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IRIS COLOBOMA

Writer's picture: Munib ur RehmanMunib ur Rehman

FACE AND FACIAL SYMMETRY

Ear Deformities – CHARGE SYNDROME

Choanal Atresia – CHARGE SYNDROME

Microcephaly – MECKEL GRUBER SYNDROME

Facial Defects - MECKEL GRUBER SYNDROME

 

 

EXTRAOCULAR MOTILITY

Esotropia if Macula is involved          

 

IRIS AND PUPIL

The defect in the iris is the shape of a keyhole. ‘Typical’ colobomas occur in the inferonasal quadrant, whereas those occurring in any other location are termed ‘atypical.’

 

LENS

Lens coloboma is seen as the loss of zonular fibers in the area with the resultant flattening of the equatorial lens. They are mostly asymptomatic and appear only on dilation of the pupil.

Cataract may be seen as associated with retino-choroidal colobomas.

 

POSTERIOR SEGMENT

There may be associated colobomas of the optic nerve, retina, and choroid.

RETINA – Retinal involvement is seen as the replacement of the neurosensory retina to glial tissue with no underlying RPE or choroid. This appears as an area of whitening, often with pigment deposition at the junction of the coloboma normal retina. Breaks in the retina should be carefully screened out as there is an increased chance of their occurrence, also it is quite challenging to identify them because of the lack of underlying contrast.

 

FELLOW EYE

Usually bilateral condition

 

SYSTEMIC CONDITIONS

CHARGE SYNDROME

C - Coloboma

H – Heart Defects

A – choanal Atresia

R – Retarded Growth

G – Genital Defects

E – Ear Abnormalities.

 

MECKEL GRUBER SYNDROME

Microcephaly

Occipital encephalocele

Congenital heart defects

Polydactyly

Facial clefts

Polycystic renal and hepatic diseases.

 

Sjogren-Larsson Syndrome

Microphthalmos

Mental retardation

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