Clinical Features and Surgical Outcome of Pediatric Rhegmatogenous Retinal Detachment

Med. J. Cairo Univ., Vol. 77, No. 2, September: 33-38, 2009 www.medicaljournalofcairouniversity.com Clinical Features and Surgical Outcome of Pediatric Rhegmatogenous Retinal Detachment ASSER A.E. ABD EL-MEGUID, M.D.; AHMAD S. EL-ANTABLY, M.D.; REHAB R. KASSEM, M.D. and AHMAD M. KAMAL, M.D. The Department of Ophthalmology, Faculty of Medicine, Cairo University. Abstract Purpose: To report the etiology, clinical characteristics and surgical outcome in a series of children presenting with rhegmatogenous retinal detachment (RRD) to a tertiary referral center in Egypt. Methods: A retrospective analysis of, a non comparative interventional case series of aged between 2 and 8 years who were operated upon over the period interval between June 2005 to January 2008 for primary RRD and completed a follow-up period of 3 months. Result: The study included 30 eyes of 27 8 males and 9 females with an average age at presentation of 0.37±3.89 years with a range of 4 to 8 years. They were followed up for a time interval of 7 to 26 months with an average of.3 ±5.6 months. Retinal lesions as the primary cause of the retinal detachment has been diagnosed in 53%, postsurgical retinal detachment in 27% while post traumatic in 0%. Late presentation was a common feature and bilateral retinal detachment at presentation was present in 9 cases only 3 of them were operable. Standard three port vitrectomy with silicon oil endotamponade was performed in 29 eyes. There was a rate of retinal reattachment of 94% with an average procedures of 2.4. All successfully operated with a reattached retina regained hand movement or better visual acuity. Conclusion: The retinal reattachment rate in pediatric RRD is encouraging, however it is adversely affected by the presence of PVR making the average no of surgeries per eye higher than in adult cases. Visual improvement is limited by the delayed presentation, but in the majority of cases visual acuity greater than or equal to counting fingers was achieved allowing for ambulatory vision postoperatively. Better health care system may allow for earlier detection of RD with the result of better visual outcome. Key Words: Retinal detachment Pediatric retinal detachment Pediatric screening program. Introduction THE incidence of Rhegmatogenous retinal detachment (RRD) in children comprises a small fraction of all cases of RD, different reports had reported an incidence rate between 2%-6% [-4]. The clinical features at presentation are different from those of adults due to delay in diagnosis caused by poor subjective complaints [5,6]. Accordingly there is an associated various complicating entities in the form of proliferative vitreoretinopathy (PVR) and worse visual acuity at time of presentation [7]. These factors poses a difficult challenge to the surgeons and is reflected on the suboptimal visual gain postoperatively. This is more pronounced in developing countries where modern medical facilities and trained personnel are not widely available [8]. We describe the clinical characteristics, etiology and surgical outcome of pediatric RRD, age 2-8 years, of presenting to tertiary referring center in Egypt. Methods Included in this retrospective non comparative study, is an interventional case series of aged between 2 and 8 years who presented to one of two centers, Abouelrish Cairo university hospital or Nasr City national insurance hospital, and were operated upon during the interval between June 2005 and January 2008, for primary rhegmatogenous retinal detachment. Patients had been operated upon by the first author, not necessarily at place of presentation, and had been examined and followed by the same working team (the authors). Inclusion criteria comprised with pediatric retinal detachment who completed 3 months followup after the last surgery. Patients with cicatricial changes due to retinopathy of prematurity and those with non-rhegmatogenous retinal detachment were excluded from the study. Anatomical success was defined as persisting retinal attachment at last follow-up (at least 3 months postoperatively) in the absence of silicone oil. 33

