Zhengping Hu1,2, Jian Cao3,4, Eun Young Choi1,2, Yun Li5,6. 1. Schepens Eye Research Institute, Mass. Eye and Ear, Boston, MA, USA. 2. Harvard Medical School, Boston, MA, USA. 3. Department of Ophthalmology, The 2nd Xiangya Hospital of Central South University, Changsha, Hunan, People's Republic of China. 4. Hunan Clinical Research Center of Ophthalmic Disease, Changsha, Hunan, People's Republic of China. 5. Department of Ophthalmology, The 2nd Xiangya Hospital of Central South University, Changsha, Hunan, People's Republic of China. yun.li@csu.edu.cn. 6. Hunan Clinical Research Center of Ophthalmic Disease, Changsha, Hunan, People's Republic of China. yun.li@csu.edu.cn.
Abstract
BACKGROUND: Sturge-Weber syndrome is a disorder marked by a distinctive facial capillary malformation, neurological abnormalities, and ocular abnormalities such as glaucoma and choroidal hemangioma. CASE PRESENTATION: We report a case of progressively formed retinal vessel malformation in a premature male infant with Sturge-Weber syndrome and retinopathy of prematurity, after treatment with intravitreal anti-vascular endothelial growth factor (VEGF). The baby was born at 30 weeks gestation with a nevus flammeus involving his left eyelids and maxillary area. On postmenstrual age week 39, he received intravitreal anti-VEGF. Diffuse choroidal hemangioma became evident at 40 weeks, with the classic "tomato catsup fundus" appearance. These clinical findings characterized Sturge-weber syndrome. He presented with posterior retinal vessel tortuosity and vein-to-vein anastomoses at 44 weeks. CONCLUSION: This is a rare case of documented progression of retinal vessel malformations in a patient with Sturge-Weber syndrome and retinopathy of prematurity.
BACKGROUND:Sturge-Weber syndrome is a disorder marked by a distinctive facial capillary malformation, neurological abnormalities, and ocular abnormalities such as glaucoma and choroidal hemangioma. CASE PRESENTATION: We report a case of progressively formed retinal vessel malformation in a premature male infant with Sturge-Weber syndrome and retinopathy of prematurity, after treatment with intravitreal anti-vascular endothelial growth factor (VEGF). The baby was born at 30 weeks gestation with a nevus flammeus involving his left eyelids and maxillary area. On postmenstrual age week 39, he received intravitreal anti-VEGF. Diffuse choroidal hemangioma became evident at 40 weeks, with the classic "tomato catsup fundus" appearance. These clinical findings characterized Sturge-weber syndrome. He presented with posterior retinal vessel tortuosity and vein-to-vein anastomoses at 44 weeks. CONCLUSION: This is a rare case of documented progression of retinal vessel malformations in a patient with Sturge-Weber syndrome and retinopathy of prematurity.
Authors: José Carlos Rivera; Przemyslaw Sapieha; Jean-Sébastien Joyal; François Duhamel; Zhuo Shao; Nicholas Sitaras; Emilie Picard; Ellen Zhou; Pierre Lachapelle; Sylvain Chemtob Journal: Neonatology Date: 2011-10-03 Impact factor: 4.035
Authors: Matthew D Shirley; Hao Tang; Carol J Gallione; Joseph D Baugher; Laurence P Frelin; Bernard Cohen; Paula E North; Douglas A Marchuk; Anne M Comi; Jonathan Pevsner Journal: N Engl J Med Date: 2013-05-08 Impact factor: 91.245