Retinoschisis Detected with Handheld Spectral-Domain Optical Coherence Tomography in Neonates with Advanced Retinopathy of Prematurity
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CLINICAL SCIENCES Retinoschisis Detected With Handheld Spectral-Domain Optical Coherence Tomography in Neonates With Advanced Retinopathy of Prematurity Rajeev H. Muni, MD, FRCSC; Radha P. Kohly, MD, FRCSC, PhD; Alexander C. Charonis, MD; Thomas C. Lee, MD Objectives: The recent development of handheld spectral- nal detachment following laser photocoagulation. Reti- domain optical coherence tomography (HHSD-OCT) has nal detachment was not detected on clinical examination enabled us to obtain high-resolution retinal scans of pre- in the 5 remaining eyes, although there was vitreoretinal mature infants with retinopathy of prematurity (ROP). The traction at the ridges of 3 of the 5 eyes. The HHSD-OCT purpose of this study is to document HHSD-OCT find- identified presumed retinoschisis in all 5 study eyes. ings in laser-treated neonates with progressive ROP. Conclusions: Tractional retinoschisis may be an impor- Methods: This is a retrospective consecutive case se- tant finding in progressive laser-treated ROP, made pos- ries of 3 patients with progressive ROP despite laser pho- sible by use of the HHSD-OCT. This finding may have sig- tocoagulation. All patients were transferred from periph- nificant implications for how we classify and treat patients eral neonatal intensive care units (NICUs) to the whose ROP is progressing despite laser treatment. Fur- Children’s Hospital Los Angeles. All patients had a com- thermore, the use of the HHSD-OCT can provide valu- plete ocular examination, fundus photography, fluores- able insight into the interaction of the retina, vitreous, and cein angiography, and HHSD-OCT. ridge in patients with progressive ROP, and it allows shal- low detachments and retinoschisis to be diagnosed earlier Results: All 3 patients had active progressive ROP de- and more accurately than would otherwise be possible. spite prior laser photocoagulation. Of the 6 eyes, 1 was excluded from the study because it had an exudative reti- Arch Ophthalmol. 2010;128(1):57-62 ETINOPATHY OF PREMATU- low nature of the detachment and the re- rity (ROP) is a potentially duced prominence of the retinal pigment blinding condition of pre- epithelium in premature infants, espe- mature and low-birth- cially those of white race. weight infants. Infants with Optical coherence tomography (OCT) Rhigh-risk prethreshold ROP benefit from has revolutionized the diagnosis and man- early laser photocoagulation of the avas- agement of several adult vitreoretinal dis- cular retina, with lower rates of vision loss eases. The standard OCT setup requires the and improved anatomic outcomes.1 How- patient to be cooperative, while sitting up- ever, as found in the Early Treatment of right and holding their head steady on the Retinopathy of Prematurity Study, despite chin rest. As a result, the use of OCT in very young children and infants can be problem- Author Affiliations: Retina Video available online at atic. The recent development of the hand- Institute, The Vision Center, www.archophthalmol.com held spectral-domain OCT (HHSD-OCT) Childrens Hospital Los Angeles, (3DSDOCT platform; Bioptigen, Durham, Doheny Eye Institute, early laser photocoagulation, 9% of in- North Carolina) has enabled us to obtain University of Southern fants with high-risk prethreshold disease high-resolution retinal scans of premature California, Los Angeles will still have an unfavorable outcome.1 This infants with ROP. Unlike other imaging sys- (Drs Muni and Lee); occurs secondary to progressive fibrovas- tems used in children such as the RetCam Department of Ophthalmology cular proliferation at the junction of the vas- (Clarity Medical Systems, Pleasanton, Cali- and Vision Sciences, University cular and avascular retina. Stage 4 ROP rep- fornia), the HHSD-OCT involves a no- of Toronto, Hospital for Sick resents a tractional retinal detachment contacttechniquewiththehandpieceplaced Children and St Michael’s 2 Hospital, Toronto, Ontario, secondary to this fibrovascular prolifera- closetothepatient’seye.Scottetal conclude Canada (Drs Muni and Kohly); tion. Although surgery can be beneficial in that the HHSD-OCT is a safe, noninvasive, and the Retina Service, Athens some cases, it is often difficult to deter- and effective method of obtaining in vivo Vision Institute, Athens, Greece mine the severity of early stage 4 disease. high-resolution information regarding reti- (Dr Charonis). This may be partially related to the shal- nal morphologic features in children. (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 1), JAN 2010 WWW.ARCHOPHTHALMOL.COM 57 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 A B Figure 1. Fundus photograph of 360° of cicatricial stage 3 retinopathy of prematurity with traction at the base in the right (A) and left (B) eyes. sedated and, in some cases, paralyzed with vecuronium. After A discussing the results of the clinical examination and investi- gations with the parents, treatment decisions were made. Data were