|Year : 2018 | Volume
| Issue : 2 | Page : 87-93
Pediatric retinal detachment: An overview
Consultant, Smt.Kanuri Santhamma Centre for Vitreo retinal Diseases, LV Prasad Eye Institute, Hyderabad, Telangana, India
|Date of Web Publication||28-Aug-2018|
LV Prasad Eye Institute, Kallam Anji Reddy Campus, LV Prasad Marg, Banjara Hills, Hyderabad - 500 034, Telangana
Source of Support: None, Conflict of Interest: None
Retinal detachment is rare in children. It is different from adults in terms of its presentation, etiologies, and management. It remains a challenge to manage these cases due to delayed presentation and mostly, bilateral involvement. The various etiologies, their management and the surgical tips that is specific to each type of retinal detachment is discussed here.
Keywords: Coats' disease, familial exudative vitreoretinopathy, pars plana vitrectomy, retinoschisis, rhegmatogenous
|How to cite this article:|
Balakrishnan D. Pediatric retinal detachment: An overview. Kerala J Ophthalmol 2018;30:87-93
| Introduction|| |
Retinal detachment (RD) in children is different from adults regarding its etiology, presentation, management, and prognosis. RD in children is less common than in adults (3.2%–6.2%). The management is challenging, as it requires a team consisting of parents, pediatric ophthalmologist, retina specialist, anesthetist, pediatrician, and visual rehabilitation team. This article gives an overview of pediatric RD, its etiology, and management. The discussion about retinopathy of prematurity (ROP) and tumors is beyond the scope of this article.
| General Considerations in Pediatric Age Group|| |
Birth history including birth weight, gestational age, postnatal course is important, especially in diseases ROP and familial exudative vitreo retinopathy (FEVR). History of trauma can be misleading in children as parents may attribute trauma, for vision loss or bleeding in an eye with an already preexisting pathology such as retinoblastoma, retinoschisis, and familial exudative vitreoretinopathy (FEVR). Hence, it is very important to rule out any life-threatening conditions such as retinoblastoma or metastasis by correlating the history with clinical findings and investigations before any surgical intervention. Family history is important, especially in diseases, such as FEVR and retinoblastoma, where screening of family members is indicated.
The symptoms and ocular features depend on the etiology and time of presentation. The common symptoms are reduced vision, poor fixation, leukocoria, strabismus, pain, and redness. A change in visual behavior, an irritable nature, or less mobility may be the presenting symptom in children who present with bilateral disease. In many a cases the visual problem is noticed, when the better eye is affected, or any sequelae or secondary complications such as leukocoria or squint is present, as unilateral disease may go unnoticed. The clinical features may vary from a normal anterior segment, corneal opacity, shallow anterior chamber, cataract, RD, or neovascular glaucoma. As per literature, 0%–26% of children have bilateral involvement, as the pathology may be present in fellow eye also.
B-scan ultrasonography is a useful investigation in children with leukocoria and trauma. It is useful in differentiating various conditions such as retinoblastoma, Persistent fetal vasculature (PFV), RD due to ROP and FEVR, Coats' disease, trauma, etc. A widefield retinal imaging, including color fundus photography, fluorescein angiography (FA) and autofluorescence, is useful in managing pediatric retinal diseases. RetCam and Optos are the two available imaging systems. This is used to screen, diagnose and monitor diseases such as ROP and FEVR. Optical coherence tomography (OCT) and electroretinogram (ERG) are used in diagnosing diseases such as X-linked retinoschisis and macular edema in newborn.
A proper evaluation of both eyes mostly under anesthesia is mandatory. The kind of surgical intervention depends on the type of RD and the prognosis. A proper refraction and appropriate glasses with amblyopia therapy are mandatory for success of those eyes with good visual potential. Parents cooperation, involvement, awareness about the nature of disease, and need for visual rehabilitation are crucial for a successful outcome.
The most common type of RD in children is rhegmatogenous RD. The other types being exudative, tractional and combined RD.
The major causes of RD in children are:
- Rhegmatogenous RD
- Intraocular surgery
- RD associated with diseases such as Marfan syndrome, Sticklers syndrome
- High myopia (nonsyndromic)
- Idiopathic or spontaneous dialysis
- Others-combined mechanism rhegmatogenous RDs (RRD) associated with any of the conditions listed below.
