In Sturge-Weber syndrome, the ocular manifestation is
**Core Concept**
Sturge-Weber syndrome is a rare neurocutaneous disorder characterized by the presence of a port-wine stain, typically on the face, and associated with leptomeningeal angiomatosis. The ocular manifestations are a crucial part of the syndrome, and understanding these manifestations is essential for diagnosis and management.
**Why the Correct Answer is Right**
The ocular manifestation of Sturge-Weber syndrome is glaucoma, particularly infantile glaucoma. This is due to the presence of choroidal hemangiomas, which can lead to increased intraocular pressure (IOP) and subsequent glaucoma. The choroidal hemangiomas are thought to be responsible for the abnormal development of the trabecular meshwork, leading to impaired aqueous outflow and increased IOP.
**Why Each Wrong Option is Incorrect**
**Option A:** Glaucoma is not the only ocular manifestation of Sturge-Weber syndrome, but it is the most common and serious complication. Other ocular manifestations, such as choroidal hemangiomas and optic disc drusen, are also present but are less common.
**Option B:** Optic disc drusen are a feature of Sturge-Weber syndrome, but they are not the primary ocular manifestation. Drusen are composed of deposits of fibrillar material and can be seen in the optic disc, but they are not as clinically significant as glaucoma.
**Option C:** Choroidal hemangiomas are a feature of Sturge-Weber syndrome, but they are not the primary ocular manifestation. Hemangiomas are benign vascular tumors that can cause increased IOP and glaucoma, but they are not the primary cause of ocular disease in this syndrome.
**Option D:** Leptomeningeal angiomatosis is a feature of Sturge-Weber syndrome, but it is not an ocular manifestation. Angiomatosis refers to the abnormal growth of blood vessels in the leptomeninges, which can cause seizures, developmental delay, and other neurological symptoms.
**Clinical Pearl / High-Yield Fact**
It is essential to remember that glaucoma is a common and serious complication of Sturge-Weber syndrome. Early detection and management of glaucoma are crucial to prevent vision loss and other complications. A thorough ophthalmological examination, including tonometry and gonioscopy, is essential in the diagnosis and management of Sturge-Weber syndrome.
**Correct Answer:** C.