collected in accordance with the Health Insurance Port- ability and Accountability Act. RESULTS CASE 1 B Case 1 is that of a male infant born after 24 weeks of ges- tation with a birth weight of 630 g, delivered by a 30-year- old, gravida 2, para 1, African American mother via nor- mal spontaneous vaginal delivery. The patient was transferred to the NICU of an outside hospital, and his course was complicated by respiratory insufficiency, intraven- tricular hemorrhage, anemia of prematurity, and sepsis. The infant was treated with full laser photocoagulation in both Figure 2. Handheld spectral-domain optical coherence tomography of the eyes for zone 2, stage 3 ROP with plus disease at another right (A) and left (B) eyes, showing bilateral peripheral retinoschisis. institution at 39 weeks of age. Despite treatment, the ROP Retinoschisis in the left eye extends posteriorly into the macula. continued to progress in both eyes, at which point the de- cision was made to transfer the patient to Childrens Hos- pital Los Angeles 3 weeks after treatment for further care. Here we describe a consecutive case series of 3 pa- On transfer, examination using anesthesia revealed per- tients with progression of their ROP despite laser pho- sistent tunica vasculosa lentis in both eyes. Examina- tocoagulation in which high-resolution HHSD-OCT was tion of the posterior segment revealed 360° of cicatriz- performed. In all cases, it was unclear whether the ROP ing stage 3 ROP in posterior zone 2, extending high into had progressed to stage 4A based on clinical examina- the vitreous with good laser treatment of the avascular tion. The HHSD-OCT demonstrated presumed retinos- retina in both eyes (Figure 1). In the left eye, there was chisis extending posteriorly from the temporal ridge in traction at the base of the ridge associated with eleva- all 3 cases. This is a previously undescribed finding dur- tion of the retinal vessels just posterior to the ridge, with ing the acute phases of advanced ROP. These findings no evidence of vitreous condensation or other mem- assisted us in determining whether to proceed with more branes over the retinal surface. The view in the right eye aggressive treatment, including lens-sparing pars plana showed prominent anterior vitreous membranes extend- vitrectomy (LS-PPV) in 1 case, although the natural his- ing from the ridge itself; however, there was no evi- tory of this finding is unclear, and careful observation dence of any vitreous membranes overlying the retina pos- could be an option. Based on our experience, we believe terior to the ridge. In addition, there was no evidence on that HHSD-OCT can be a useful tool in the diagnosis and clinical examination by multiple observers of a retinal de- management of progressive ROP. tachment. The HHSD-OCT revealed findings consistent with bilateral presumed retinoschisis most prominently METHODS noted in the region just posterior to the temporal ridge in both eyes, with extension into the macula in the left This is a retrospective consecutive case series of 3 patients with eye (Figure 2; videos 1 and 2 [http://archophthalmol- progressive ROP despite laser photocoagulation. All patients .com]). Although there was no retinal detachment seen were transferred from peripheral neonatal intensive care units on HHSD-OCT, the decision was made to proceed with (NICUs) to a tertiary care pediatric retina center at the Chil- dren’s Hospital Los Angeles because of progressing ROP. All bilateral 23-gauge LS-PPV based on the retinoschisis- patients had complete ocular examination, fundus photogra- like findings. The patient required repeat LS-PPV 2 weeks phy, fluorescein angiography, and examination with the HHSD- later for postoperative vitreous hemorrhage in both eyes. OCT. Fluorescein angiography and HHSD-OCT examina- Two weeks following the second operation, the patient was tions were performed in all 4 quadrants while the infants were found to be stable, with regressing ROP in both eyes and (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 1), JAN 2010 WWW.ARCHOPHTHALMOL.COM 58 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 A A B C B Figure 3. Active stage 3 retinopathy of prematurity despite laser treatment. Figure 5. RetCam photographs 2 weeks following repeated laser treatment in Active (perfused) stage 3 with mild plus disease in the right (A) and the right eye (A) and intravitreal bevacizumab treatment in the left eye (B) moderate plus disease in the left (B) eye can be seen. C, Fluorescein show resolution of plus disease and regression of the stage 3 retinopathy of angiogram of the left eye with the RetCam reveals leakage from the ridge prematurity in both eyes. with peripheral nonperfusion both anterior and posterior to the shunt. A A Stage 3 Artifact Retinoschisis B B Figure 6. Handheld spectral-domain optical coherence tomography of the Figure 4. Handheld spectral-domain optical coherence tomography shows a right (A) and left (B) eyes performed 2 weeks following treatment shows ridge with early tractional retinoschisis extending from the posterior aspect improvement in the extent of the tractional retinoschisis.