- Congenital anomalies
- Morning glory syndrome
- Optic disc pit
- Vitreoretinal dystrophy
- X-linked retinoschisis
- Tractional RD (TRD)
- Retinal vascular or vasoproliferative diseases
- FEVR (ROP and FEVR can have exudative RD also)
- Incontinentia pigmenti (IP)
- Developmental anomalies
- Persistent fetal vasculature (PFV) previously called as Persistent hyperplastic primary vitreous (PHPV) (PFV can present with combined RRD also)
- Exudative RD
- Coats' disease
- Choroidal hemangioma.
(Systemic causes of exudative RD are beyond the scope of this article).
| Rhegmatogenous Retinal Detachment|| |
Pediatric rhegmatogenous RDs (PRRD) are uncommon (3%–12%)., Most of the studies have shown that males are affected (70%–80%) more, while others did not show any gender difference. The mean age at presentation is between 9 and 12 years. The management is challenging due to various factors such as anatomic variations, delayed presentation, proliferative vitreoretinopathy (PVR), macula-off detachments with poor presenting visual acuity. These cases should be treated aggressively and with great care as, up to 40% of eyes, which were treated, had better visual outcomes than the fellow eye.
The most common presenting symptom is decrease in visual acuity. The duration of symptoms vary from days to years (mean 30 days). Figvas et al. reported a mean of 52 days from the initial symptom to the time of presentation. Other features are squint, leukocoria, cataract, hypotony, phthisis, uveitis and rarely with pain and redness secondary to neovascular glaucoma. About 12%–26% of cases had bilateral involvement at presentation and 70%–90% had predisposing pathologies in the fellow eye. Lattice degeneration is the most common fellow eye abnormality. Retinal hole or horse shoe tear are usually associated with PRRD and retinal dialyses (20%–42%) are seen in traumatic cases and some idiopathic cases. Giant retinal tear is seen mostly associated with syndromes such as Stickler, where the risk of second eye involvement is also high. Delayed presentation with PVR (13%–60%) and macula-off detachment was noted in 26%–74% of children.,,
The most common cause of PRRD is trauma (26%–90%) followed by myopia (6%–34%) and intraocular surgery (34%).,, The other causes are atopic dermatitis, combined RD due to retinal break-in cases associated with developmental or vasoproliferative diseases such as coloboma, FEVR, ROP, or PFV.
Trauma is the main cause in children above 10 years of age while developmental or congenital causes were more in those <10 years of age. The chances of developing RD after trauma in children were found to be 23%–51%. The visual outcomes are better in closed globe injuries than open globe as the PVR changes are more in latter. Other ocular findings such as hyphema, cataract, glaucoma, optic neuropathy, choroidal rupture, vitreous hemorrhage, and uveitis may be present in 50% of cases. The visual outcomes are better in traumatic RD when compared to TRD as they present early with a history of trauma.
Idiopathic RD accounts for 20% of cases. Retinal dialysis was seen in 77% of idiopathic RRDs in children. This idiopathic retinal dialysis is usually seen in inferotemporal quadrant and is often subclinical. This can have bilateral presentation and the fellow eye also need to be screened. The mean age of presentation is usually 20 years. A genetic predisposition was also suggested and hence recommended family screening also.
Myopia may be syndromic or nonsyndromic. The most common associated syndromes are Stickler and Marfan syndrome. Myopia, empty vitreous, radial lattices, and RD are the ocular features of Stickler syndrome. The type 1 Stickler syndrome is more common, with defect in type II collagen and associated with deafness, orofacial features (flattened facial features) and arthritis. Type 2 is due to defects of type XI collagen and is less common. Marfan syndrome is an autosomal dominant disease that occurs due to mutation in fibrillin 1 gene. Various cardiac, skeletal and ocular abnormalities may be present. Myopia, ectopia lentis, lattice degeneration and RD are the ocular features. RD occurs in 5%–11% of cases.
Congenital anomalies are the major cause of RD in children <10 years. A higher prevalence of up to 53% is seen in children in western countries with mostly bilateral involvement. Coloboma results from faulty closure of the embryonal fissure. The incidence of RD is almost 40% in these eyes. The RD may occur due to a break with in the hypoplastic tissue in the coloboma or in the normal retina. The detection of break-in coloboma is difficult many a times due to the lack of contrast and associated nystagmus.
