Test 36
(Matching Paper: Retina)

In response to considerable discussion the Examinations Committee of the College has decided to update the format of the Part 3 MRCOphth Examination from April, 2003.

An extended matching paper will replace the existing MCQs and they will be no negative marking. The test will probably be easier than the previous format. To familiarize the candidates with the new formats, test 34 and onward will follow the new style as described below by the College.
 

40 questions over a 3-hour period. The questions will be divided as follows. 5 pathology questions 5 microbiology questions 15 general ophthalmology questions 15 Medicine and Neurology in relation to ophthalmology Extended matching questions are multiple choice items organized into sets that use one list of items in the set. The extended matching set includes four components  1.A theme  2.an option list  3.a lead in statement  4.at least four item stems, as illustrated below  This will involve 40 questions (probably 200 stems) over a period of 3 hours.

1. Match the stem questions (a-d) with the options (A-N) listed below:
 

A. Leber's congenital amaurosis B. myotonic dystrophy C. Kearn-Sayre syndrome D. Wagner's syndrome E.  Stickler's syndrome  F.  Bardet-Biedl syndrome G. Refsum's disease

H. Friedreich's ataxia I.  Bassen-Kornzweig syndrome J.  Usher's syndrome K. gyrate atrophy L.  birdshot retinopathy M. punctate inner choriopathy N. Stargardt's disease

 

a.	A 30 year old man has cerebellar sign, deafness, elevated phytanic acid and  pigmentary retinopathy. Answer G. Refsum's disease is an autosomal recessive condition. The clinical findings  include a peripheral neuropathy, ataxia, an increase in cerebrospinal fluid  protein with a normal cell count, and retinitis pigmentosa. Elevated serum  phytanic acid is seen in all cases.
b.	A 15 year old girl has absence of low-density plasma lipoproteins with abnormal shape red blood cells and pigmentary retinopathy. Answer I. It is also known as abetalipoproteinaemia, it is an autosomal recessive disorder with malabsorption, absence of low-density plasma lipoproteins and star-shaped erythrocytes and pigmentary retinopathy similar to retinitis pigmentosa. Treatment is with low fat diet, and vitamin A, E and K.
c.	A 30 year old man has empty vitreous with myopia of -11.00D. He also has mid-facial hypoplasia and peripheral arthropathy. Answer E. Stickler's syndrome fits the description best. Wagner's syndrome has no facial  features or joint symptoms.
d.	A 19 year old woman has been wheel-chair bound since the age of 12 with  kyphoscoliosis, cerebellar signs and pigmentary retinopathy. Answer H. Friedreich's ataxia is a rare autosomal recessive disorder characterized by  cerebellar kyphoscoliosis, pes cavus, diabetes mellitus, optic atrophy and  pigmentary retinopathy. Most patients lose their mobility in their teens..

2. Match the stem questions (a-d) with the options (A-N) listed below:
 

A. Oguchi's disease B. Chediak-Higashi syndrome C. Hermansky-Pudlak syndrome D. Nettleship-Falls albinism E. Stargardt's disease F. Leber's congenital amaurosis G. congenital rubella

H.  DIDMOAD I.   gyrate atrophy J.   retinopathy of prematurity K.  Coat's disease L.  achromatopsia M. vitamin A defiency N.  Norrie's disease.

 

a.	A 20 year old man has poor vision for the past few years. Fundoscopy reveals white flecks in both eyes. Fluorescein angiography shows reduced fluorescence from the choroidal circulation.. Answer E. Stargardt's disease is the most common form of hereditary macular degeneration. It is characterized by white flecks in the the posterior segment and macular  abnormalities (atrophy often occurs at late stage). Fluorescein angiography typically shows dark choroid as a result of accumulation of abnormal materials in the RPE layer.
b.	A 17 year old boy has white hair and nystagmus. Ocular examination pink iris and  blond fundus. He is prone to recurrent infection.  Answer B. Chediak-Higashi syndrome consists of oculocutaneous albinism with immune  deficiency. The patient is susceptible to recurrent infection.
c.	A 25 year old man has poor vision at night. The ERG shows normal full-field ERG. The fundi show colour changes during dark adaptation. Answer A. Oguchi's disease is a form of congenital stationary night blindness. Patients  usually require 2 to 12 hours to attain normal dark-adapted rod thresholds  and show a characteristic change from a golden brown colour of the fundus in the light-adapted state to a fundus of normal colour in the dark-adapted state (the “Mizuo phenomenon”).
d.	A 2 year old boy has poor vision since the age of 5 months old with absent ERG and  poor pupillary reaction to light. He also has the tendency to rub his eyes with his  knuckers. Answer F. The criteria for the diagnosis of Leber's congenital amaurosis include visual  impairment before age 6 months, poor pupillary reactions, and markedly attenuated  or absent electroretinographic (ERG) responses. Eye rubbing is common and can result in endophthalmos due to orbital fat atrophy.

 

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