Question
A 10 year old boy presented with frequently changing the prescription of eye glasses,not associated with eye pain,redness or night blindness.Biomicroscopically,the lens is giving a picture as shown in the image ,bilaterally and retro-illumination shows an oil droplet configuration.He is also suffering from hearing problem and blood in the urine.Which of the following syndrome is associated with this condition ?
A. Down’s syndrome
B. Alport’s syndrome
C. Marfan’s syndrome
D. Lowe’s syndrome
Show Answer
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Correct Answer » B
Explanation
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Ans:B. Alport’s syndrome.
The patient is suffering from Bilateral Anterior Lenticonus.
Lenticonus
- It is a congenital anomaly of the eye characterized by a conical protrusion on the crystalline lens capsule and the underlying cortex that can reach a diameter of 2 to 7 mm.
- If the bulging is spherical, instead of conical, the condition is referred to as lentiglobus.
- It produces a decrease in visual acuity and irregular refraction that cannot be corrected by either spectacle or contact lenses
- Biomicroscopically lenticonus is characterized by a transparent, localized, sharply demarcated conical projection of the lens capsule and cortex, usually axial in localization.
- In an early stage, retro-illumination shows an oil droplet configuration.
- Using a narrow slit, the image of a conus is observed.
- Retinoscopically the oil droplet produces a pathognomonic scissors movement of the light reflex. This phenomenon is due to the different refraction in the central and the peripheral area of the lens.
- A-scan ultrasonography may reveal an increased lens thickness and B- scanultrasonography may show herniated lenticular material, suggestive of a lenticonus.
- Types
- Lenticonus anterior:
- Bilateral,Males > Females, associated with Alport syndrome.
- Alport syndrome encompasses a group of inherited, heterogeneous disorders involving the basement membranes of the kidney and frequently affecting the cochlea and eye as well(Anterior Lenticonus,Hereditary nephritis,Deafness)
- Cataract,flecked retinopathy, posterior polymorphous dystrophy and corneal arcus juvenilis may be encountered in association with lenticonus anterior that occurs as a part of the Alport syndrome.
- Anterior lenticonus is not present at birth but is manifested by a slowly progressive deterioration of vision, requiring patients to change the prescription of their glasses frequently. The condition is not accompanied by eye pain, redness, or night blindness, and no defect in color vision occurs.
- Lenticonus posterior:
- Unilateral,sporadis,female> male
- lenticonus posterior is more common than lenticonus anterior and is sometimes found in Lowe syndrome.
- Amblyopia, cataract, strabismus and loss of central fixation may be observed in association with lenticonus posterior.