Morning glory optic disc anomaly is a congenital disc anomaly. Serous RD occur in 26%–38% of eyes with morning glory syndrome. Other associated abnormalities were ipsilateral intracranial vasculature abnormalities and transsphenoidal form of basal encephalocele.
Optic disc pit is a gray or white, round or oval depression seen in the optic disc. The common location is temporal and inferotemporal. Serous RD is seen in 25%–75% of cases. It is seen bilaterally in 15% of cases.
X-linked retinoschisis is a retinal degeneration that affects males early in the life. There occurs mutation in the RS 1 gene that codes for a protein retinoschisin that strongly binds to cell surfaces, synthesized primarily by photoreceptors, resulting in splitting of the neural retinal layers. The disease is characterized by macular spoke-wheel schisis (98%–100%) and peripheral schisis (in 50%). The clinical features are outer retinal breaks, inner retinal breaks, and vitreous veils (bare retinal vessels) [Figure 1]. RD and vitreous hemorrhage are the two vision-threatening complications of this disease. The RD occurs in the presence of both inner and outer retinal breaks. ERG and OCT are useful diagnostic tests. ERG shows reduced amplitude b wave in scotopic response, described as “electronegative response”. OCT shows schisis in various retinal layers including deeper retinal layers, contrary to the earlier thought.
|Figure 1: Fundus photo of retinoshisis with outer retinal holes with curled up margins with inner schitic wall with break|
Click here to view
Management of pediatric rhegmatogenous retinal detachments
The management of PRRD is challenging owing to its specific etiology, anatomic variations, and associated PVR changes. The two surgical options are scleral buckle (SB) with or without encircling band and vitrectomy or a combination of both. The preferred surgery in PRRD is SB till to date even in cases with PVR up to Grade C. The advantages of SB are no vitreous manipulation as PVD is usually absent in many of these cases, less risk of cataract progression or secondary glaucoma, negate the need for second surgery such as silicone oil removal and postoperative positioning that is difficult to maintain in children. Vitrectomy with or without encirclage is done in RD with more than Grade C PVR. Silicone oil is the preferred tamponade compared to gas as it has less chance of intraocular pressure spike in the postoperative period and the need for long tamponade, especially in complex cases of FEVR or trauma.
The management of RD in coloboma depends on the location of the break. RD with break in the normal retina is managed as any other RRD either by scleral buckling or vitrectomy. It is difficult to identify breaks in the intercalary membrane (ICM) due to lack of contrast. The ICM breaks should be identified intraoperatively using high magnification. The recommended surgical method is vitrectomy with fluid-air exchange (FAE) to settle the retina through the ICM break if present and a relaxing incision in sagittal plane is recommended if the ICM remain taut after FAE. Three to four rows of endolaser along the edge of the coloboma, sparing the disc margin should be done. Silicone oil is the preferred tamponade in coloboma RD. The success rate after surgical intervention varied from 57% to 87%.
RD associated with X-linked retinoschisis (RS) is very rare and occurs in about 10%–15% of cases. The surgical indications in RS are vitreous hemorrhage and RD. SB or pars plana vitrectomy (PPV) may be done in these cases, but PPV is the preferred surgical technique. The role of prophylactic barrage laser is controversial in RS as the primary pathology can occur in any location of retina. The surgical challenge is to induce posterior vitreous detachment over the inner schitic wall. The excision of inner wall should be done in those cases to settle the RD and to prevent PVR. Many of the successful cases maintain a visual acuity of 20/100–20/400.
