J Korean Ophthalmol Soc.  2017 Nov;58(11):1234-1241. 10.3341/jkos.2017.58.11.1234.

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

• Kim MJ
• Lee WJ
• Park KH
• Kim TW
• Lee EJ
• Yu YS
• Jeoung JW

• KMID: 2395864
• DOI: http://doi.org/10.3341/jkos.2017.58.11.1234

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.

Keyword

Facial angioma; Facial port-wine stain; Glaucoma; Nevus flammeus; Sturge-Weber syndrome

MeSH Terms

Drainage
Glaucoma*
Hemiplegia
Humans
Intellectual Disability
Intraocular Pressure
Medical Records
Ocular Hypertension
Port-Wine Stain*
Reoperation
Retrospective Studies
Seizures
Sturge-Weber Syndrome
Trabeculectomy

Figure

• 

  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.

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