th ed Philadelphia, PA: Wolters Kluwer/Lippincott Williams & Wilkins; 2011. 21. Charles S, Randolph JC, Sigler EJ. Pars plana vitectomy reoperation without removing silicone oil. Retina 2012;32:1664–1665.
Purpose: To describe a method of 25-gauge pars plana vitrectomy (PPV) with posterior hyaloid scissor segmentation in a child with recurrent retinal detachment caused by vitreous traction under silicone oil. Methods: A two-port, 25-gauge PPV was performed under previously instilled silicone oil with retinotomy and internal drainage of subretinal fluid. Results: A 5-year-old monocular girl with familial exudative vitreoretinopathy presented with decreased vision and a recurrent retinal detachment 3 weeks after PPV and air– silicone exchange for rhegmatogenous retinal detachment. Ophthalmoscopy, fundus photography, and optical coherence tomography revealed persistent posterior hyaloid adherent to multiple retinal foci inducing a tractional detachment. The patient underwent two-port PPV revision with scissor segmentation of vitreous traction. Follow-up examination at 3 months revealed the absence of vitreous traction, an attached retina, and markedly improved vision. Conclusion: Posterior vitreous sequestered under silicone oil inducing consecutive retinal detachment may be successfully managed with two-port interface PPV and scissor segmentation of vitreous traction. RETINAL CASES & BRIEF REPORTS 7:183–187, 2013
detachment (RD).2–4 Histologically, FEVR has been reported to demonstrate exudate, vascular thickening, retinal pigment epithelial metaplasia, retinal disorganization and atrophy, and vitreoretinal membranes.5,6 The genetic features of FEVR are heterogeneous and include incomplete penetrance, variable expressivity, and multiple gene mutation associations.7–9 The clinical course is highly variable, ranging from asymptomatic carriers to severe retinal dysplasia with total RD with a poor prognosis.3,10–12 The fluorescein angiographic features include peripheral retinal avascularity, neovacularization, and focal vascular leakage within areas of vitreoretinal traction.13
From the *Charles Retina Institute, Memphis, Tennessee; and †Division of Vitreoretinal Surgery, Hamilton Eye Institute, University of Tennessee at Memphis, Memphis, Tennessee.
F
amilial exudative vitreoretinopathy (FEVR) is an inherited vitreoretinal disorder with variable clinical features and heterogeneous morphology. First described by Criswick and Schepens1 in a series of six patients, the morphologic features of FEVR include tortuous retinal vasculature, incomplete retinal vascularization, retinal folds, subretinal exudate, and vitreoretinal traction occasionally leading to retinal 183
184
RETINAL CASES & BRIEF REPORTS´ 2013 VOLUME 7 NUMBER 3
Retinal detachment occurring in FEVR may result from an exudative, rhegmatogenous, or tractional process11,14–16 and has been reported to have a variable surgical success rate with guarded visual prognosis. Tractional RD may occur as a result of contraction of fibrocellular vitreoretinal membranes composed of vascular elements and glia.16,17 The purpose of the present report was to describe the clinical course, imaging characteristics, and successful surgical technique used in a child with FEVR and vitreous tractioninduced RD under previously placed silicone oil (SO).
