Tag: Disorders Of Lens

Disorders Of Lens

DISORDERS OF LENS

Q. 1

Which type of Lenticonus is more common in males?

 A

Posterior

 B

Anterior

 C

Both anterior and posterior are equally common

 D

Inferior

Q. 1

Which type of Lenticonus is more common in males?

 A

Posterior

 B

Anterior

 C

Both anterior and posterior are equally common

 D

Inferior

Ans. B

Explanation:

Anterior lenticonus is most commonly seen in males. Posterior lenticonus is common in females.
 
Lenticonus:
It is a conical deformation in the center of either the anterior or posterior surface of the lens.
Anterior lenticonus can cause a pupillary block when when the pupil contracts round the protruding lenticonus.
Usually the lens is transparent, but polar opacities in the capsule and subcapsular zone can occur.
The central area of the lens is highly myopic due to the increased curvature in this condition, refraction in the periphery may be emmetropic.
Ophthalmoscopically, the area of lenticonus is seen as a separate greyish central round zone with a normal red glow surrounding it.
 
Ref: Clinical Opthalmology By Kalevar, Page 484

Q. 2

Which of the following syndrome is associated with ectopia lentis?

 A

Down’s

 B

Edward’s

 C

Marfan’s

 D

Turner’s

Q. 2

Which of the following syndrome is associated with ectopia lentis?

 A

Down’s

 B

Edward’s

 C

Marfan’s

 D

Turner’s

Ans. C

Explanation:

Dislocation of lens with systemic anomalies:
  • Marfans syndrome: In this condition, lens is displaced upwards and temporally (bilaterally symmetrical)
  • Homocystinuria: In it the lens is usually subluxated downwards and nasally
  • Weil-Marchesani syndrome: Forward subluxation of lens is seen
  • Ehlers-Danlos syndrome: In this, ocular features are subluxation of lens and blue sclera
  • Hyperlysinemia: Occasionally associated with ectopia lentis
  • Stickler syndrome: Occasionally associated with ectopia lentis
  • Sulphite oxidase deficiency: It is an autosomal recessive condition with ectopia lentis
Ref: Comprehensive Ophthalmology by AK Khurana, 4th edition, Page 202-203.

Q. 3

Ectopia lentis (eye lens dislocation) is seen in

 A

Marfan’s syndrome

 B

Congenital rubella

 C

Homocystinuria

 D

a and c

Q. 3

Ectopia lentis (eye lens dislocation) is seen in

 A

Marfan’s syndrome

 B

Congenital rubella

 C

Homocystinuria

 D

a and c

Ans. D

Explanation:

A i.e. Marfan’s syndrome ; C i.e. Homocystinuria

Quiz In Between


Q. 4

Which prominent ocular manifestation is associated with Marfan’s syndrome :

 A

Microcornea

 B

Microspherophakia

 C

Megalocornea

 D

Ectopia lentis

Q. 4

Which prominent ocular manifestation is associated with Marfan’s syndrome :

 A

Microcornea

 B

Microspherophakia

 C

Megalocornea

 D

Ectopia lentis

Ans. D

Explanation:

D i.e. Ectopia lentis


Q. 5

A 25 year old boy presents with renal failure.

His uncle died of renal failure three years ago.

Slit lamp examination reveals Lenticonus / Keratoconus.

The likely diagnosis is:

 A

Autosomal Dominant Polycystic Kidney (ADPCKD)

 B

Autosomal Recessive polycystic kidney (ARPCKD)

 C

Alport’s syndrome

 D

Denysh-Drash Syndrome

Q. 5

A 25 year old boy presents with renal failure.

His uncle died of renal failure three years ago.

Slit lamp examination reveals Lenticonus / Keratoconus.

The likely diagnosis is:

 A

Autosomal Dominant Polycystic Kidney (ADPCKD)

 B

Autosomal Recessive polycystic kidney (ARPCKD)

 C

Alport’s syndrome

 D

Denysh-Drash Syndrome

Ans. C

Explanation:

Answer is C (Alport’s syndrome)

Association of Renal failure with Lenticonus / Keratoconus with a positive family history suggests a diagnosis of Alport’s syndrome.