34 Clinical Features & Surgical Outcome of Pediatric Retinal Detachment Results The study included 30 eyes of 27 8 males and 9 females with an average age at presentation of 0.37±3.89 years with a range of 4 to 8 years. They were followed up for a time interval of 7 to 26 months with an average of.3 ±5.6 months. The most common complain at presentation was decrease visual acuity, 2 (Table Fig. ). Of those two were referred from the pediatrician and one from the school doctor. In eight retinal detachment was diagnosed after a surgical procedure. Four after a cataract surgery, two after a glaucoma procedure, one after scleral fixation of an intraocular lens and one after a previous vitrectomy due to vitreous hemorrhage. Posttraumatic retinal detachment was diagnosed in three. Three presented with complicated cataract and one patient with squint in the affected eye. Of the 27 nine of them had retinal detachment in the other eye. In six of them the detachment (in the non presenting eye) was inoperable while in three it was early and was operated upon. The visual acuity in the presenting eye ranged from light perception to 5/60 with the majority having hand motion or counting finger. The vision in the other eye ranged from 6/24 to 6/6 when there was no associated detachment. In the six with inoperable detachment in the other eye the vision was no PL in 4 and PL in 2. The three operable cases had visual acuity of 6/24 in 2 and 6/60 in one. In three the visual acuity was not recorded. There were various retinal lesions recorded as the cause of retinal detachment (Table 2). Traction retinal tears including horse shoe tears were reported in 2 cases, atrophic retinal tears associates with retinal degenerations were reported in 6 cases, giant retinal tear in 4 cases and retinal dialysis in 5 cases while in 3 cases the retinal lesion was not recorded. There was no clear documentation of the duration of the retinal detachment at time of presentation. The macula was off in all cases in the presenting eyes. In the three with operable RD in the other eye the macula was off in one of them. In all cases there was a varying degree of associated PVR. In twenty nine eyes the procedure was standard three port vitrectomy with silicon oil injection. Silicon oil was removed after 3 to 6 months. An additional encircling element, no 240 (2.5 mm width), was applied to 9 with clear lens. In one patient a buckling procedure was performed. In cases with lens opacities there was primary lens removal during the vitrectomy procedure. If the lens was totally opaque preventing the proper assessment of retinal condition (3 ), a lensectomy was done leaving the anterior capsule to support a posterior chamber intraocular lens (IOL) which was implanted at the end of the surgery. In case of lens opacity which did not hinder the retinal evaluation preoperatively, a lens removal with insertion of foldable IOL was done at the start of surgery (2 ). In 4 the lens opacified after vitrectomy procedure and was removed at the time of silicon removal. At the end of follow-up 28 (93.3%) eyes were attached, of them two with silicon oil. In those two silicon oil was not removed due to associated hypotony. In the two other eyes with failure of retinal reattachment due to severe reproliferation in spite of multiple surgeries (4 in one of the eyes), silicon was left to maintain the shape of the eye. Accordingly the final success rate (ie eyes with attached retina for more than 3 months without silicon) was 26 eyes (86.6%). The average number of surgeries needed was 2.4 with a range of one to 5. Silicon oil removal was considered a separate operation. Sixteen needed only one vitrectomy operation. All successfully operated had hand movement visual acuity or better Figs. (2,3). Of the 26 successfully operated 7 had visual improvement, in 5 the visual acuity stayed the same and in two the visual acuity was not recorded. In two there was decrease in visual acuity, this was attributed in one patient due to intraoperative iatrogenic macular hole and in the other patient to the postoperative increase in the intraocular pressure (IOP). Complications encountered (Table 3) included hypotony which prevented the removal of silicon oil in two. Increase of the IOP was controlled with antiglaucoma medication and with early silicon removal. This was encountered in 6, of them 2 were known to be glaucomatous and silicon removal did not help in decreasing the IOP. Band keratopathy was encountered in one patient due to silicon oil in the anterior chamber. Inraoperative complications included crystalline lens injury in one eye and iatrogenic macular hole in another one patient.

