OSLI Retina

June 2019

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June 2019 · Vol. 50, No. 6 377 ■ S U R G I C A L R E V I E W ■ Visual and Anatomical Outcomes With Vitrectomy in Posterior or Combined Persistent Fetal Vasculature in an Asian Population Chun-Ting Yeh, MD; Kuan-Jen Chen, MD; Laura Liu, MD, PhD; Nan-Kai Wang, MD, PhD; Yih-Shiou Hwang, MD, PhD, An-Ning Chao, MD; Tun-Lu Chen, MD; Chi-Chun Lai, MD; Wei-Chi Wu, MD, PhD BACKGROUND AND OBJECTIVE: To investigate clini- cal features and surgical outcomes of vitrectomy in posterior or combined persistent fetal vasculature (PFV) in an Asian pediatric population. PATIENTS AND METHODS: This study was a retro- spective, noncomparative, interventional case se- ries relating the surgical outcome of PFV. Eyes that underwent pars plicata vitrectomy and/or lensec- tomy for posterior or combined PFV between 2006 and 2015 were included. The main outcome mea- sures were the anatomic and functional results as well as the complications after the vitrectomy with or without lensectomy. RESULTS: A total of 25 eyes of 18 patients younger than 8 years of age were included in the study. The mean age of the patients receiving first pars plicata vitrectomy and/or lensectomy was 15.2 months ± 21.7 months (range: 1 month to 83 months). Post- operatively, successful anatomic correction in the posterior segment was observed in 20 eyes (80%). In addition, 19 of the 25 eyes (76%) had visual acu- ity (VA) better than 20/4000, and the mean logMAR VA of these 19 eyes was 1.74 (range: 0.48 to 2.30). The mean change of axial length of the eyes receiv- ing surgery was 0.7 mm ± 1.4 mm (range: –1.0 mm to 2.4 mm; P = .18). None of the patients ended up with phthisis or glaucoma. CONCLUSIONS: This study suggests that vitrectomy and/or lensectomy in patients with posterior or combined PFV with macular involvement may re- sult in an acceptable anatomical outcome; howev- er, the functional outcome remained poor despite surgical intervention in these patients. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:377-384.] INTRODUCTION In 1955, Reese 1 first introduced a peculiar and rare congenital ocular syndrome presented with cataract, secondary glaucoma, and retinal detachment in a mi- cro-ophthalmic eye. Regarding the abnormal regres- sion of primary vitreous and hyaloid vasculature in the index cases, he named the disease as persistent hyperplastic primary vitreous (PHPV). It was not until 1997 that Goldberg 2 integrated iris vascular remnants and omnibus manifestations in the anterior segment into the disease spectrum and further categorized the disease into anterior, posterior, or combined anterior and posterior types based on the location of vascular malformations. To emphasize the pathogenic failure of the fetal vascular system to spontaneously apopto- sis, he renamed the disease as persistent fetal vascu- lature (PFV). Like many rare congenital diseases, the exact pro- portions of three categories are inconclusive in terms of different study results, albeit the "pure" posterior PFV is extremely uncommon. 3 Clinically, anterior PFV usually presents with congenital cataract, and can be treated successfully with cataract extraction and intraocular lens (IOL) implantation. 4,7 Combined From the Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan (CTY, KJC, LL, NKW, YSH, ANC, TLC, CCL, WCW); and Chang Gung University, College of Medicine, Taoyuan, Taiwan (KJC, LL, NKW, YSH, ANC, TLC, CCL, WCW). Originally submitted June 17, 2018. Revision received June 17, 2018. Accepted for publication November 5, 2018. Supported by Chang Gung Memorial Hospital research grants (CMRP- G3G30581 and CMRPG3F0191~3) and a Ministry of Science and Technolo- gy grant (MOST 106-2314-B-182A-040-MY3). These funding organizations play no role in the design and conduct of the study; collection, manage- ment, analysis, and interpretation of the data; and preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. The authors report no relevant financial disclosures. Address correspondence to Wei-Chi Wu, MD, PhD, No. 5, Fu-Hsin Rd., Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan 333; email: weichi666@gmail.com. doi: 10.3928/23258160-20190605-06

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