Congential Optic Nerve Anomalies MBBS Notes | EduRev

Created by: Appar Malik

MBBS : Congential Optic Nerve Anomalies MBBS Notes | EduRev

 Page 1


CONGENITAL OPTIC NERVE ANOMALIES 
1.  Without systemic associations 
2.  With systemic associations 
•  Optic disc coloboma 
•  Morning glory anomaly 
•  Optic nerve hypoplasia 
•  Aicardi syndrome 
•  Megalopapilla 
•  Peripapillary staphyloma 
•  Optic disc dysplasia 
•  Tilted optic disc 
•  Optic disc drusen 
•  Optic disc pit 
•  Myelinated nerve fibres 
Page 2


CONGENITAL OPTIC NERVE ANOMALIES 
1.  Without systemic associations 
2.  With systemic associations 
•  Optic disc coloboma 
•  Morning glory anomaly 
•  Optic nerve hypoplasia 
•  Aicardi syndrome 
•  Megalopapilla 
•  Peripapillary staphyloma 
•  Optic disc dysplasia 
•  Tilted optic disc 
•  Optic disc drusen 
•  Optic disc pit 
•  Myelinated nerve fibres 
Tilted disc 
•  Common, bilateral 
•  Frequent myopia and astigmatism 
•  V A - normal 
•  Small disc, oval or D-shaped 
•  Axis oblique (most common),  
   horizontal or vertical 
•  Situs inversus and inferior crescent 
•  Hypopigmented inferonasal fundus 
•  Superotemporal field defects 
    not obeying vertical midline 
Page 3


CONGENITAL OPTIC NERVE ANOMALIES 
1.  Without systemic associations 
2.  With systemic associations 
•  Optic disc coloboma 
•  Morning glory anomaly 
•  Optic nerve hypoplasia 
•  Aicardi syndrome 
•  Megalopapilla 
•  Peripapillary staphyloma 
•  Optic disc dysplasia 
•  Tilted optic disc 
•  Optic disc drusen 
•  Optic disc pit 
•  Myelinated nerve fibres 
Tilted disc 
•  Common, bilateral 
•  Frequent myopia and astigmatism 
•  V A - normal 
•  Small disc, oval or D-shaped 
•  Axis oblique (most common),  
   horizontal or vertical 
•  Situs inversus and inferior crescent 
•  Hypopigmented inferonasal fundus 
•  Superotemporal field defects 
    not obeying vertical midline 
Optic disc drusen 
•  Uncommon, bilateral and familial 
•  Associations - RP, angioid streaks and Alagille syndrome 
•  VA - usually normal 
Buried drusen 
•  Absent optic cup  
•  Pink or yellow colour 
•  Indistinct ‘lumpy’ margins 
•  Anomalous branching patterns  
   with premature branching 
•  Absent venous engorgement  
Page 4


CONGENITAL OPTIC NERVE ANOMALIES 
1.  Without systemic associations 
2.  With systemic associations 
•  Optic disc coloboma 
•  Morning glory anomaly 
•  Optic nerve hypoplasia 
•  Aicardi syndrome 
•  Megalopapilla 
•  Peripapillary staphyloma 
•  Optic disc dysplasia 
•  Tilted optic disc 
•  Optic disc drusen 
•  Optic disc pit 
•  Myelinated nerve fibres 
Tilted disc 
•  Common, bilateral 
•  Frequent myopia and astigmatism 
•  V A - normal 
•  Small disc, oval or D-shaped 
•  Axis oblique (most common),  
   horizontal or vertical 
•  Situs inversus and inferior crescent 
•  Hypopigmented inferonasal fundus 
•  Superotemporal field defects 
    not obeying vertical midline 
Optic disc drusen 
•  Uncommon, bilateral and familial 
•  Associations - RP, angioid streaks and Alagille syndrome 
•  VA - usually normal 
Buried drusen 
•  Absent optic cup  
•  Pink or yellow colour 
•  Indistinct ‘lumpy’ margins 
•  Anomalous branching patterns  
   with premature branching 
•  Absent venous engorgement  
Optic disc drusen 
Occasional complications 
•  Emergence  from disc surface during 
    early ‘teens’  
•  Waxy pearl-like irregularities 
•  Choroidal neovascularization 
•  Nerve fibre bundle defects 
Exposed  
Page 5


CONGENITAL OPTIC NERVE ANOMALIES 
1.  Without systemic associations 
2.  With systemic associations 
•  Optic disc coloboma 
•  Morning glory anomaly 
•  Optic nerve hypoplasia 
•  Aicardi syndrome 
•  Megalopapilla 
•  Peripapillary staphyloma 
•  Optic disc dysplasia 
•  Tilted optic disc 
•  Optic disc drusen 
•  Optic disc pit 
•  Myelinated nerve fibres 
Tilted disc 
•  Common, bilateral 
•  Frequent myopia and astigmatism 
•  V A - normal 
•  Small disc, oval or D-shaped 
•  Axis oblique (most common),  
   horizontal or vertical 
•  Situs inversus and inferior crescent 
•  Hypopigmented inferonasal fundus 
•  Superotemporal field defects 
    not obeying vertical midline 
Optic disc drusen 
•  Uncommon, bilateral and familial 
•  Associations - RP, angioid streaks and Alagille syndrome 
•  VA - usually normal 
Buried drusen 
•  Absent optic cup  
•  Pink or yellow colour 
•  Indistinct ‘lumpy’ margins 
•  Anomalous branching patterns  
   with premature branching 
•  Absent venous engorgement  
Optic disc drusen 
Occasional complications 
•  Emergence  from disc surface during 
    early ‘teens’  
•  Waxy pearl-like irregularities 
•  Choroidal neovascularization 
•  Nerve fibre bundle defects 
Exposed  
FA of optic disc drusen  
Autofluorescence prior to dye injection 
Late hyperfluorescence confined to disc 
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