Asser A.E. Abd El-Meguid, et al. 35 4 2 Number of 0 8 6 4 2 0 Diminished vision Postsurgical: cataract surgery glaucoma surgery scleral fixation of intraocular vitrectomy for vitreous hemorrhage Posttraumatic Complicated cataract Strabismus Number of 2 8 4 2 3 3 Presentation Fig. (): Differenternt presentations of pediatric retinal detachment. 0.7 0.6 Preoperative visual acuity Postoperative visual acuity Decimal visual acuity 0.5 0.4 0.3 0.2 0. 0 2 3 4 5 6 7 8 9 0 2 3 4 5 6 7 8 9 20 2 22 23 24 25 26 27 28 29 30 Operated eyes Fig. (2): Preoperative vs postoperative visual acuity. Visual acuity is presented in decimal fraction where 0.000= Light perception, 0.00= Hand movement, 0.0= Counting fingers. Decimal postoperative visual acuity 0. 0.0 0.00 0.000 0.000 0.00 0.0 0. Decimal preoperative visual acuity Fig. (3): Preoperative vs postoperative visual acuity. Visual acuity is presented in decimal fraction where 0.000= Light perception, 0.00 = Hand movement, 0.0= Counting fingers NB: A single point may represent more than one eye. Table (): Different presentations of pediatric rhegmatogenous retinal detachment (27 ). Presentation Number of Percentage of Diminished vision 2 44 Post-surgical: 8 30 cataract surgery 4 5 glaucoma surgery 2 7 scleral fixation of 4 intraocular lens vitrectomy for vitreous hemorrhage 4 Post-traumatic 3 Complicated cataract 3 Strabismus 4

36 Clinical Features & Surgical Outcome of Pediatric Retinal Detachment Table (2): Types of retinal breaks causing retinal detachment in operated eyes (30 eyes). Type of retinal break Number of Percentage of Traction retinal tears 2 44 (horse-shoe tears) Atrophic retinal tears with 6 22 retinal degenerations Giant retinal tears 4 5 Retinal dialysis 5 9 Not documented 3 Table (3): Postoperative complications after surgery for pediatric retinal detachment. Complication Number of High intraocular pressure 6 Hypotony 2 Band-shaped keratopathy Crystalline lens injury Iatrogenic macular hole Discussion In this retrospective study we tried to enlighten some of the facts concerning retinal detachment in children in Egypt. For although the incidence reported in different studies for retinal detachment in children is low, the impact of this potentially blinding disease is high considering the vision threatening pathology in the fellow eye, the emotional state of the child's family and the financial aspect of a handicapped child. The etiological factors responsible for the retinal detachment was assumed in this series according to the most prominent factor immediately recorded before the detachment. Accordingly the factors responsible for retinal detachment could be established in 27 eyes excluding the three cases presenting with cataract which could be of traumatic origin. Ocular trauma was directly related to retinal detachment in 0% of cases, while 27% of eyes had ocular surgery previous to the development of RRD. Retinal lesions in the form of horse shoe breaks, atrophic retinal holes, degenerative myopia and giant retinal breaks, without other associated factors were identified in 53% of cases. Previous repots have reported a higher incidence of trauma ranging between 23-53% [,6,9-] compared to the surgical factor which had a rate of 5% to 34% [,6, 9-]. In our series the incidence of traumatic RRD was low but within the average of traumatic retinal detachment when considering all age groups which is % [2]. However if add the cases presenting of with cataract (3 cases) the incidence in our series will be 20% which although still low but comparable to the other studies. The relative high incidence of post surgical cases may be related to the fact that we are a referral center. Other publications arising from referral centers have reported a similar high incidence of the post surgical cases in relation to posttraumatic. Their incidence ranged between 34% to 6% [6,9,]. Post surgical retinal detachment is documented in literature for some surgeries like post cat 0.02% to 3.6% [4] and post molteno implant 5% [3]. Unfortunately statistical data from the referring physicians were lacking to see whether the incidence rate is within the percentage of other publications. The incidence