Q. 6

Anterior lenticonus is seen in:

 A

Marfan’s syndrome

 B

Ehler Danlos syndrome

 C

Weil-Marchessani syndrome

 D

Homocystinuria

Q. 6

Anterior lenticonus is seen in:

 A

Marfan’s syndrome

 B

Ehler Danlos syndrome

 C

Weil-Marchessani syndrome

 D

Homocystinuria

Ans. A

Explanation:

Ans. Marfan’s syndrome

Quiz In Between


Q. 7

Anterior lenticonus may be associated with:

 A

Alport’s syndrome

 B

Lowe’s syndrome

 C

Marfan’s syndrome

 D

Homocystinuria

Q. 7

Anterior lenticonus may be associated with:

 A

Alport’s syndrome

 B

Lowe’s syndrome

 C

Marfan’s syndrome

 D

Homocystinuria

Ans. A

Explanation:

Ans. Alport’s syndrome


Q. 8

A patient presents to the emergency department with uniocular diplopia. Examination with oblique illumination shows golden crescent while examination with co-axil illumination show a dark crescent line. Which of the following is the most likely diagnosis:

 A

Lenticonus

 B

Coloboma

 C

Microspherophakia

 D

Ectopia lentis

Q. 8

A patient presents to the emergency department with uniocular diplopia. Examination with oblique illumination shows golden crescent while examination with co-axil illumination show a dark crescent line. Which of the following is the most likely diagnosis:

 A

Lenticonus

 B

Coloboma

 C

Microspherophakia

 D

Ectopia lentis

Ans. D

Explanation:

Ans. Ectopia lentis


Q. 9

Ectopia lentis is seen in all of the following except:

March 2007

 A

Marfan’s syndrome

 B

Homocystinuria

 C

Weil-Marchesani syndrome

 D

Down syndrome

Q. 9

Ectopia lentis is seen in all of the following except:

March 2007

 A

Marfan’s syndrome

 B

Homocystinuria

 C

Weil-Marchesani syndrome

 D

Down syndrome

Ans. D

Explanation:

Ans. D: Down syndrome

Ectopia lentis is a displacement or malposition of the eye’s crystalline lens from its normal location.

More common:

  • Marf an syndrome
  • Homocystinuria
  • Weil-Marchesani syndrome
  • Sulfite oxidase deficiency
  • Hyperlysinemia

Less common:

  • Ehlers-Danlos syndrome
  • Crouzon disease
  • Refsum syndrome
  • Sturge-Weber syndrome
  • Pierre Robin syndrome

Quiz In Between


Q. 10

Ectopia lentis is seen in all except ‑

 A

Marfan syndrome

 B

EDS

 C

Trauma

 D

Osteogenesis imperfecta

Q. 10

Ectopia lentis is seen in all except ‑

 A

Marfan syndrome

 B

EDS

 C

Trauma

 D

Osteogenesis imperfecta

Ans. D

Explanation:

Ans. is ‘d’ i.e., Osteogenesis imperfecta


Q. 11

A 30 year old male suffered from eye trauma followed by red painful eye ,decreased distance visual acuity ,poor near vision  and monocular diplopia.This condition may lead to increased intraocular pressure and considered an ocular emergency .Identify this condition.

 A

Lenticonus

 B

Lentiglobus

 C

Coloboma

 D

Ectopia Lentis

Q. 11

A 30 year old male suffered from eye trauma followed by red painful eye ,decreased distance visual acuity ,poor near vision  and monocular diplopia.This condition may lead to increased intraocular pressure and considered an ocular emergency .Identify this condition.

 A

Lenticonus

 B

Lentiglobus

 C

Coloboma

 D

Ectopia Lentis

Ans. D

Explanation:

Ans:D.)Ectopia Lentis

The patient is suffering from traumatic inferior dislocation of lens:Ectopia Lentis.

