• Vitreoretinal

    Introduction

    Pediatric retinal detachment (RD) is a rare and complicated disease, with a previously reported incidence that ranges from 3% to 13%.1 Unlike RDs in adults, pediatric RDs tend to have a chronic duration, worse presenting visual acuity, macula involvement, and proliferative vitreoretinopathy (PVR) development at presentation.1,2 Surgical repair of RD in pediatric patients is a challenge for the vitreoretinal surgeon, especially due to the associated systemic comorbidities usually present in this young population.1,3

    RD in children can be rhegmatogenous (traumatic or nontraumatic) (RRD), tractional (TRD), or exudative. Visual and anatomical outcomes vary among categories of RD and also depend on systemic findings. Therefore, we describe the most common types of rhegmatogenous, tractional, and exudative retinal detachment and further discuss current surgical management of pediatric patients with RD.

    Presenting Characteristics

    Pediatric RDs have features that make them unique. The tendency for late diagnosis is an important characteristic that leads to macular involvement in 60%-85% of the cases and PVR grade C or worse in 20%-60%; these findings might be related to a delay in diagnosis due to delayed reporting of issues and higher degree of intraocular cellular activity and proliferation in the active immune system of young patients.1,3-11

    Up to 30% of the patients have bilateral RD at presentation or experienced a detachment in the fellow eye before their nineteenth birthday.2,4,11,12 Fellow eye retinal lesions, excluding eyes with history of trauma, have been reported to occur in up to 82.2% of the patients, with the most common causative finding being lattice degeneration.2,3,5,8

    Rhegmatogenous Retinal Detachment

    Pediatric RRDs account for 0.5%-8% of all patients suffering from RRD.4-6,8,10,11,13,14 Predisposing factors include trauma (21.1% to 53%), myopia (11.5%-41.44%), previous intraocular surgery (30%-51%) and congenital-developmental anomalies (17%-65%).3-7,10

    RRDs tend to be unilateral, of a known and short duration, and with better anatomic and functional outcomes.2 Surgical techniques used to repair RDs in children follow similar principles to those in adults: release of traction from the retinal defects, reattachment of the neurosensory retina to the retinal pigment epithelium (RPE), and creation of retinal to RPE adhesion with laser.4

    A thorough in-office retinal examination is usually difficult in the pediatric population. Therefore, in the operating room the eye to be treated should be examined with careful identification of any retinal breaks and extent of the subretinal fluid. As well, this is an excellent opportunity to examine the fellow eye and identify any retinal pathology that could predispose to an RD and subsequently treated with prophylactic laser retinopexy.4

    Surgical Management

    Options for surgical repair of pediatric rhegmatogenous retinal detachment are basically external reapposition of the retina versus intraocular subretinal fluid drainage and reattachment. Most of the RDs in children (< 18 years of age) should be approached initially with a scleral buckle, especially in patients with a clear lens, anterior breaks, and absence of PVR.4 Scleral buckles can be segmental, radial and encircling bands, and it is recommended that the width of the buckle extend from the ora serrata anteriorly to the posterior edge of the retinal breaks.15 In the past, temporary balloon buckles have also been described to be effective, but in this vulnerable population placement and further manipulation can result in unintended problems and therefore are not recommended. Scleral buckles work well in the pediatric population because the vitreous is more formed and can tamponade the retinal breaks once the traction has been relaxed by the buckle’s indentation and support.4

    There are several reasons to avoid intraocular surgery in children if possible. A child’s posterior hyaloid is often adherent to the retina and difficult to elevate anteriorly past the anterior border of the break; if any vitreous is left and complete release of traction is not accomplished, remaining anterior vitreous can contract and cause redetachment of the retina.4,16 Vitrectomy is also associated with a much higher rate of complications including cataract formation and endophthalmitis.4

    Primary scleral buckling has been associated with a high rate of anatomic success, ranging from 70% to 90%, even in cases with PVR, as previously published by Akabane and co-authors.4,9-11,17

    Indications for primary vitrectomy in children are limited and include poor fundus view due to presence of cataract or vitreous opacities, total RD or extensive RD (more than 3 quadrants), presence of PVR, posterior breaks, giant retinal tear, and when no breaks can be identified on dilated fundus examination.10