of predisposing retinal lesions reported in our study, 53%, was within the average incidence reported in other studies which varied between 3%-56% [,4]. Previous studies have reported myopia, isolated or associated with other systemic conditions as Stickler and Marfan [0,5,6] among the most commonly predisposing factor for retinal lesion. However there was a variability in incidence which may be related to racial factor, considering that myopia is more common in Asian population [9,0,5,6], also that in some series the refraction was not recorded []. In our series 9 out of the 6 classified in this group had bilateral retinal detachment which made the preoperative recording of refraction difficult. Postoperatively, the operative procedure in the form of buckle application or lens removal would have changed the refraction, accordingly it was difficult to record the incidence of refractive errors in this group. The recording of a familial systemic underlying syndrome was not recorded due to the lack of medical records from the families presenting with a child having retinal detachment. In addition it was difficulty to recruit family members from distant rural areas for examination. Family history was also difficult to rely on since in most cases it was obtained from an uneducated family member or a remote relative who is not completely oriented of the disease. Given the young age of the (0.5 ±3.7 years), it is not surprising that most did not complain early of visual symptoms. The macula was off in the presenting eye in all cases with varying degree of PVR. This is consistent with other reports which had a high incidence of macular involvement with varying degree of chronicity [7,9, ]. Lack of subjective complain, may be also a factor in the high incidence of bilateral presentation. Previous publications have reported an 8% [6,9] incidence of bilaterality at time of presentation.

Asser A.E. Abd El-Meguid, et al. 37 We had a higher rate of bilateral involvement. Nine in our series had retinal detachment in the other eye at presentation (33%) and if we excluded the post traumatic (3) and the post surgical (8) cases the percentage rises to about 56% (9 out of 6). The discrepancy in the bilateral involvement in our series could reflect the leakage in the child healthcare and the necessity to do regular visual screening especially in the preschool age by trained medical personnel, eg during the vaccination program. This may help earlier detection and consequently may save some vision in the cases which became inoperable. Due to the complexity of the retinal detachment and associated PVR, vitrectomy was the procedure of choice in this case series except in one case where a buckle procedure was done in the other non presenting eye. A comparable high rate of vitrectomy has been mentioned in other reports. This ranged between 67% to 74% [9]. Silicon oil as an endo-tamponade was used with variable degree in other reports, it ranged between 23% to 88% [9,]. In our series all cases performed vitrectomy received silicon oil as an endotampoande. Silicon oil has the advantage of being transparent allowing for some visual rehabilitation in the operated eye. This will allow for better spatial orientation for with healthy other eye and some visual gain for the one eyed, 9 with detachment in the other eye. Furthermore silicon oil requires minimal positioning compared to gas. In addition the longer tamponading effect of silicon oil was felt to be necessary in these cases with advanced PVR for it has the advantage of keeping the macula attached in the presence of peripheral proliferation which would have caused a recurrent total retinal detachment and thus giving the chance to select the optimum time for a second interference. The anatomical outcome of surgical intervention was favorable in most cases, we had a success rate of 86.6% and if we added with attached retina under silicon oil the reattachment rate will rise to 93.3%. Other reports have reported a similar incidence of reattachment rate which varied between 79% to 88% [6,9,]. Younger age, worse initial visual acuity, greater extent of retinal detachment, and the presence of PVR are