ONH - CONGENITAL OPTIC NEUROPATHIES Flashcards
what is ON hypoplasia?
abnormally small ONH characterized by a reduced number of optic nerve fibers.
who gets ON hypolasia?
- seen in children whose mother either had an endocrine disorder (DM) or abused drugs (quinine, alcohol, LSD) during pregnancy.
symptoms of ON hypoplasia?
vision loss ranging from mild to sever (20/20 - NLP)
signs of ON hypoplasia?
- unilateral or bilateral
- small disc (double ring sign)
- severe – roving eye & min pupillary rxn
tx for ON hypoplasia?
- no tx
- refer to low vision specialist
what is morning glory disc syndrome?
congenital malformation of the disc
who gets morning glory disc?
- females who have midline facial defects & forebrain anomalies
symptoms of morning glory?
severe vision loss in the afflicted eye(s)
signs of morning glory disc?
- enlarged funnel shaped optic nerve with overlying glial tissue (glial hyperplasia).
- BV leave the nerve in spoke-like pattern
- ON surrounded by elevated pigment
- secondary serous RD
complication of morning glory disc?
- CNVM
- serous RD
tx for morning glory disc?
- no tx
- refer to low vision
what is optic nerve drusen?
congenital anomaly which result in drusen (hyaline bodies) buried in the nerve fiber layer surrounding the optic nerve.
symptoms of ON drusen?
asymptomatic or can have enlarged blind spot
signs of ON drusen?
- bilateral
- ONH will look swollen with possible blurred margins
- hyaline material may look like refractile clumps
how to dx/ddx ON drusen from actual edema?
- B-scan
- FAF
- OCT
what is optic nerve pit?
- gray, white, or yellow oval or orund depression in the optic nerve.
symptoms of optic disc pit?
asymptomatic unless serous macular detachment forms.
signs of optic disc pit?
- temporal gray-white depression of the optic nerve which can range from 0.1-0.7 DD in size.
- possible serous detachment
tx for optic disc pit?
- asymptomatic – monitor yearly.
- serous RD – observe if not in the macula & laser photocoagulation if in the macula & monitor monthly.
what is tilted disc syndrome?
it is characterized inferonasal tilting of the optic disc
where is the neural tissue concentrated in tilted disc syndrome?
- concentrated at the superior and superior-temporal aspect
- deficient at the inferior & inferior-nasal aspect
what is the most common VF defect in a tilted disc?
- superior bitemporal defects
what is tilted disc syndrome associated with?
high myopia
signs of Tilted disc syndrome?
- bilateral (common)
- inferonasal ON tilt
- situ inversus
