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The 2016 Founders Award Lecture

Shields, Carol L. MD*; Say, Emil A. T. MD*; Pefkianaki, Maria MD, MSc, PhD*; Regillo, Carl D. MD; Caywood, Emi H. MD; Jabbour, Pascal M. MD§,¶; Shields, Jerry A. MD*

doi: 10.1097/IAE.0000000000001382
Original Study

Purpose: To evaluate rhegmatogenous retinal detachment (RRD) in eyes with retinoblastoma after intraarterial chemotherapy (IAC).

Design: Retrospective case series.

Methods: Chart review.

Main outcome measure: Development of RRD in the IAC era.

Results: Of 167 eyes in 157 consecutive patients, mean patient age at diagnosis of retinoblastoma was 19 months. Intraarterial chemotherapy was primary (75/167, 45%) or secondary (92/167, 55%). There were 10 eyes (10/167, 6%) that developed RRD after IAC. The RRD was mostly related to rapid tumor regression with atrophic retinal hole, occurring within one month (n = 8) or 12 months (n = 2) of IAC. Rhegmatogenous retinal detachment was found after primary (6/75, 8%) or secondary (4/92, 4%) IAC. Of primary cases, RRD was found in Group D (1/38 [3%], P = 0.1075) or Group E (5/30 [17%], P = 0.0348). For primary IAC (n = 75 eyes), RRD was found in endophytic (5/22 [23%], P = 0.0073), exophytic (0/29 [0%], P = 0.0760), or combined endophytic/exophytic pattern (1/24 [4%], P = 0.6575). A comparison of eyes with RRD (n = 10) versus without RRD (n = 157) found significant differences including greater mean age at presentation (38 vs. 18 months, P = 0.0522), greater 4-quadrant vitreous seeding (5/10, 50% vs. 27/157, 17%, P = 0.0236), and absence of subretinal fluid (3/10, 30% vs. 102/157, 65%, P = 0.0236). The cause of RRD was tumor regression–related atrophic retinal hole(s) in 7 (7/10, 70%) (unifocal [1/10, 10%] or multifocal [6/10, 60%] holes), cryotherapy-induced single atrophic hole in 2 (2/10, 20%), and single flap-tear from posterior vitreous detachment in one (1/10, 10%). In 4 (4/10, 40%) eyes with RRD, proliferative vitreoretinopathy was noted. The RRD was not related to intravitreal injection in any case, as in primary IAC no case had previous injection and in secondary IAC the injections were performed many months previously. Primary RRD repair involved pars plana vitrectomy in three, scleral buckle without drainage in one, laser barricade in one, and observation in five eyes. After 24 months mean follow-up, the retina showed complete reattachment (3/10, 30%), partial reattachment (2/10, 20%), and persistent detachment in all observed eyes (5/10, 50%). Enucleation was necessary for tumor recurrence (4/10, 40%) or neovascular glaucoma (1/10, 10%). There were no tumor-related metastases or death.

Conclusion: After IAC for retinoblastoma, RRD occurs in 6%, mostly in advanced eyes with extensive endophytic tumor and generally from atrophic retinal hole after rapid tumor regression.

Rhegmatogenous retinal detachment was found in 10 (6%) of 167 consecutive eyes with retinoblastoma managed with intraarterial chemotherapy, most often in older children with Group E tumor, with extensive vitreous seeding and tumor invading nearly the entire retina.

*Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania;

The Retina Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania;

The Nemours Center for Cancer and Blood Disorders, Nemours/Alfred I. DuPont Hospital for Children, Thomas Jefferson University, Wilmington, Delaware; and

Departments of §Neurovascular and Endovascular Surgery, and

Neurological Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania.

Reprint requests: Carol L. Shields, MD, Ocular Oncology Service, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107; e-mail:

Supported by Eye Tumor Research Foundation, Philadelphia, PA (C.L.S., J.A.S.), the Carlos G. Bianciotto Fund for Retinoblastoma Research, Philadelphia, PA (C.L.S., J.A.S.), and the Lucille Weidman Fund for Pediatric Cancer, Philadelphia, PA (J.A.S., C.L.S.). The funders had no role in the design and conduct of the study, in the collection, analysis and interpretation of the data, and in the preparation, review, or approval of the manuscript. C. L. Shields has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Presented in part at the Founders Award Lecture of the American Society of Retina Specialists on August 10, 2016, San Francisco, California. (C.L.S.).

None of the authors has any conflicting interests to disclose.

© 2017 by Ophthalmic Communications Society, Inc.