associated with less favorable visual outcome. However all successfully operated regained hand movement visual acuity or better. The study has showed that we had a higher incidence of bilaterality also a higher rate of resorting to vitrectomy with silicon oil. This may reflect the more delayed presentation in our cases. Only 3 were referred from other medical personnel involved in the child health care system. These finding may reflect the lower health care for the children in our series. It is also is important in this context to mention that the in the post surgical group may have been subjected to improper surgical technique. This is suggested by the clinical picture at presentation, a post cataract cases presented with a mal poisoned lens and vitreous incarceration in the wound. The multiplicity of surgical interference, the patient undergone a scleral fixation intraocular lens was subjected to more than 4 surgeries. In addition to the intraoperative findings of the type of retinal lesion(s) identified as the cause of the retinal detachment, the post vitrectomy case had giant retinal break. As with other retrospective studies this study had its limitations in reporting the incidence of syndromes which is expected to be high in a country with high consanguinity marriage. Also full ophthalmological data as visual acuity was missing from some records. However these data presents an overlooked problem in the child health care system in our country which is the lack of attention to the visual performance. Including visual performance in the child screening programs may help to save sight for a lot of children through earlier interference. Also it is important that surgical procedures in this young age group should be done in specialized centers to avoid complications. References - BUTLER T.K., KIEL A.W. and ORR G.M.: Anatomical and visual outcome of retinal detachment surgery in children. Br. J. Ophthalmol., 85 (2): 437-9, 200. 2- SCHARF J. and ZONIS S.: Juvenile retinal detachment. J. Pediatr Ophthalmol., 4 (5): 302-4, 977. 3- TASSMAN W.: Retinal detachment in children. Trans Am. Acad Ophthalmol. Otolaryngol., 7 (3): 455-60, 967. 4- WINSLOW R.L. and TASMAN W.: Juvenile rhegmatogenous retinal detachment. Ophthalmology, 85 (6): 607-8, 978. 5- SADEH A.D., DOTAN G., BRACHA R., et al.: Characteristics and outcomes of paediatric rhegmatogenous retinal detachment treated by segmental scleral buckling plus an encircling element. Eye, 5 (Pt ): 3-3, 200. 6- FIVGAS G.D. and CAPONE A. Jr: Pediatric rhegmatogenous retinal detachment. Retina., 2 (2): 0-6, 200. 7- YOKOYAMA T., KATO T., MINAMOTO A., et al.: Characteristics and surgical outcomes of paediatric retinal detachment. Eye, 8 (9): 889-92, 2004. 8- WADHWA N., VENKATESH P., SAMPANGI R. and GARG S.: Rhegmatogenous retinal detachments in children in India: clinical characteristics, risk factors, and surgical outcomes. J. Aapos., 2 (6): 55-4, 2008.

38 Clinical Features & Surgical Outcome of Pediatric Retinal Detachment 9- GONZALES C.R., SINGH S., YU F., et al.: Pediatric rhegmatogenous retinal detachment: clinical features and surgical outcomes. Retina., 28 (6): 847-52, 2008. 0- CHEN S.N., JIUNN-FENG H. and TE-CHENG Y.: Pediatric rhegmatogenous retinal detachment in taiwan. Retina., 26 (4): 40-4, 2006. - WEINBERG D.V., LYON A.T., GREENWALD M.J. and METS M.B.: Rhegmatogenous retinal detachments in children: risk factors and surgical outcomes. Ophthalmology, 0 (9): 708-3, 2003. 2- HAIMANN M.H., BURTON T.C. and BROWN C.K.: Epidemiology of retinal detachment. Arch. Ophthalmol., 00 (2): 289-92, 982. 3- WATERHOUSE W.J., LLOYD M.A., DUGEL P.U., et al.: Rhegmatogenous retinal detachment after Molteno glaucoma implant surgery. Ophthalmology, 0 (4): 665-7, 994. 4- OKINAMI S., OGINO N., NISHIMURA T. and TANO Y.: Juvenile retinal detachment. Ophthalmologica, 94 (2-3): 95-02, 987. 5- WANG N.K., TSAI C.H., CHEN Y.P., et al.: Pediatric rhegmatogenous retinal detachment in East Asians. Ophthalmology, 2 (): 890-5, 2005. 6- CHANG P.Y., YANG C.M., YANG C.H., et al.: Clinical characteristics and surgical outcomes of pediatric rhegmatogenous retinal detachment in Taiwan. Am. J. Ophthalmol., 39 (6): 067-72, 2005.