PERSISTENT HYPERPLASTIC VITREOUS

Persistent Hyperplastic Primary Vitreous (PHPV) is a congenital eye disorder characterized by the abnormal development of the vitreous body, which is the clear gel that fills the inside of the eye. It is a rare condition, affecting about one in every 10,000 to 20,000 infants.

Symptoms:

  • The most common symptom of PHPV is a white reflex in the pupil, known as leukocoria, which can be seen in photographs taken with a flash.
  • Strabismus, or crossed eyes, is another common symptom.
  • Nystagmus, or involuntary eye movements, may also be present.
  • PHPV can also cause severe visual impairment or blindness.

Causes:

  • PHPV is caused by the failure of the hyaloid artery, which is responsible for the development of the vitreous body, to regress during fetal development.
  • It can also be caused by a mutation in the PAX6 gene, which is responsible for the development of the eye.
  • PHPV can be inherited in an autosomal dominant manner, meaning that a child has a 50% chance of inheriting the condition if one parent is affected.

Diagnosis:

  • PHPV is usually diagnosed during a routine eye exam in infancy.
  • A thorough examination of the eye, including a dilated fundus examination, is necessary to confirm the diagnosis.
  • Imaging tests, such as ultrasonography or MRI, may also be used to confirm the diagnosis and determine the extent of the condition.

Treatment:

  • The treatment of PHPV depends on the severity of the condition.
  • In mild cases, observation and frequent follow-up examinations may be all that is needed.
  • In more severe cases, surgery may be necessary to remove the abnormal tissue and prevent further visual impairment.
  • Surgery is usually performed between the ages of 6 and 12 months, and the prognosis is generally good if the surgery is done early.
  • In some cases, it may be necessary to repair or remove the lens, and use of contact lenses or glasses may also be required to improve vision.

PHPV is a congenital disorder which requires early diagnosis and treatment in order to prevent blindness. Parents should be aware of the signs and symptoms of PHPV and seek prompt medical attention if they suspect their child may be affected.

Comments

Post a Comment

Popular posts from this blog

ABETALIPOPROTEINEMIA (BASSEN-KORNZWEIG SYNDROME) Retinopathy

Image
Abetalipoproteinemia, also known as Bassen-Kornzweig syndrome, is a rare genetic disorder that affects the body's ability to absorb and transport fats and fat-soluble vitamins. One of the ways that abetalipoproteinemia can affect the eyes is by causing retinitis pigmentosa, a condition that causes vision loss. Retinitis pigmentosa is a degenerative eye disease that affects the retina (the light-sensitive layer at the back of the eye). It is characterized by the gradual loss of photoreceptor cells (cells that detect light and send signals to the brain) and the accumulation of pigment in the retina. This can cause vision loss and may lead to blindness in severe cases. Abetalipoproteinemia can also cause optic atrophy, a condition in which the optic nerve (the nerve that carries visual information from the eye to the brain) becomes thin and weak. This can cause vision loss and other visual problems. In conclusion, abetalipoproteinemia can affect the eyes by causing reti...
Read more

What is myopia?

Image
Myopia is nearsightedness and this condition of eye occurs when the power of eye is increased from the normal powers of eye. The normal powers of eye is 58DS and the eye with >58DS eye power will be marked as myopic eye.  Definition   When parallel light rays coming from the infinity focus Infront of the retina while the accommodation should be at rest this condition of eye is known as myopia.
Read more

Scleritis

Scleritis 👌is a painful inflammation (swelling) the sclera. The tough, fibrous tissues of the sclera form a protective outer layer for the eye and make up 83 percent of the eye’s surface. In almost half of all cases, scleritis is associated with an underlying autoimmune disorder such as rheumatoid arthritis. There are two main types of scleritis: anterior and posterior. Anterior scleritis, the most common type, affects the front portion of the sclera. There are three types of anterior scleritis: Diffuse scleritis is the most common type and fortunately the most treatable. This type displays widespread redness and inflammation throughout the whole or a portion of the front portion of the sclera.     Nodular scleritis, is characterized by the presence of nodules , often tender to the touch, on the surface of the eye.     Necrotizing scleritis is the most severe form of anterior scleritis. It has the ability to destroy scleral tissues an...
Read more