    In children, up to age 7 (when the ciliary body, lens, and retina reach adult size), the approach to vitrectomy is slightly different compared to the approach used in adults.18 The pars plana is incompletely developed; therefore, the peripheral retina should be inspected prior to surgery to confirm the presence of pars plana or pars plicata for trocar insertion. In cases where pars plana is adequate, sclerotomies should be placed much closer to the limbus (1.5 mm-3 mm), and smaller-gauge instruments, such as 25 or 27 gauge, should be utilized.18-20 When in doubt of adequate pars plana and in microphthalmic eyes, incisions should be created anteriorly at the limbus or iris root to prevent iatrogenic retinal breaks that can lead the eye to an inoperable state.20 Retinectomies are not uncommon in pediatric eyes undergoing vitrectomy in order to release vitreoretinal traction; according to previous reports in the literature, up to 38% of the patients need a retinectomy, especially patients with high grade PVR.4,8 Lensectomy is a common procedure as well, when the view is not optimal or when fibrovascular membranes develop behind the lens.4,8

    The use of silicone oil as a retinal tamponade is usually reserved for complex RDs. Nonetheless, many authors prefer its use for all types of RD in children, owing to the difficulties of postoperative positioning and intraocular pressure monitoring. However, there is exception to this preference in cases of RD secondary to choroidal coloboma because of the potential risk of the oil's getting into the subretinal space through the colobomatous defect.4,15,19,21 Silicone oil also allows for earlier visual rehabilitation, especially in the younger population that remains at risk for amblyopia.19,21

    There are several complications that are associated with pars plana vitrectomy or with scleral buckling or that are common to both procedures. Vitreous and retinal hemorrhage, iatrogenic retinal break formation, glaucoma, hypotony, keratopathy, cataract, and silicone oil emulsification are some of the complications previously reported after vitrectomy.19,22 Diplopia, strabismus, and scleral rupture have been reported after scleral buckling.22 Endophthalmitis, retinal incarceration, and episcleritis can be found after either pars plana vitrectomy or scleral buckling.19,22 All these adverse events should always be in the differential when postoperative complications develop.

    Genetic Disorders Predisposing to Rhegmatogenous Retinal Detachment

    These are the basic principles in surgically treating RRD in the pediatric population, but there are always complicated cases that become an exception to the rule and have to be evaluated on a case-by-case basis. Such is the case for patients with Stickler syndrome.

    Stickler syndrome is the most common cause of inherited RRD.23 It is characterized by generalized arthropathy, cleft palate, flat face, hearing loss, mandibular hypoplasia, an abnormal vitreous, myopia, early-onset cataract and glaucoma.4,15,24 When these patients present with a retinal detachment, due to the presence of abnormal vitreous in the retrolenticular space and possible cataract at the time of diagnosis, primary vitrectomy and lensectomy should be considered to release the anterior vitreous traction. Patients with Stickler syndrome diagnosis are also at a high risk of developing bilateral retinal detachments (up to 51%), and many authors support the idea of performing standard 360-degree laser prophylaxis in the fellow eye to prevent further RD in the fellow eye as previously published by Ang and colleagues.23

    Patients with Marfan syndrome constitute another group that belongs in this subgroup of inherited anomalies. The decision regarding the type of surgical approach depends on the type of retinal detachment, the presence of cataract or amount of displacement of the lens, pupil dilation, and the presence of thin sclera.2,4 In cases with a simple retinal detachment, a clear lens with minimal displacement, and good pupillary dilation, a primary scleral buckle can be considered; however, one must always be aware of thin sclera and its ability to support sutures and of the tension of the buckle leading to scleromalacia.2,4 Complicated cases with major lens displacement or cataract and poor dilation should be managed with primary pars plana vitrectomy and lensectomy with possible use of iris hooks, to enlarge the pupillary aperture to allow for peripheral dissection and visualization.2,4

