Klippel-Trénaunay syndrome (KTS) is a neuro-oculocutaneous disorder consisting of a vascular nevus involving an extremity, varicosities of that extremity, and hypertrophy of bone and soft tissue. When arteriovenous malformation is also present, the disease is called Klippel-Trénaunay-Weber syndrome. Occurrence of KTS is sporadic and the etiology is unknown. Ophthalmic findings include vascular anomalies of the orbit, iris, retina, choroid, and optic nerve, as well as optic nerve and chiasmal gliomas. There is also a risk of glaucoma.
KTS is a complex syndrome with no single treatment protocol. Treatment is individualized to the patient. At present, many of the symptoms may be treated, but there is no cure for this syndrome.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.