0:00

Thus far, we have reviewed the traumatic injuries and

0:07

infectious inflammatory injuries of the globe.

0:12

This next entity,

0:13

Persistent Hyperplastic Primary Vitreous,

0:16

sort of crosses the boundaries between a congenital

0:19

abnormality as well as a vascular abnormality

0:22

when we're using our vitamin C and D mnemonic.

0:27

These are two different patients who have PHPV.

0:32

Both of the patients show microphthalmos on the left side.

0:37

In both of the cases,

0:39

one sees a vertically oriented line which leads from the

0:46

region of the retina anteriorly to the lens of the eye.

0:52

This is the persistence of the embryonic

0:56

hyaloid vascular system,

0:58

the hyaloid artery in what is known as Cloquet's canal.

1:08

PHPV is discovered in infants who demonstrate

1:14

leukocoria, as well as microphthalmos.

1:18

However,

1:19

these patients may also present with retinal hemorrhages.

1:25

Because of the abnormal development of the

1:28

choroid and retina in these patients,

1:30

the retinal hemorrhages may not follow the typical

1:35

location that we say with regard to 10 o'clock

1:38

and 2 o'clock along the globe.

1:40

In this case,

1:41

the upper image shows an area of blood products

1:46

in the affected left globe which extends far anteriorly,

1:52

greater than expected for our typical retinoblastoma,

1:55

which should end at the ora serrata

1:58

at 10 o'clock and 2 o'clock

2:00

As you can see,

2:02

the lens of the eye and the ciliary apparatus is generally

2:07

abnormally developed in patients who have PHPV.

2:11

And therefore, retinal detachments may proceed further

2:16

anteriorly than typical in a normal eye.

2:21

To distinguish this lesion from retinoblastoma,

2:24

remember we say that PHPV does not calcify and is usually

2:29

associated with a small globe as opposed to the most

2:33

common cause of leukocoria, retinoblastoma,

2:36

which is associated with a normal-sized

2:38

globe or slightly larger globe.

2:42

The abnormal lens is another factor that

2:45

distinguishes PHPV from retinoblastoma,

2:49

which has a normal lens of the eye.

2:52

The next congenital anomaly that we wish to speak

2:56

about is the coloboma.

2:58

A coloboma is a congenital

3:00

anomaly where there is a defect in the

3:02

retina and the ocular membranes.

3:05

This is typically projecting into the insertion site of

3:10

the optic nerve.

3:11

Although, you can have colobomas,

3:14

medially and laterally located.

3:18

Colobomas are diseases that we see in infancy

3:23

and they are to be distinguished from the other entity

3:26

that may cause an enlarged,

3:29

elongated globe, the staphyloma.

3:33

Staphyloma is seen on these images.

3:36

Once again, with staphyloma,

3:39

we see thinning of the uveoscleral membrane

3:45

as opposed to the normal thickness

3:48

of those ocular membranes.

3:51

And usually, staphylomas project towards

3:54

the temporal side of the globe.

3:57

The most common cause of staphyloma is axial myopia.

4:02

However, one can see it after trauma

4:05

or endophthalmitis, anything that causes weakening

4:08

of the uveoscleral membranes.

4:11

You may also see this with rheumatoid arthritis

4:14

and other collagen vascular diseases.