Case Report A 5-year-old white child with a history of FEVR presented with decreased vision in her left eye. The child was the product of a 41-week gestation and weighed 2.6 kg at birth. The patient’s mother had a history of high myopia and asymptomatic FEVR, confirmed by recent ophthalmoscopy, and with stable visual acuity of 20/40 in both eyes. There was no history of prematurity, perinatal oxygen supplementation, or intensive care unit admission for either the patient or her mother. Best-corrected Snellen visual acuity (BCVA) was hand motion in her right eye and 20/100 in her left eye. Ophthalmoscopy revealed a total RD in the right eye with a large temporal retinal break through the fovea and absent peripheral retina in this meridian and was considered a poor candidate for operative repair because of multiple previous surgeries at an outside institution. The left eye demonstrated atrophic peripheral retina with incomplete vascularization of the superior and temporal retina. Examination under anesthesia was performed including laser retinopexy to several regions of atrophic holes and overlying gliosis within the superior and temporal retina of the left eye. Three weeks later, the patient presented with decreased vision in her left eye. Examination revealed hand motion only BCVA and a total RD with multiple new retinal breaks in the peripheral temporal retina, adjacent to but not involving the recent laser retinopexy. The patient underwent standard 3-port 25-gauge pars plana vitrectomy (PPV) with internal drainage of subretinal fluid, endophotocoagulation, and air–SO exchange. Induction of complete posterior vitreous detachment was attempted intraoperatively but adherence of the posterior hyaloid precluded safe separation from the retinal surface in the nasal and inferior quadrants. Posterior hyaloid separation was achieved in the region of the temporal retina and culprit retinal breaks. The patient achieved complete retinal reattachment intraoperatively and the vitreous cavity was filled with SO for postoperative endotamponade. On the first postoperative day, BCVA was 20/200 and ophthalmoscopy revealed an attached retina with .90% SO fill. Three-week follow-up examination revealed decrease in BCVA to light perception only and the findings are presented in Figure 1. The patient was treated with PPV revision using a two-port approach. Interface vitrectomy was performed under the previously placed SO and the persistent posterior hyaloid strands were severed with 25-gauge curved scissors. This maneuver was performed using conformal, curved 25-gauge scissors (Alcon, Fort Worth, TX) to segment posterior vitreous at a preretinal location throughout the The authors have no financial or conflicts of interests to disclose. Reprint requests: Eric J. Sigler, MD, Charles Retina Institute, 6401 Poplar Avenue, Suite 190, Memphis, TN 38119; e-mail: [email protected]
postequatorial nasal and inferior region. This resulted in complete retinal reattachment. Macular reattachment was confirmed by intraoperative optical coherence tomography. Removal of small amounts of additional peripheral vitreous was performed with the microvitrector positioned beneath the vitreous–silicone interface. No additional retinopexy or SO infusion was necessary. On the first postoperative day, BCVA was improved to 20/400 and ophthalmoscopy revealed a completely reattached retina with absence of posterior hyaloid traction and SO-filled vitreous cavity. Three-month follow-up examination revealed BCVA of 20/100 and no evidence of RD or posterior hyaloid traction. Spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany) throughout the clinical course is presented in Figure 2. Imaging at 3-month follow-up is demonstrated in Figure 3.
Discussion The present case illustrates the inherent difficulties involved in RD repair in pediatric patients with FEVR and provides a potentially useful surgical technique for repair of consecutive tractional RD in FEVR. To date, a least five reports involving 111 eyes have described the challenges present in RD repair in FEVR.4,10,16,18,19 Most of these have been treated with scleral buckling, with a minority treated with combined PPV/scleral buckling or consecutive PPV after failed scleral buckling. To our knowledge, there are no previous reports of early tractional detachment because of persistent posterior hyaloid under SO without contracture or membrane formation in FEVR. During the initial PPV, care was taken to meticulously remove the posterior hyaloid from regions of RD surrounding all retinal breaks. During induction of the peripapillary and nasal posterior hyaloid separation, firm adhesions were encountered and were left in place to avoid posterior iatrogenic retinal breaks. Ikeda et al18 in perhaps the largest and best documented series of PPV for rhegmatogenous RD in FEVR, reported iatrogenic breaks in 22 of 28 eyes undergoing initial surgical repair. Although no iatrogenic breaks were created in this case, the consecutive detachment occurred in the early postoperative period because of incomplete posterior hyaloid separation. One possible cause of a new retinal break, in this case, was necrosis of retinal tissue caused by laser retinopexy or alteration in vitreoretinal interface adjacent to retinopexy. The former seems unlikely because the areas of previous retinopexy were attached during the consecutive RD. This illustrates the delicate balance between difficult artificial posterior vitreous detachment and the avoidance of surgically induced retinal breaks, in both pediatric vitreoretinal cases and those with potentially thin, atrophic or dysplastic retinal tissue. Interface vitrectomy is a previously described technique for PPV with surgical tasks performed at the
FAMILIAL EXUDATIVE VITREORETINOPATHY
185
Fig. 1. Preoperative fundus photography and optical coherence tomography of the patient with FEVR and RD under SO. A. Fundus photograph of the right eye demonstrating tortuous retinal vasculature with a large temporal retinal break extending to the foveal region (arrow) and a complete RD. B. Composite fundus photograph of the left eye 2 weeks after vitrectomy and SO with persistent posterior hyaloid (arrow) visible at the vitreous–oil interface, and tractional RD involving the central macula (asterisk). C. Fundus photograph of left eye demonstrating posterior hyaloid attached through the nasal peripapillary region, green arrow denoting raster position for spectral domain optical coherence tomography. D. Tractional RD due to posterior hyaloid adherence (arrow) with SO meniscus visible (arrowhead).
