What is Aphakia and its treatment

What is Aphakia? What is the difference between aphakia and pseudophakia? How do you manage a case of uniocular aphakia after cataract operation due to trauma? (on a patient of 20years age)

Definition:-
Literally, Aphakia means the absence of the
the crystalline lens in the eyeball. Optically it
means absence of the crystalline lens from its
normal anatomical position in the pupillary
area. Aphakia may be due to surgical removal, a perforating wound or ulcer, or congenital anomaly. It causes a loss of accommodation, hyperopia, and a deep anterior chamber. Complications include detachment of the vitreous or retina and glaucoma.


Aphakia
Pseudophakia
What is it?
Aphakia means the absence of the
the crystalline lens in the eyeball. Optically it
means absence of the crystalline lens from its
normal anatomical position in the pupillary
area.
An eye in which the natural lens is replaced with an intraocular lens
Diagnosis
3rd  and 4th Purkinje image absent, iridodonesis present, increased anterior chamber depth, Black jet pupil
3rd and 4th Purkinje image present bright glassy reflex present behind the pupil, mild iridodonesis and slight increase in anterior chamber depth present.
Conditions where it happens
Congenital,Traumatic,As a complication of cataract surgery(Nowadays IOL implantation is the preferred method along with cataract surgery; excepting some conditions like microphthalmos, recurrent uveitis, severe subluxated lens)
As a cataract surgery procedure.
IOL maybe present
(IOL maybe used to correct refractive errors i.e.:-Phakic IOL, Ocular albinism)
Visual Acuity
Hardly 1/60 without glass
Vision improves, May be normal. Depends upon the implanted IOL
Refractive Status
high hypermetropia with astigmatism
Variable refractive error(depends upon the implanted IOL)
Retinoscopy
Poor glow
Normal
Near vision
Requires glass
Requires glass; but in case of multifocal lens used, may not require


TREATMENT OF the case:-
The person In the question is a 20year old patient(Young) and he became aphakic after operation for traumatic cataract. It is not known whether the person was previously suffering from any refractive error or not. So there are three possibilities
a)      The non-traumatic eye may be emmetropic
b)     The non-traumatic eye may be myopic
c)      The non-traumatic eye may be hypermetropic.
These are important in making a decision regarding treatment policy of the patient.
Uncorrected unilateral aphakia is one of the leading causes of monocular visual impairment. The treatment options are as follows:-

A)Spectacles.
B)Contact lenses.
C)Surgery on the cornea – to change the shape of the cornea.
D) An intraocular lens(Secondary)
1)Posterior chamber intraocular lens implantation
2)Iris Claw lens
3) Anterior chamber Intraocular lens implantation


Before starting the discussion regarding treatment options, let us consider the optics and optical defects in Aphakia

Optics and Optical defects of Aphakia:-
1)      The eye becomes highly hypermetropic
2)      Total power of the eye is reduced to about +44 D from +60 D.
3)      The anterior focal point becomes 23.2 mm in front of the cornea
4)      The posterior focal point is about 31 mm behind the cornea,i.e about 7mm behind the eyeball. (The anteroposterior length of the eyeball is about 24 mm)
5)      The two principal points are almost at the anterior surface of the cornea
6)      The nodal points are very near to each other and are located about 7.75mm behind the anterior surface of cornea.
7)      There is a total loss of accommodation
8)      Associated with high astigmatism
A)Aphakic Spectacles:
To correct the refractive error in aphakia about 10 dioptres of convex lenses are required for distance vision and about 13 dioptres for near vision along with correction for surgically induced astigmatism(Assuming that the patient was emmetropic). Such high power lenses are associated with numerous physical and optical problems. The most important of these problems are:
1.             Magnification: Each dioptre of convex power leads to about 3 % magnification of the image and a the difference of image size between the two eyes(aniseikonia) of about 5 % is tolerable. Thus 10 dioptre aphakic spectacles lead to about 30 % magnification of the image which gives rise to diplopia i.e., two images of one object are seen one small (from the normal eye) and other larger (from the aphakic eye). Moreover, when the objects appear larger they appear falsely closer than reality, and this leads to physical in-coordination(spatial disorientation or disturbances in-depth perception).
2.             Roving Ring Scotoma: The edge of a convex lens acts as a prism and the higher the power of the convex lens the greater is the prism angle. In aphakic spectacles, the prism angle being large, the light falling at the edge of the lens bends towards the centre of the lens (base of the prism) and does not reach the pupil and is, therefore, not seen. This results in an area of the visual field which is not visible to the patient, or scotoma. And because the edge of the lens is present all around the lens like a ring, so it gives rise to a ring-shaped scotoma. The position of this scotoma is not fixed in the visual field because the eye keeps moving (or roving) in relation to the aphakic spectacle. Hence, the result is a roving ring scotoma. 
3.             Jack-in-the-box Phenomenon: The presence of the above scotoma leads to another interesting phenomenon. If an interesting object appears in the periphery of the patients visual field, it appears blurred (because the light is passing from the side of the spectacle frame). The person tends to move his head towards the object in order to see it clearly. But as he turns the head the object comes to lie in the area of scotoma and thus disappears. As he turns his head further so that the object comes to lie in front of the spectacle in the visible area and so reappears again clear and sharp. This sudden disappearance and sharp reappearance of the objects is called jack-in-the-box phenomenon. 
4.             Pin Cushion Effect: The magnification of image is more at the periphery of the lens due to prism effect. Therefore, all the objects appear stretched out at the corners like apin-cushion. 
5.             Spherical Aberrations: The light converges more near the edge of the lens than at the center so the rays of light falling near the edge are brought to focus in front of the rays falling at the center. This results poor quality of image despite appropriate correction of refractive error.
6.             Chromatic Aberrations: The shorter the wave-length the more is the refraction a ray of light undergoes. Therefore, in VIBGYOR the violet end undergoes greater refraction than the red end of the spectrum. This causes diffraction of light and makes the edges of white object appear rain-bow colored.
7.             Restricted field of vision
8.             Coloured vision:- Occurs due to the absence of natural filter of a crystalline lens and due to chromatic aberration
9.             Cosmetic blemish:-Due to thick glasses
10.           Cumbersome to use:- as the glass is very thick and heavy
11.           Problem in near vision:-Thick bifocal glasses are specially difficult to adjust with.
As our patient in the question is a young patient with uniocular high hypermetropia,spectacle correction is not possible in this patient due to above stated problems with aphakic glass correction.

