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Kim, Lee, Park, Kim, Lee, Yu, and Jeoung: Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain
Original Article

Journal of the Korean Ophthalmological Society 2017; 58(11): 1234-1241.

Published online: 15 November 2017

DOI: https://doi.org/10.3341/jkos.2017.58.11.1234

Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain

Mi Jin Kim, MD1, 2, Won June Lee, MD1, 2, Ki Ho Park, MD, PhD1, 2, Tae-Woo Kim, MD, PhD1, 3, Eun Ji Lee, MD, PhD1, 3, Young Suk Yu, MD, PhD1, 2, Jin Wook Jeoung, MD, PhD1, 2

1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea.

2Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea.

3Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea.

Address reprint requests to Jin Wook Jeoung, MD, PhD. Department of Ophthalmology, Seoul National University Hospital, #101 Daehak-ro, Jongno-gu, Seoul 03080, Korea. Tel: 82-2-2072-2438, Fax: 82-2-741-3187, neuroprotect@gmail.com

Received 6 July 2017       Revised 18 September 2017       Accepted 20 October 2017

©2017 The Korean Ophthalmological Society

The Korean Ophthalmological Society

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

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.

Keywords: Facial angioma, Facial port-wine stain, Glaucoma, Nevus flammeus, Sturge-Weber syndrome

Figures and Tables

Figure 1

Sturge-Weber syndrome patient who underwent trabeculectomy (patient 6). (A) Distribution of left-sided facial port-wine stain that does not cross the midline, and left-sided glaucoma. (B) One-week postoperative appearance. Mildly engorged conjunctival and episcleral vessels with hypovascular bleb. (C) Optic disc photography revealing deep cupping with a cup/disc ratio of 0.9 in the left eye compared to right eye.

jkos-58-1234-g001
Table 1

Clinical characteristics of patients with a port-wine stain

jkos-58-1234-i001

Values are presented as the mean ± SD or n (%) unless otherwise indicated.

*A t-test was used for continuous variables; †A chi-square test was used for categorical variables.

Table 2

Clinicopathological characteristics summarized by eyes of patients with PWS (n = 116)

jkos-58-1234-i002

Values are presented as mean ± SD unless otherwise indicated. ‘P1’ means ‘comparison between 1 & 2 & 3 (analysis of variance [ANOVA])’, and ‘P2’ means ‘*Comparison between 1 and 2 by post hoc test; †Comparison between 1 and 3 by post hoc test; ‡Comparison between 2 and 3 by post hoc test’.

PWS = port wine stain; C/D = cup/disc; IOP = intraocular pressure.

Table 3

Treatment of glaucoma eyes with port-wine stain

jkos-58-1234-i003

TLO = trabeculotomy; TLE = trabeculectomy.

Notes

Conflicts of Interest The authors have no conflicts to disclose.

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Supplementary Material

Supplementary Table 1

Clinicopathlological characteristics in glaucoma treatment group (n = 46)

Supplementary Table 2

Details of 11 patients who have undergone glaucoma surgery and at least 10 years of follow-up
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