interface between retina or vitreous and air, SO, or perfluorocarbon liquids.20 The present case illustrates both the appearance of vitreous gel and posterior hyaloid at the interface between vitreous and SO, and the use of a two-port “under oil” interface vitrectomy for successful reoperation on consecutive RD. Rather
than a remove-and-replace approach to this case, we chose a two-port technique20,21 to use the physical properties of the oil–vitreoretinal interface to our advantage. The interface between vitreous and oil allows improved visualization of residual vitreous and stabilization of intraocular tissue through viscous
Fig. 2. Spectral domain optical coherence tomography of the patient with FEVR and tractional RD. A. Scanning laser ophthalmoscopic image showing the raster position (green dashed line) in subsequent images. B. One year before RD showing an attached macula with moderately attenuated retinal elements in the temporal region. C. Retinal detachment under SO after initial vitrectomy involving the foveal region (asterisk). D. Three months after two-port vitrectomy under SO with scissor segmentation of vitreous traction, the macula is reattached.
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Fig. 3. Postoperative fundus photography and optical coherence tomography of the patient with RD and FEVR. A. Fundus photograph of the right eye 3 months after two-port PPV with scissor segmentation of posterior hyaloid demonstrating attached macula and absence of previously present vitreous. B. Fundus photograph of the nasal retina demonstrating attached retina and absence of vitreous traction. C. Composite fundus photograph demonstrating attached retina and pigment epithelial alterations in the region of previously adherent vitreous and tractional RD; green arrow indicates position of raster for optical coherence tomography. D. The macula is attached with no residual vitreous traction.
dampening of retinal motion.20,21 The vitreous cutter can be used under oil at the interface between vitreous and oil, and scissor segmentation may be used to sever focally adherent vitreous strands. Theoretically, with residual vitreous traction after initial PPV, a remove-andreplace technique for SO may have induced longer operating time,21 diminished visibility of the culprit vitreous traction, and increased risk of iatrogenic retinal breaks. Additional methods of providing retinal stabilization, such as perfluorocarbon heavy liquids, may additionally be used for interface vitrectomy.20 In the context of retinal breaks, however, the very low viscosity and relatively lower surface tension of perfluorocarbons probably reduce the efficacy of these substances compared with SO for interface vitrectomy. Air provides spring dampening of retinal motion and high surface tension and may be used to perform interface vitrectomy.20,21 However, in an oil-filled eye, both these options are impractical because of the need for a removeand-replace approach for SO, and the absence of viscous dampening of retinal motion achieved when operating under oil may make these options less efficacious.20,21 Triamcinolone acetate particulate staining may be used to better visualize vitreous, but it is difficult to use under SO because of sequestration beneath the oil–vitreous interface, and we have found that this is unnecessary for two-port reoperations.21 In conclusion, we have presented a challenging surgical case highlighting the particular difficulties present with RD repair in FEVR and in pediatric