    Other inherited syndromes that can predispose to retinal detachment and should be carefully managed are X-linked juvenile retinoschisis and choroidal coloboma. Patients with X-linked juvenile retinoschisis present with foveal schisis in a spoke-wheel pattern (100% of patients) and peripheral splitting of the retina, most often found inferotemporal (50% of patients).25-26 The splitting occurs in the nerve fiber layer.27 Management options include an inner wall retinectomy or careful dissection of the posterior hyaloid. Scleral buckle alone is not indicated in this pathology, due to the presence of foveal schisis, but can be combined with vitrectomy when RRD develops due to peripheral retinal breaks.2,4

    Pediatric patients with choroidal coloboma can also develop RRD, due to retinal breaks that develop at the borders of the coloboma. Their repair is a surgical challenge, especially if the optic nerve is involved and if there are associated ocular abnormalities, including microphthalmia, cataract, and lens coloboma.2,15 Due to the absence of choroid and RPE, these breaks cannot be treated with laser retinopexy, and due to the presence of posterior breaks scleral buckle is not helpful in these patients.2,4 The recommended approach for these cases is primary vitrectomy, internal drainage of subretinal fluid, endotamponade with gas, endolaser along the edges of the coloboma posterior to the equator, and transscleral cryotherapy anterior to the equator to isolate the retina from the coloboma.2,15

    Exudative Retinal Detachment

    Coats Disease

    Coats disease is a bilateral nonhereditary condition characterized by retinal telangiectasia, exudation, retinal macrocysts and hemorrhage, optic disc and retinal neovascularization, and exudative retinal detachment.15,28 Shields and co-authors further classified the disease into 5 stages.28 (Table 1) Recently, Daruich and colleagues suggested subdividing stage 2B into 2B1 without subfoveal nodule and stage 2B2 with subfoveal nodule.29

    Surgical repair of RD in combination with laser photocoagulation and cryotherapy is recommended in advanced stages (Stage 3 or 4), in which laser or cryotherapy alone would not be enough.15 Currently, no surgical gold standard treatment exists, but many authors have described several surgical approaches. Adam and coauthors recommended a “less is more” surgical approach: Patients are treated with minimally invasive surgery that includes placement of infusion, external drainage of subretinal fluid (SRF) via a posterior sclerotomy, and cryopexy of retinal telangiectasias.30 On the other hand, other authors have recommended more invasive techniques that include vitrectomy, retinotomy, and use of silicone oil or gas as tamponade in addition to scleral buckle in advanced disease.31,32 Despite the recent use of intravitreal triamcinolone and anti-vascular endothelial growth factor (anti-VEGF) agents as adjuvant therapy to improve visual acuity and resorption of SRF and exudate, stage 3 disease often progresses to stages 4 and 5, and the aim of treatment in advanced end-stage Coats diseases is to maximize useful vision and to preserve the cosmesis and the comfort of the patient.15

    Table 1. Classification of Coats disease
    Stage 1 Retinal telangiectasia only
    Stage 2 Telangiectasia and exudation
    2A Extrafoveal exudation
    2B Foveal exudation
    Stage 3 Exudative RD
    3A Subtotal RD
    3B Total RD
    Stage 4 Total RD and glaucoma
    Stage 5 Advanced end-stage disease

    Tractional Retinal Detachment

    Persistent Fetal Vasculature

    Persistent fetal vasculature (PFV) can present as an isolated finding or in association with a systemic disease such as Norrie disease or Walker-Warburg syndrome.15 Indications for intraocular surgery include opaque media, progressive glaucoma secondary to angle closure, presence of vitreoretinal traction and RRD, or progressive tractional retinal detachment (TRD).15 The surgery of choice includes vitrectomy with earlier transection of the persistent hyaloid stalk, with or without lensectomy, and the release of traction on the ciliary body by excision of the retrolental tissue and removal of epiretinal membranes in the posterior pole.15 Control of bleeding with diathermy while transecting the proximal side of the stalk is crucial to maintaining a view in order to complete the procedure.15

    Bosjolie and Ferrone suggested early vitrectomy in children (13 months of age or younger) with TRD and reported reattachment of the retina with reversal of retinal dragging, showing that 60% of their patients retained functional vision (20/800 or better) at the end of follow-up.33 These results show the importance of performing early vitrectomy in children to maximize visual rehabilitation by early and aggressive management of amblyopia.