In PRRD, the mean number of surgical interventions needed in children varied from 1.2 to 2.2, being up to 5 procedures per patient., The anatomical success ranged from 62% to 80%.,, Fivgas and Capone reported 80% success in myopic RD, but only 40% in those who had undergone prior surgery. He also noticed that the mean number of surgeries were more in myopic eyes (2.8) than in those with prior intraocular surgery (1.6). The eyes with myopia were found to have better visual and anatomical outcomes than in those with developmental or congenital etiologies. The visual outcomes were less in recent studies, especially in those with silicone oil injection, when compared to the previous studies mostly because of poor prognosis eyes being taken up for vitrectomy with the advances in the surgical techniques. The outcomes in different studies are not comparable due to the varying inclusion criteria. The visual outcomes varied from 50% achieving >20/400 to 41% having >20/800., The major prognostic factors noted were presenting visual acuity and presence of PVR. Weinberg et al. reported the predictive factors for poor visual outcomes as poor preoperative vision (undetermined or light perception only), macula-off status, the need for vitrectomy, and use of silicone oil.
| Tractional Retinal Detachment|| |
The main causes of TRD in children are vasoproliferative diseases such as ROP, FEVR, and IP; developmental disease such as PFV and trauma. Rarely infective or inflammatory diseases such as toxocara and pars planitis can cause peripheral tractional detachment and hypotony. Non-ROP causes of pediatric TRD will be discussed here.
| Familial Exudative Vitreo Retinopathy|| |
FEVR is a retinal vascular disease that is mostly inherited as autosomal dominant but can be recessive or X-linked. The disease shows variable expression and incomplete penetrance. It is a lifelong disease characterized by avascular peripheral retina with periods of relapse. A family history is important along with screening of family members. The disease is classified into five stages.
- Stage 1: avascular retina without extraretinal vessels
- Stage 2: avascular retina with extraretinal vessels
- No exudate
- With exudate
Stage 3: partial RD-fovea spared
- No exudate
- With exudate
Stage 4: partial RD-fovea involved
- No exudates
- With exudate
Stage 5: Total RD
- No exudate
- With exudate.
The clinical features depend on the stage at presentation, varying from peripheral avascular retina, neovascularization (NVE), subretinal exudates, exudative detachment, TRD or combined RRD, and vitreous hemorrhage [Figure 2]. RD may occur in 26%–64% of affected individuals. The wide field fundus FA is being recently used in the diagnosis and follow-up of these patients.
|Figure 2: Fundus photo of familial exudative vitreoretinopathy showing disc and macular drag with temporal peripheral straightening of vessels with tractional retinal detachment and subretinal exudates|
Click here to view
The management of these patients depends on the stage of the disease. In doubtful cases, FA is useful in detecting NVEs. The prophylactic laser treatment of avascular retina is indicated in cases of leaking NVE, subretinal exudates, or exudative detachment. In view of the higher incidence of RD in older population, prophylactic barrage laser treatment of atrophic retinal holes and lattices in avascular retina is recommended. The surgical management depends on the type of detachment and associated PVR changes and location of break. The SB would be sufficient when the break is in periphery with no traction and disc drag. In cases, which need vitrectomy, a broad buckle is recommended as the peripheral retina is usually thin with firm vitreo retinal adhesion and the chances of iatrogenic retinal breaks are high. A bimanual surgery will help to do controlled peripheral vitreous dissection with less risk of iatrogenic breaks. These patients need lifelong follow-up as the disease can recur.
| Persistent Fetal Vasculature/persistent Hyperplastic Primary Vitreous|| |
PFV or PHPV is a congenital anomaly due to incomplete regression of primary vitreous or fetal hyaloid vasculature. It is usually unilateral (90%). The severity can range from Mittendorf dot to severe combined form involving both anterior and posterior segment. The types of PHPV depending on the extent of involvement are as follows:
- Anterior PHPV-involves anterior segment and the lens characterized by retrolental membrane, cataract, elongated ciliary process, and glaucoma with a normal posterior segment
- Posterior PHPV-characterized by microphthalmos, vitreous stalk, disc and macula hypoplasia, retinal fold, TRD, and hypopigmentation of macula
- Combined – involves both the anterior and posterior segment.
The anterior retina or ora serrata may be inserted onto the ciliary body in cases of posterior or combined PFV.
B scan ultrasonography shows a short-axial length and a posterior stalk along with its anterior attachment. An immersion B scan or ultrasound biomicroscopy is useful to assess the retrolental membrane and its attachment to the posterior capsular.
