Fig. 26.1
Pediatric aphakia is corrected using spectacles
- 1.
Thick and heavy spectacle lenses lead to reduced compliance.
The nose bridge and ears have not yet fully developed in infants and very young children, which makes it difficult to achieve a stable fit for the spectacles. Thick and heavy aphakic lenses can make wearing uncomfortable, and most parents are unaware that decreased compliance affects spectacle correction results (Fig. 26.2). With regard to frame material, lightweight titanium, flexible metal or special memory plastic materials may be chosen to ensure proper fitting. EP plastic, a light synthetic material resistant to deformation, and nylon, which does not usually break and can be manipulated back to shape after dropping or being trampled. To reduce the lens thickness and weight, spectacle lenses with high refractive index may be chosen. But it should be noted that colour aberration can reduce the corrective effect due to increased Abbe number in the case of higher refractive index. In the past 10 years, press-on lenses have been preferred for their light weight and reduced thickness. However, press-on lenses with high powers can cause altered imaging to a certain extent. Moreover, there is space between the pressure film and the lens, which may harbor dust and cause unstable adherence and even peeling.
Fig. 26.2
Improper frame fitting in an aphakic child when he is able to see over the spectacles
- 2.
Magnification
The magnification changes by about 1 %, for every 0.50D alteration in the power of spectacles. When differences between bilateral magnifications reach approximately 5 %—namely, a difference of 2.50D in the refractive power—the human visual system often fails to tolerate unequal visual inputs, thereby causing interocular suppression or confusion. In the case of aphakic eyes following monocular congenital cataract surgery, pediatric patients may develop severe binocular diplopia because of the great difference in magnification when wearing spectacles. Difficulty in fusion, exacerbates suppression, and refusal to wear spectacles due to discomfort will ensue and impair rehabilitation of monocular and binocular vision. In children with binocular congenital cataracts, postoperative binocular correction allows for similar or identical binocular magnifications, which can be better tolerated among infants and young children.
- 3.
Significant spherical aberration
The spectacle lenses for aphakic children are usually convex with high diopters, through which light rays are refracted. In an emmetropic eye, imaging on the retina is presented as a curved image instead of a planar retinal image, which makes objects apparently uneven with saccadic and tracking eye movements. Additionally, most children with congenital cataracts have concurrent congenital nystagmus, in which the center of the visual axis has deviated from the optical center that can even result in a prism effect. These abnormalities cause diminished visual acuity and sharpness.
- 4.
Obscured peripheral vision
Since the area of spectacle lenses is limited, not all peripheral light rays can enter the eyes after being refracted through the lenses, and some of them even bypass the lenses. Consequently, this part of the image cannot be clearly focused onto the retina and thus becomes a blurry field of vision. In addition, decentration of high power lenses often produces a prismatic effect, leaving a circular peripheral scotoma and narrowing of the visual field.
- 5.
Peripheral optical defocus
Optical lenses only correct refractive error in the central field, yet refraction varies between the peripheral and central fields. Therefore, when spectacle correction is worn by children with congenital cataract, their peripheral field is often significantly uncorrected, which disrupts emmetropization and impairs visual development. Currently, new designs in soft CLs that can correct peripheral defocus are undergoing large-scale clinical trials (in myopia control). It is expected that the novel design of CLs will be used for refractive correction following congenital cataract surgery in the future.
26.2.2 Contact Lenses (Near and Distant)
Compared with spectacles, CLs have considerable advantages in the refractive correction of aphakic eyes. Advances in technological design and materials science have eliminated the following CLs drawbacks: thick and heavy lenses, uncomfortable foreign body sensation, poor oxygen permeability, and severe ocular surface disruption.
CL correction has the following advantages and disadvantages over spectacles:
Advantages of CLs: (1) There is no magnification or minification of retinal image even in the case of high refractive errors, as CLs are adjacent to the front principal point of the eye. For postoperative anisometropia, the relative consistency in the size of binocular images is unlikely to cause impaired fusion and interocular suppression. (2) CLs do not obscure the peripheral visual field, and eliminate image distortion, aberration, and chromatic aberration. (3) Wearing CLs is comfortable and does not affect daily activities. Unlike spectacles, fogging will not form on CLs when entering a sudden change in hot or cold environment, and CLs do not cause pain or allergic reactions to the ears, nose, or skin on the temporal sides of the face. CLs are less susceptible to deformation, dropping of lenses, and even lens breakage after blowout trauma, which may lead to severe eye injuries. These features are especially beneficial to hyperactive children. (4) Children wearing CLs do not have cosmesis concern compared to thick and heavy eyeglasses, which attracts mocking or isolation in group activities. Therefore, wearing CLs is more favorable to the development of children’s physical and mental health.
