Abstract
Phacomatoses are a group of neurocutaneous disorders characterized by vascular and pigmentary birthmarks, often involving multiple organ systems. Klippel–Trenaunay syndrome (KTS), a rare phacomatosis, is characterized by the classical triad of cutaneous capillary hemangiomas, venous malformations, and soft tissue overgrowth. Glaucoma secondary to angle dysgenesis, raised episcleral venous pressure, or excess angle pigmentation is observed. We report a case of KTS that presented with cutaneous capillary hemangiomas and limb hypertrophy along with unilateral buphthalmos. This eye was noted to have persistent tunica vasculosa lentis and persistent pupillary membrane with pupillary block glaucoma that was treated with pupilloplasty. To the best of our knowledge, this mechanism of glaucoma in KTS has not been reported before. The purpose of this article is to document this rare presentation as well as highlight the importance of detecting the underlying mechanism for appropriate management.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.