    Retinopathy of Prematurity

    Retinopathy of prematurity (ROP) is a vasoproliferative disorder of the retina in preterm infants that is characterized by abnormal vascularization.34-35 Treatment in early stages with laser or anti-VEGF is required, but retinal detachment can occur in up to 25% of previously treated eyes, causing profound visual loss.36

    A variety of techniques for managing ROP-related retinal detachments, including observation, scleral buckle (SB) alone, lens-sparing vitrectomy (LSV) alone, LSV plus SB, and vitrectomy with lensectomy have been published.37-44 But there is not enough randomized prospective information to guide our management. The treatment of choice would depend on the stage (4A, 4B, or 5) of the disease and the presence of tractional membranes toward the posterior capsule of the lens.

    Since Maguire described a 2-port LSV, many authors have adopted this technique to treat babies with stage 4A or 4B retinal detachments.40-43 This technique involves a standard conjunctival opening, with placement of sclerotomies 0.5 mm-1 mm from the limbus, through the iris root.43 Regardless of previous recommendations on not placing an infusion cannula, due to the risk of lens injury by direct mechanical contact or by hydrostatic forces from the infusion stream, several authors have adopted a 3-port LSV with good results as well.37-44

    Authors agree that stage 4A and 4B retinal detachments should be treated by performing a core vitrectomy and addressing the vitreous in sequential planes: ridge to anterior hyaloid, ridge to ridge, ridge to periphery, ridge to nerve, ridge to vitreous base, and circumferential along the ridge.37,40 Most of the dissection can be done with the vitrectomy probe, but intraocular forceps or scissors can be occasionally needed.37,40 The posterior hyaloid is often really adherent to the retina, and its removal is not recommended.37 At the end of the procedure, a good peripheral examination with scleral depression should be done to rule out the presence of any retinal breaks.37 If no breaks are identified, partial fluid-air exchange has been recommended to prevent incarceration of the retina in the sclerotomies site.42 If retinal breaks are noted, then a total fluid-air exchange with the use of tamponade (silicone oil or gas) should be performed.37

    SB alone has also been reported for the treatment of stage 4A and 4B retinal detachments, in an attempt to prevent intraocular surgery in these patients. Nonetheless, SB has disadvantages such as:

    1. Membranes may continue to proliferate with increase in traction,
    2. A second procedure is needed to remove or segment the buckle in order to allow the eye to grow,
    3. Chow and coauthors reported an induced anisometropia of -9.5 D after SB placement and a hyperopic shift of 5.5 D after division of the SB, probably affecting normal visual development,
    4. Anatomic and functional success rate in experienced surgeons is lower compared to LSV (60%-75% vs 85%-97%).36,37,39-42,45-,47

    SB placement, in addition to cryotherapy to the avascular retina, for stage 3+ in zone 1 ROP, as a preventive measure for progression to retinal detachment has been reported. However, there is not enough prospective data to support its use.39

    The appropriate moment for retinal surgery in these patients is when the vascular activity has subsided, and a retinal detachment is present.40 Treating an eye with high vascular activity can lead to worse postoperative inflammation and vitreous hemorrhage. Anti-VEGF therapy has been used to reduce vascular activity, as previously published by Cernichiaro-Espinosa and coauthors, but one must always be aware that anti-VEGF therapy can increase contraction of fibrovascular membranes and result in the need for vitrectomy.44,48

    In stage 5 ROP, vitrectomy is the standard of care. Lensectomy can be added to the procedure if retrolental fibroplasia is present.35,48 In cases with retrolental fibroplasia, corneal side port incisions should be considered, with the use of an anterior chamber maintainer, in order to prevent entry to the subretinal space and formation of iatrogenic breaks.35 The anterior capsule has to be removed, followed by careful dissection of the posterior capsule and retrolental membrane, to expose the underlying retina.35 Dissection should be continued until the posterior funnel is opened up, with posterior removal of the fibrous stalk from the optic nerve head.35 Surgery for stage 5 ROP is challenging and functional, and anatomic results are generally poor, with a reattachment rate ranging from 13% to 50% and VA limited to LP or NLP.35,45,46,48 But leaving these eyes untreated will lead to permanent loss of visual function.35,48