Ectopia lentis

  • It is a displacement or malposition of the eye’s crystalline lens from its normal location. 
  • A partial dislocation of a lens is termed lens subluxation ; a complete dislocation of a lens is termed lens luxation .
  • Pathophysiology
    • Disruption or dysfunction of the zonular fibers of the lens.
  • Anterior lens luxation
    • With anterior lens luxation, the lens pushes into the iris or actually enters the anterior chamber of the eye. This can cause glaucoma, uveitis, or damage to the cornea. Anterior lens luxation is considered to be an ophthalmological emergency.
  • Posterior lens luxation
    • With posterior lens luxation, the lens falls back into the vitreous humour and lies on the floor of the eye. This type causes fewer problems than anterior lens luxation, although glaucoma or ocular inflammation may occur. 
  • Common presenting symptoms (visual disturbance) include the following:
    • Red painful eye (secondary to trauma)
    • Decreased distance visual acuity (secondary to astigmatism or myopia)
    • Poor near vision (loss of accommodative power)
    • Monocular diplopia.
  • Retinal detachment is one of the most serious consequences of a dislocated lens.
  • Cause:
    • Traumatic dislocation is the most common cause
    • Most common systemic conditions that are associated with ectopia lentis:
      • Marfan syndrome (bilateral,upward and outward)
        • Most frequent cause of heritable ectopia lentis. The syndrome is transmitted as an autosomal dominant trait 
        • Features include tall stature, arachnodactyly, joint laxity, mitral valve prolapse, aortic dilatation, axial myopia, and increased incidence of retinal detachment. 
      • Homocystinuria (bilateral,downward and inwards)
        • An inborn error of metabolism most often caused by a near absence of cystathionine b-synthetase (the enzyme that converts homocysteine to cystathionine). 
        • Patients typically have fair skin with coarse hair, osteoporosis, mental retardation (nearly 50%), seizure disorder, marfanoid habitus, and poor circulation.
      • Weill–Marchesani syndrome(downwards)
        • Characterized by skeletal malformations (eg, short stature, brachycephaly, limited joint mobility, well-developed muscular appearance) and ocular abnormalities (eg, ectopia lentis, microspherophakia, lenticular myopia). 
      • Sulfite oxidase deficiency
      • Hyperlysinemia

Q. 12

A 28 years old man has anterior lenticonus and ESRD now. His maternal uncle also died of similar illness. Diagnosis is

 A

ARPKD

 B

ADPKD

 C

Oxalosis

 D

Alport’s syndrome

Q. 12

A 28 years old man has anterior lenticonus and ESRD now. His maternal uncle also died of similar illness. Diagnosis is

 A

ARPKD

 B

ADPKD

 C

Oxalosis

 D

Alport’s syndrome

Ans. D

Explanation:

Ans. d. Alport’s syndrome

  • The given clinical picture is typical of Alport’s syndrome, which is characterized by triad of hereditary nephritis (ESRD), sensorineural deafness and ocular abnormalities (anterior lenticonus).

Alport’s Syndrome (Characterized by triad of)

Hereditary Nephritis

  • Hematuria (earliest feature)Q
  • Mild ProteinuriaQ
  • Chronic glomerulosclerosis
  • Progressive renal insufficiency
  • Nephrotic syndrome (rare)

Sensorineural Deafness

  • MC extra-renal abnormalityQ
  • AsymmetricQ

Ocular Abnormalities

  • Anterior lenticonusQ
  • Dot and Fleck retinopathy
  • Recurrent corneal erosionsQ and opacitiesQ
  • CataractQ
  • Retinal detachment
  • Nystagmus
  • Leiomyomatosis

Treatment of Alport’s Syndrome

  • Primary treatment is control of systemic hypertension and use of ACE inhibitors to slow renal disease progression

Quiz In Between


Q. 13

Posterior lenticonus is seen in

 A

Alport’s syndrome

 B

Lowe’s syndrome

 C

Marfan syndrome 

 D

Homocystinuria

Q. 13

Posterior lenticonus is seen in

 A

Alport’s syndrome

 B

Lowe’s syndrome

 C

Marfan syndrome 

 D

Homocystinuria

Ans. B

Explanation:

Ans. is ‘b’ i.e., Lowe’s syndrome 

  • Lenticonus refers to cone-shaped elevation of the anterior pole (anterior lenticonus) or posterior pole (posterior
  • lenticonus) of the lens.
  • Anterior lenticonus is seen in Alport’s syndrome.
  • Posterior lenticonus is seen in Lowe’s syndrome.
  • On distant direct ophthalmoscopy, lenticonus present as an oil globule lying in the center of red reflex.
  • Slit-lamp examination confirms the diagnosis.

Q. 14

A young tall, thin male with arachnodactyly has ectopia lentis in both eyes. most likely diagnosis is

 A

Marfan’s syndrome

 B

Homocystinuria

 C

Ehler’s-Danlos Syndrome

 D

Rheumatoid arthritis

Q. 14

A young tall, thin male with arachnodactyly has ectopia lentis in both eyes. most likely diagnosis is

 A

Marfan’s syndrome

 B

Homocystinuria

 C

Ehler’s-Danlos Syndrome

 D

Rheumatoid arthritis

Ans. C

Explanation:

Ehler’s-Danlos syndrome

  • Hyperelasticity of skin (Rubber Person Syndrome)
  • Hypermobile joints
  • Affect Type V Collagen and Type I Collagen.
  • arachnodactyl

Quiz In Between



Disorders Of Lens

DISORDERS OF LENS


DISORDERS OF LENS

Ectopic lentis

  • Defined as displacement or malposition of the crystalline lens of the eye.
  • The lens is considered dislocated (luxated) when it lies completely outside the lens patellar fossa.
  • The lens is described as subluxated when it is partially displaced but contained within the lens space.

Causes are:

  1. Homocystinuria
  2. Marfan syndrome
  3. Weil-Marchesani syndrome
  4. Ehler Danlos syndrome
  5. Consecutive/ spontaneous
  6. Hyperlysinemia
  7. Sulphite oxidase deficiency
  8. Stickler syndrome
  9. Trauma
DISEASE  TYPICAL LENS DISLOCATION
Marfan syndrome Superotemporal
Homocystinuria Inferonasal
Weil-Marchesani syndrome Forward
  • Clinical features includes unicular diplopia, with irregular depth of anterior chamber.
  • Slit lamp examination evaluates lens position & identifies phacodonesis or cataract.
  • Slit lamp (oblique illumination) typically shows the edge of the lens as shining (bright) golden crescent.
  • Coaxial illumination (Direct ophthalmoscopy) shows the edge of subluxated lens as a dark crescent line.
  • On retinoscopy the aphakic area is hypermetropic while the phakic area is generally shows index myopia.

Fundus shows two discs:

  1. One large through the phakic area
  2. one small through the aphakic area

Congenital abnormalities

1. Coloboma of the lens:

  • A notch is seen in the inferior quadrant of the equator.
  • Occurs due to defective developmen of the suspensory ligament in that part.

2. Anterior lenticonus:

  • Cone-shaped anterior axial bulge.
  • May occur in Alport’s syndrome.

3. Posterior lenticonus:

  • Posterior axial bulge.
  • May occur in Lowe’s syndrome

4. Micro-spherophakia:

  • A small spherical lens may occur as an isolated finding or as a feature of Weip Marchessano’s or Marfan’s syndrome.

5. Microphakia is a small lens which occur in Lowe’s syndrome.

6. Lentiglobus: Generalised hemi-spherical deformity.

Exam Important

  • Ectopia lentis is associated with Marfan’s syndrome.

Causes of Ectopia lentis are:

  1. Homocystinuria
  2. Marfan syndrome
  3. Weil-Marchesani syndrome
  4. Ehler Danlos syndrome
  5. Sulphite oxidase deficiency
  • Posterior lenticonus is seen in Lowe’s syndrome.
  • Anterior lenticonus associated with Alport’s syndrome.
  • Anterior Lenticonus is more common in males.
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