J Korean Ophthalmol Soc > Volume 58(11); 2017 > Article
Journal of the Korean Ophthalmological Society 2017;58(11):1234-1241.
DOI: https://doi.org/10.3341/jkos.2017.58.11.1234    Published online November 15, 2017.
Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain.
Mi Jin Kim, Won June Lee, Ki Ho Park, Tae Woo Kim, Eun Ji Lee, Young Suk Yu, Jin Wook Jeoung
1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea. neuroprotect@gmail.com
2Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea.
3Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea.
안면화염혈관종 환자에서의 녹내장 발생양상 및 치료
서울대학교 의과대학 안과학교실1, 서울대학교병원 안과2, 분당서울대학교병원 안과3
Correspondence:  Jin Wook Jeoung,
Email: neuroprotect@gmail.com
Received: 6 July 2017   • Revised: 18 September 2017   • Accepted: 20 October 2017
To characterize the development of glaucoma, age of glaucoma onset, and treatments for patients with a facial port-wine stain (PWS). METHODS: We performed a retrospective analysis of the medical records of 58 patients (116 eyes) with facial PWS between January 2000 and August 2016. We noted patients' age at the initial examination, cup-to-disc ratio, corneal diameter, occurrence of ocular hypertension, development of glaucoma, age of glaucoma onset, and treatments. We compared the clinical features of eyes that developed glaucoma with those that did not develop glaucoma. Among those eyes with glaucoma, we investigated the differences between eyes that underwent surgery and those that did not undergo surgery. RESULTS: Among the 58 patients with a facial PWS (116 eyes), glaucoma was diagnosed in 38 patients (46 eyes; 39.66%). Of these, 26 patients (27 eyes; 58.69%) underwent glaucoma surgery. PWS-associated glaucoma usually developed by the age of 2 years (85.61%). In all patients, glaucoma developed on the same side of the face as the PWS. Of the 58 patients, 19 (32.76%) showed neurological symptoms, including seizures, developmental delays, intellectual disabilities, or hemiplegia, and 32 (55.17%) were diagnosed with Sturge-Weber syndrome. The mean number of glaucoma surgeries was 1.55 ± 0.93. The initial surgery included trabeculectomy (7 eyes), trabeculotomy (5 eyes), combined trabeculotomy/trabeculectomy (13 eyes), and aqueous drainage device insertion (2 eyes). The mean age at the first surgery was 35.14 ± 50.91 months. In 18 of 27 eyes (66.67%), the postoperative intraocular pressure (IOP) was controlled to below 21 mmHg, but 9 eyes (33.33%) showed elevated IOP and required a reoperation. CONCLUSIONS: PWS can be accompanied by ocular hypertension or glaucoma, so patients require regular ophthalmic examinations. When glaucoma occurs, it often does not respond to medication, making it difficult in some cases to control the IOP, so appropriate glaucoma surgery is necessary.
Key Words: Facial angioma;Facial port-wine stain;Glaucoma;Nevus flammeus;Sturge-Weber syndrome

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