The management of PFV is either observation or surgery. Observation may be done in cases with an isolated hyaloid artery if lenticular opacity or membrane is not in visual axis or no anatomic worsening on follow-up. The type of surgical intervention in PFV depends on the extent of anterior and posterior segment involvement. The purpose of surgical intervention in these patients is to preserve globe and prevent complications such as phthisis, secondary glaucoma, provide a cosmetically acceptable black pupil than a white pupil due to cataract or retrolental membrane, and also to provide useful vision with proper amblyopia therapy in eyes with good potential. Early surgical intervention is recommended for anterior PFV-cataract extraction with intraocular lens (IOL) placement, cauterization of vascular stalk, and anterior vitrectomy. Almost 30% of these cases may require a membranectomy later. Appropriate postoperative refraction and intense amblyopia therapy are likely to provide better visual outcomes in these cases.
One of the predicting factors for the visual outcome is the age at presentation, wherein babies operated within 1–3 months of age improved to visual acuity of >20/200. The placement of IOL in these cases is shown to have better visual outcomes and less incidence of glaucoma. The visual prognosis is better in anterior PHPV with intervention, while in the posterior and combined forms, the prognosis depends on the associated optic disc or macular abnormalities.
| Incontinentia Pigmenti|| |
IP or Bloch–Sulzberger syndrome is a rare X-linked dominant genodermatosis that is usually seen in females, as it is lethal in males. It is a multisystem ectodermal disease that affects skin, teeth, eyes, and central nervous system (CNS). Even though the skin lesions are diagnostic their absence does not exclude it. The diagnostic criteria were described by Landy and Donni. The skin lesions occur in four stages from vesicles to hyperpigmentation and scarring. The dental lesions are mainly hypodontia, delayed eruption, and malformation such as conical teeth. The CNS manifestations are epilepsy, motor, and mental retardation and rarely microcephaly. The ocular manifestations are peripheral avascular retina with NVE, vasculitis such as presentation and RD. FA findings are avascular peripheral retina, subtle neovascularization and irregular foveal avascular zone. B-scan ultrasound is useful to differentiate it from retinoblastoma, when present with vitreous hemorrhage, cataract, and/or RD.
All children diagnosed with IP should be screened within 1 or 2 weeks of birth as the disease may progress rapidly earlier in life. A prophylactic ablative therapy to the avascular retina with NVE is recommended. Vitreoretinal surgery is needed in advanced disease; however, the prognosis is poor like in Stage V ROP. As these patients with ocular involvement are likely to have CNS involvement, they should be referred for a neurological evaluation.
| Exudative Retinal Detachment|| |
Coats disease is a congenital nonhereditary sporadic retinal vascular disease characterized by telangiectasia, subretinal, and intraretinal exudation and exudative RD. The disease may be complicated by vitreous hemorrhage, neovascular glaucoma, or phthisis bulbi. It occurs most commonly unilaterally in young males. The disease has been classified into five stages.
- Stage 1: Retinal telangiectasia only
- Stage 2: Telangiectasia and exudation
- Extrafoveal exudation
- Foveal exudation
Stage 3: Exudative RD
- Subtotal detachment
- Total RD
- Stage 4: Total RD and glaucoma
- Stage 5: Advanced end-stage disease.
The management and prognosis depend on the stage of disease at presentation. The aim of treatment is to preserve the vision and the globe. Those eyes with Stage 1 disease or asymptomatic advanced disease may be observed. Laser photocoagulation or cryotherapy is indicated in Stage 2 diseases with or without intravitreal antivascular endothelial growth factors or steroids in cases of macular edema. The laser should be done to the telangiectasia and also to the avascular peripheral retina using neodymium-doped yttrium aluminum garnet double frequency laser. Cryotherapy is indicated in cases with shallow SRF and peripheral lesions using double freeze and thaw technique. The laser and cryotherapy may require multiple sessions with an interval of 3–4 months. In bullous exudative RDs, an external drainage of subretinal fluid should be done before the laser or cryotherapy. Internal drainage retinotomies should be avoided, as these are primarily exudative RD. A combined pars plana vitrectomy may be required in cases with associated PVR. A combination treatment is tried in Stage 3 and advanced disease. Enucleation may be considered in painful blind eyes.
| Conclusion|| |
The management of pediatric RDs still remains a challenge due to its varied etiology, delayed presentation, and the need for multidisciplinary intervention. However, a good anatomical and or visual outcome can be achieved by appropriate screening, surgical intervention, and visual rehabilitation.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]