Hello
Talking about this rare syndrome, I read about in ophthalmology lecture (class of secondary glaucomas) today... So let's start with it-
Talking about this rare syndrome, I read about in ophthalmology lecture (class of secondary glaucomas) today... So let's start with it-
- It is also known as Encephalo Trigeminal Angiomatosis (ETA)
• Rare congenital Neurological and skin disorder (phakomatoses)
• Caused by Somatic Acivating Mutation in GNAQ gene.
• Port -wine stains (nervus flammeus) -
○ Usually seen on Forehead and Upper Eyelid of one side of face; present since birth.
○ Light Pink to Deep Purple
○ Caused by Overabundance of capillaries around Ophthalmic branch of Trigeminal nerve.
• Associated with
○ Secondary Glaucoma (in 50% patients)
○ Buphthalmos (enlarged eyeball due to increased intraocular tension)
○ Leukocoria (white pupillary reflex)
○ Neurologic manifestations- seizures, convulsions (on side of body opposite to birth mark), mental retardation, calcification of tissue and loss of nerve cells in cerebral cortex.
○ Ipsilateral Leptomeningeal Angioma (on same side of birth mark; calcification of underlying brain and atrophy of affected region)
• Malformed blood vessels in the piamater overlying the brain on same side of head as the birthmark.
• Radiological appearance shows Tram-Track Appearance on CT, bilaterally.
• Treatment strategies include Laser surgery, Hemispherectomy
- Latanoprost, a Prostaglandin analogue, is suitable drug for decreasing intra ocular pressure. (1 drop daily in evening)
• Rare congenital Neurological and skin disorder (phakomatoses)
• Caused by Somatic Acivating Mutation in GNAQ gene.
• Port -wine stains (nervus flammeus) -
○ Usually seen on Forehead and Upper Eyelid of one side of face; present since birth.
○ Light Pink to Deep Purple
○ Caused by Overabundance of capillaries around Ophthalmic branch of Trigeminal nerve.
• Associated with
○ Secondary Glaucoma (in 50% patients)
○ Buphthalmos (enlarged eyeball due to increased intraocular tension)
○ Leukocoria (white pupillary reflex)
○ Neurologic manifestations- seizures, convulsions (on side of body opposite to birth mark), mental retardation, calcification of tissue and loss of nerve cells in cerebral cortex.
○ Ipsilateral Leptomeningeal Angioma (on same side of birth mark; calcification of underlying brain and atrophy of affected region)
• Malformed blood vessels in the piamater overlying the brain on same side of head as the birthmark.
• Radiological appearance shows Tram-Track Appearance on CT, bilaterally.
• Treatment strategies include Laser surgery, Hemispherectomy
- Latanoprost, a Prostaglandin analogue, is suitable drug for decreasing intra ocular pressure. (1 drop daily in evening)
That's all
- Jaskunwar Singh
- Jaskunwar Singh
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