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Spot the fundoscopic finding

Discussion in 'Spot Diagnosis' started by neo_star, Feb 15, 2013.

  1. neo_star

    neo_star Moderator

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    Fundoscopy of an infant whose mother had a bout of viral illness with rash during first trimester.

    fundus of child.jpg

    Can you identify the condition ?
     

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    Last edited: Feb 16, 2013

  2. dupuytren

    dupuytren Bronze Member

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    salt and pepper retinopathy in congenital rubella
     

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  3. Emergency medicine Mike

    Emergency medicine Mike Bronze Member

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    Congenital rubella.
     

  4. neo_star

    neo_star Moderator

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    Answer: "Salt-and-pepper" retinopathy as a manifestation of "Congenital Rubella Syndrome".

    Discussion


    Congenital rubella is a multisystem disorder resulting from exposure of the fetus to maternal rubella during the first trimester of pregnancy.
    If infection occurs 0”“28 days before conception, there is a 43% chance the infant will be affected. If the infection occurs 0”“12 weeks after conception, there is a 51% chance the infant will be affected. If the infection occurs 13”“26 weeks after conception there is a 23% chance the infant will be affected by the disease.
    Infants are not generally affected if rubella is contracted during the third trimester, or 26”“40 weeks after conception.


    The classic triad for congenital rubella syndrome is:

    • Sensorineural deafness (58% of patients)
    • Eye abnormalities—especially retinopathy, cataract and microphthalmia (43% of patients)
    • Congenital heart disease—especially patent ductus arteriosus (50% of patients)

    "Salt and-pepper" retinopathy of the retina is the most common ocular manifestation of congenital rubella.

    Other manifestations of CRS may include:


    • Spleen, liver or bone marrow problems (some of which may disappear shortly after birth)
    • Mental retardation
    • Small head size (microcephaly)
    • Eye defects
    • Low birth weight
    • Thrombocytopenic purpura (presents as a characteristic blueberry muffin rash)
    • Hepatomegaly
    • Micrognathia

    ref - Congenital rubella syndrome - Wikipedia, the free encyclopedia

    "Salt-and-pepper" retinopathy


    This fine speckling of the retina is the commonest ocular manifestation of congenital rubella. It is called "salt-and-pepper" because there are tiny flecks of dark pigment mixed with fine areas of whitish depigmentation. Usually the pigment alteration is diffuse, but it may be most prominent either around the macula or in the retinal periphery.These signs reflect diffuse damage to the retinal pigment epithelium (RPE).

    (Y) The good news is that the RPE is never damaged enough to interfere with vision or to cause major abnormalities in the electroretinogram (ERG), the standard objective test used to evaluate outer retinal function.

    The preservation of vision and a relatively or completely normal ERG allows you to differentiate rubella retinopathy from the hereditary retinal degenerations called retinitis pigmentosa.

    Other causes of "salt-and-pepper" retinopathy are congenital syphilis, and toxicities of systemically administered thioridazine, choroquine, and deferoxamine.

    Other ophthalmic complications include microphthalmia (small eye), cataract, glaucoma, corneal opacification, and uveitis. Vision is usually quite poor from these problems.

    ref - The Eyes Have It: Congenital and Hereditary Systemic Diseases


    How to prevent "Congenital Rubella Syndrome"

    Ans: Vaccination

    Just one dose a month before conception. Now if you are considering Varivax ( for chickenpox )....then give varivax first, wait for about 6 weeks and then give Rubella vaccine.

    Controversies and how to practice what is safe....the chickenpox vaccine is particularly notorious for the vaccine virus causing an infection and establishing latency in the ganglia with attacks of Zoster in the future. So don't give it to anybody who is immunosuppressed; if the immunosuppression is transient, then wait for the patient to recover completely and then give the vaccine.
    Secondly some docs give MMR and chicken pox together and i guess Merck has combined all the four into a single vaccine...now there are those who argue that the four given together actually has a greater immune response than when given individually...it's completely true for DPT with all the 3 components being componets / antigens of the inactivated organisms and u additionally need adjuvants + booster doses for the immune system to respond adequately esp. in infants when the immune system is just about learning the skills.
    But not in the case of live viruses...here eventhough the viruses are attenuated, they do induce a strong immune response and actually need the full individual attention of the immune system esp. the chickenpox virus. In this case an MMR given together does not act as a force multiplier, but rather it's akin to the immune system having to open up many battle fronts and may succumb to one of the guys..mostly the varicella component.


    Bottomline - To prevent "CRS" give rubella vaccine 1 month b4 conception and if u wish to give Varivax then ensure a separation of 6 weeks between the two.


     

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  5. Rocket Queen

    Rocket Queen Super Moderator

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    Congenital rubella
     

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