Contact Lens:-
Contact lens use is an alternative for young patient with unilateral high hypermetropia because of the following reasons:-

Advantages Of contact lens:-
Less magnification of  image
Elimination of aberrations and prismatic effect of thick glasses
Wider and better field of vision
Cosmetiaclly more acceptable
Better suited for uniocular aphakia.
Though contact lens is having some disadvantages as described below,till it is a good option for the patient described in the question:-
Disadvantages of contact Lens:-
·         Take time to become accustomed to
·         Easily lost
·         Fragile and prone to tearing
·         Fiddly to handle
·         Can be more expensive than glasses
·         Require ongoing maintenance
·         Build up of dust and debris
·         Degrade in quality over time
·         Risk of eye infections(Corneal ulcer)
·         Risk of scratches
Secondary intra-ocular lens implantation:-
Surgical correction of uniocular Aphakia(Implantation of Intra-ocular lens i.e.making the patient pseudophakic):-
It is best option for the patient in the question because of the following reasons:-
1) Rapid improvement of quality & quantity of vision in IOL implantation.
2)      No spherical aberration & prismatic effect in IOL.
3)      IOL is cosmetically well accepted.
4)      Image magnification in IOL is only 0-2%
5)      No alteration of visual field in IOL.
6)      Binocular vision is possible.
Site of IOL Implantation:-
Ideallly it should be implanted in the capsular bag;but the patient in question had undergone surgery due to traumatic cataract and also kept aphakic following first surgery;So it can be concluded that the capsular bag was compromised in this patient.The reason may be due to trauma(e.g:- traumatic subluxation,traumatic posterior capsular tear) or may be surgically induced.
So, if bag is not intact ,but a rim of anteriror capsule is present all around,then Posterior chamber intra-ocular lens can be implanted in the sulcus.
If sufficient capsular support is not there, anterior chamber intra-ocular lens(IOL) implantation can be done;but the anterior chamber IOL is notorious for damaging corneal endothelium.So, in this young patient it is better to avoid anterior chamber IOL. The same is true for anterior chamber iris claw lens.
However few studies have shown better results with implantation of  posterior chamber iris claw lens in young patients.
Another alternative is implantation of Scleral-Fixated posterior chamber intra-ocular lens(Suture or glue fixed SFIOL).Hoever no surgical procedure is free of complications.It is associated with uveitis(immediate), IOL tilt(in future) and astigmatism.
Surgery on the cornea(Refractive corneal surgery):-
a)Keratophakia:- In this procedure,a lenticule prepared from the donor cornea is placed between the lamellae of the patient’s cornea.
b)EpiKeratophakia:-In this procedure,a lenticule prepared from the donor cornea is stitched over the surface of cornea after removing the epithelium.
C)Hyperopic Laser in –situ Keratomileusis(LASIK):-It is a better alternative to treat such type of patients.

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