    Treatment of ROP-related retinal detachment presents special considerations, due to the fact that it progresses during a critical period of visual development, and this increases the need for early intervention.38,41 Retina surgeons should be aware of all the potential amblyogenic factors.47 Beside the risk of organic amblyopia that is addressed through retinal reattachment, these patients are at risk of refractive and strabismic amblyopia, and coordination with a pediatric ophthalmologist is necessary to institute early treatment and to obtain better visual outcomes.47

    Combined Retinal Detachment

    Familial Exudative Vitreoretinopathy

    Familial exudative vitreoretinopathy (FEVR) is an inherited bilateral vitreoretinopathy characterized by avascular peripheral retina at presentation.2,20 This leads to peripheral retinal neovascularization, formation of fibrovascular membranes that cause vitreoretinal traction with dragging, falciform folds, RD, and lipid exudation.2,15,20

    The indication for surgical treatment is determined by the type of retinal pathology and the status of the fellow eye.20,49 In an eye with chronic and total RD where the fellow eye is normal, observation is recommended. In a patient with bilateral RD, early surgery is warranted in one or both eyes.20 The surgical method recommended when a combined RD is encountered is combined scleral buckle and vitrectomy to relieve tractional forces, and laser ablation of peripheral avascular retina.2,15,20,49,50 When obvious fibrovascular tissue is adherent to the posterior capsule, lensectomy should be added to the surgical planning.2,15,20,49 Scleral buckling procedures, besides relieving traction, also reduce the activity of fibrovascular proliferation by reducing vascular endothelial cell stretching, thus inhibiting VEGF secretion.49 Hyaloidal sheets from the adherent and contractile vitreous can be difficult to visualize intraoperatively, and multilayer posterior hyaloid may be present. Careful attention should be given in removing all the hyaloid sheets to prevent further contraction and an unsuccessful outcome.15,20 A bimanual technique with vitreous cutter and forceps is preferred in dissecting all the vitreous membranes. Dissection in the peripheral avascular area may be difficult due to strong vitreoretinal adhesions, and iatrogenic retinal breaks may occur easily.15

    In patients with stage 3 FEVR, where the macula is attached and good vision is present with a localized RD in the periphery, laser treatment is recommended, with further observation. However, in patients with stage 4 FEVR where retinal folds are present, surgical intervention is warranted.20 Unless thin folds without any vascular activity are noted, observation can be an option as well, because the risk of iatrogenic retinal breaks and no improvement in vision has been noted to be high, even if the retinal folds are released.20 Early vitrectomy before macular dragging occurs is preferred and results in better anatomical and functional outcomes.50

    Conclusions

    A thorough history that includes birth history, medical history, family history, any history of trauma, or ocular surgery is important in every patient to determine the onset of symptoms.4 An evaluation of the patient’s appearance with a complete ophthalmologic evaluation should be made in any pediatric patient.4 Several attempts should be made to assess visual acuity in each eye. Intraocular pressure, ocular alignment, refractive status, anterior segment, and posterior segment examination all should be performed.4 Presence of hypotony, strabismus, nystagmus, cataract, choroidal detachment, and uveitis are all suggestive of a long-standing retinal detachment and poor prognostic indicators.4

    Visual outcomes in pediatric patients after RD repair are generally poorer, even when anatomic success has been achieved, due to associated hereditary disorders with systemic comorbidities, and the presence of chronic detachments, macular involvement, and presence of PVR.5,12 Visual potential of an eye with a successfully attached retina can be further compromised due to amblyopia induced by strabismus or refractive changes, as a result of aphakia, silicone oil tamponade, or scleral buckle.4,5,12 A lengthy and detailed conversation with the family should always be held, and this should always include anatomic and visual prognosis, as well as the likelihood of multiple surgeries and comprehension of the extent of care required.4

    Vitreoretinal surgeons should always work together with pediatric ophthalmologists, to start early amblyopia therapy and prompt prescription of glasses or contact lenses in children that undergo any RD repair. A multidisciplinary approach and close follow-up in pediatric patients that present with retinal pathology will enhance final surgical outcomes.

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