Test 11  (paediatric ophthalmology) 

1. Causes of poor vision in aniridia include:

a. macular hypoplasia

b. glaucoma

c. cataract

d. retinitis pigmentosa

e. optic nerve hypoplasia

Answera.T   b.T  c.T  d.F  e.T
Aniridia typically presents with poor fixation and nystagmus.
Causes of poor vision include:
cornea pannus
glaucoma
cataract
macular hypoplasia
optic nerve hypoplasia

2. True statements about aniridia include:

a. it is sporadic in one third of cases

b. familial cases are autosomal dominant

c. vision can be improved with tinted glasses

d. Wilm's tumour occurs in one third of sporadic aniridia

e. Wilm's tumour does not occur in familial aniridia

Answera.F   b.F   c.T  d.T  e.F
Two third of aniridia are inherited in an autosomal 
dominant fashion. Wilm's tumour though reported
in familial cases are extremely rare. But it affects  as many as one third of sporadic cases.
Photophobia is common in aniridia and tinted glasses or contact lenses are useful and can improve vision.

3. The following features are seen in Leber's congenital amaurosis:

a. nystagmus

b. normal fundus at the early stage

c. myopic refraction

d. absent pupil responses to light

e. arteriolar narrowing in later stage

Answera.T   b.T  c.F  d.T  e.T
Leber's congenital amaurosis is an autosomal recessive condition.
Nystagmus, absent pupil responses to light and a hypermetropic refraction of greater than 3D are common features.
The fundus may be normal at presentation but with time arteriolar narrowing, optic atrophy and arteriolar narrowing occurs.

4. Chickenpox in expectant mother can produce the following
     ocular abnormalities in the newborn:

a. Horner's syndrome

b. optic nerve hypoplasia

c. aniridia

d. chorioretinitis

e. cataract

Answera.T  b.T  c.F  d.T   e.T
Intrauterine infection with chickenpox virus (varicella) can give rise to:
Horner's syndrome
cataract
optic nerve hypoplasia
microphthalmia

5. In de Morsier's syndrome:

a. the condition is hereditary in the majority of cases

b. optic atrophy is a feature

c. agenesis of the septum pellucidum is a feature

d. the corpus callosum may be malformed.

e. hypothalamic dysfunction is common

Answera.F   b.F  c.T  d.T  e.T
de Morsier's syndrome = optic nerve hypoplasia
+ agenesis of corpus callosum or septum pellucidum.
Endocrine problem is common due to abnormal hypothalamic function.

 
 

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