Outcomes of infantile-onset glaucoma associated with port wine birthmarks and other periocular cutaneous vascular malformations

Daniela Reyes-Capó, Kara M Cavuoto, Ta Chang

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Purpose: The incidence of infantile-onset secondary glaucoma associated with periocular cutaneous vascular malformations is high and the outcomes of these glaucomatous eyes have anecdotally been poor. The purpose of this study was to determine the anatomic and visual outcomes of affected eyes. Design: Retrospective case series. Methods: Consecutive patients with early-onset (younger than 36 months of age) glaucoma associated with cutaneous vascular malformations from 1995-2015 were included. Results: Seventeen eyes of 13 patients with Sturge-Weber syndrome (SW, n = 10), Klippel-Trenaunay-Weber syndrome (KTW, n = 1), cutis marmorata telangiectatica congenita (CMTC, n = 1), and phakomatosis pigmentovascularis (PPV, n = 1) were included. Three SW and 1 KTW patient had bilateral glaucoma. At presentation, mean age was 6.5 ± 9.1 months and mean intraocular pressure was 27.2 ± 6.13 mm Hg. The average number of surgical procedures per eye increased from 1.0 ± 0.5 (range, 0-2) at less than 5 years' follow-up (9 eyes) to 3.5 ± 2.3 (range, 1-7) with at least 5 years' follow-up (8 eyes). Visual acuity was better than or equal to 20/70 in 2 of 6 eyes (33%) with less than 5 years' followup and in 3 of 7 eyes (43%) with at least 5 years' follow-up. Additionally, a higher number of baseline risk factors correlated with poorer visual outcome. Conclusions: After a mean follow-up of 6.6 years, visual outcome in infantile-onset secondary glaucoma associated with cutaneous periocular vascular malformation is guarded. Increased numbers of baseline risk factors and procedures are associated with poorer vision.

Original languageEnglish (US)
Pages (from-to)95-98
Number of pages4
JournalAsia-Pacific Journal of Ophthalmology
Issue number2
StatePublished - Mar 1 2018



  • Cutaneous venous malformations
  • Pediatric ophthalmology
  • Secondary glaucoma

ASJC Scopus subject areas

  • Medicine(all)

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