patients. This monocular patient has achieved successful retinal reattachment and improved, functional visual acuity at 3-month follow-up. We suggest that a two-port interface approach to PPV for retinal reattachment may be efficaciously used in consecutive RD because of vitreous traction under previously placed SO. Key words: familial exudative vitreoretinopathy, interface vitrectomy, pars plana vitrectomy, pediatric retinal detachment, retinal detachment, silicone oil. References 1. Criswick VG, Schepens CL. Familial exudative vitreoretinopathy. Am J Ophthalmol 1969;68:578–594. 2. Miyakubo H, Inohara N, Hashimoto K. Retinal involvement in familial exudative vitreoretinopathy. Ophthalmologica 1982;185:125–135. 3. Swanson D, Rush P, Bird AC. Visual loss from retinal oedema in autosomal dominant exudative vitreoretinopathy. Br J Ophthalmol 1982;66:627–629. 4. Chen SN, Hwang JF, Lin CJ. Clinical characteristics and surgical management of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. Retina 2012;32:220–225. 5. Brockhurst RJ, Albert DM, Zakov ZN. Pathologic findings in familial exudative vitreoretinopathy. Arch Ophthalmol 1981;99:2143–2146. 6. Boldrey EE, Egbert P, Gass JD, Friberg T. The histopathology of familial exudative vitreoretinopathy. A report of two cases. Arch Ophthalmol 1985;103:238–241. 7. Shastry BS, Hejtmancik JF, Trese MT. Identification of novel missense mutations in the Norrie disease gene associated with
FAMILIAL EXUDATIVE VITREORETINOPATHY
8.
9.
10.
11.
12.
13. 14.
one X-linked and four sporadic cases of familial exudative vitreoretinopathy. Hum Mutat 1997;9:396–401. Shastry BS, Trese MT. Familial exudative vitreoretinopathy: further evidence for genetic heterogeneity. Am J Med Genet 1997;69:217–218. Toomes C, Bottomley HM, Jackson RM, et al. Mutations in LRP5 or FZD4 underlie the common familial exudative vitreoretinopathy locus on chromosome 11q. Am J Hum Genet 2004;74:721–730. Ranchod TM, Ho LY, Drenser KA, et al. Clinical presentation of familial exudative vitreoretinopathy. Ophthalmology 2011;118:2070–2075. van Nouhuys CE. Signs, complications, and platelet aggregation in familial exudative vitreoretinopathy. Am J Ophthalmol 1991;111:34–41. Machemer R, Williams JM Sr. Pathogenesis and therapy of traction detachment in various retinal vascular diseases. Am J Ophthalmol 1988;105:170–181. Canny CL, Oliver GL. Fluorescein angiographic findings in familial exudative vitreoretinopathy. Arch Ophthalmol 1976;94:1114–1120. van Nouhuys CE. Juvenile retinal detachment as a complication of familial exudative vitreoretinopathy. Fortschr Ophthalmol 1989;86:221–223.
187 15. Pendergast SD, Trese MT. Familial exudative vitreoretinopathy. Results of surgical management. Ophthalmology 1998;105:1015–1023. 16. Glazer LC, Maguire A, Blumenkranz MS, et al. Improved surgical treatment of familial exudative vitreoretinopathy in children. Am J Ophthalmol 1995;120:471–479. 17. Joshi MM, Ciaccia S, Trese MT, Capone A Jr. Posterior hyaloid contracture in pediatric vitreoretinopathies. Retina 2006;26:S38–S41. 18. Ikeda T, Fujikado T, Tano Y, et al. Vitrectomy for rhegmatogenous or tractional retinal detachment with familial exudative vitreoretinopathy. Ophthalmology 1999; 106:1081–1085. 19. Shubert A, Tasman W. Familial exudative vitreoretinopathy: surgical intervention and visual acuity outcomes. Graefes Arch Clin Exp Ophthalmol 1997;235:490–493. 20. Charles S, Calzada J, Wood B. Vitreous Microsurgery. 5th ed Philadelphia, PA: Wolters Kluwer/Lippincott Williams & Wilkins; 2011. 21. Charles S, Randolph JC, Sigler EJ. Pars plana vitectomy reoperation without removing silicone oil. Retina 2012;32:1664–1665.