Disadvantages of CLs are as follows: (1) Since infants and young children (1–3 years) are not cooperative to examination, accurate measurement of corneal curvature cannot be obtained, thus making it more difficult to produce suitable CLs. What’s more, decentration might occur and result in undesirable correction. (2) CLs are frequently lost, which causes reduced duration of refractive correction. (3) Frequent removal and wearing of CLs elicits psychological resistance in children. To address this challenge, investigators at Zhongshan Ophthalmic Center (ZOC) of Sun Yat-sen University have developed a device for wearing and removing CLs for children. A specially designed connecting tube links a vacuum bag to a suction disk; the CL is drawn to the sucking disk and firmly adheres to it through vacuum pressure. In this way, CLs may be worn and removed safely, rapidly, and conveniently, which is especially desirable in the case of child wearers (Fig. 26.3). (4) CLs of high diopters are expensive and the manufacturing process is complex. Therefore, the abovementioned factors lead to pediatric patients’ poor compliance.
Fig. 26.3
A device for wearing and removing CLs. 1 suction disk; 2 connecting tubes; 3 vacuum bags; 11 venting holes
It works as follows: The round suction disk is attached to the exterior of the CL (dotted lines) after squeezing the vacuum bag, which is then released, and the resulting vacuum firmly grips the CL and takes it out of the lens case. Subsequently, the posterior surface of the CL is aimed at the corneal surface, and the vacuum bag is squeezed again to release vacuum so that the CL is separated from the suction disk and lodges on the corneal surface snuggly.
CLs are superior to spectacles as a means of aphakic refractive correction. Nevertheless, they may cause complications including ocular surface infections and allergic hypersensitivity. The wearing and removing of CLs are demanding and require patients’ cooperation. Therefore, extensive counseling for the child and the parents can never be overemphasized.
26.2.2.1 Timing and Considerations
Generally speaking, evaluations for CLs are conducted 3 months after surgery if there are no ocular contraindications. But it should be noted that refraction should be avoided when the pediatric patients appear unwell. Guidance should be patiently provided to parents on manipulation and caring of CLs, so as to help the child accept the wearing of the CLs as soon as possible.
26.2.2.2 Principles of Prescription
Choosing appropriate powers of CL is particularly important for aphakic correction in children, and CLs of improper power can cause amblyopia. Infants and young children generally require better near vision, while distance vision becomes more important as they grow. Cycloplegic refraction for each aphakic child should be performed plus over- or under-correction. The dosage of over- or under-correction is controversial. The authors prescribe under-correction of 1.50–3.50D for infants (<1 year) and under-correction of 0.50–1.50D for toddlers (1–4 years).
26.2.2.3 Indications
- 1.
Monocular aphakia: Binocular anisometropia in children with monocular aphakia tends to exceed 10D. Since children are in the critical period of visual development, anisometropia >3D is likely to cause amblyopia. Wearing CLs reduces optical defocus and binocular disparity and is safe and effective under most circumstances.
- 2.
Binocular aphakia: Children with binocular aphakia following cataract surgery are usually highly hyperopic. Visual rehabilitation by wearing CLs may be chosen.
- 3.
Irregular astigmatism: Traumatic cataracts in children are often complicated by corneoscleral injury. Surgical repairs of the injury cause irregular astigmatism, which is difficult to correct using spectacles. Rigid gas permeable (RGP) lenses can correct irregular astigmatism by covering the irregular corneal surface employing the tear film.
- 4.
Nystagmus: Children with congenital cataracts often have nystagmus. Refractive correction using CLs in eyes with nystagmus may diminish image distortion and unstable imaging caused by spectacles and facilitate the improvement of visual function.
26.2.2.4 Considerations
The following issues need to be considered prior to the decision whether or not to wear CLs:
- 1.
Cornea: Although irregular astigmatism is an indication for wearing CLs, sometimes it is challenging due to excessive corneal irregular astigmatism and corneal scarring.
- 2.
Delayed presentation due to inability to express themselves: Very young children are unable to clearly express their abnormal sensations. For instance, when complications such as corneal epithelial injury or inflammation occur as a result of wearing CLs, there might be delayed diagnosis and treatment which in turn leads to more serious consequences, as the child cannot fully explain what is wrong. Therefore, parents should be educated about how to identify similar abnormal findings and seek medical help in good time.
- 3.
Affordability and compliance: Rapid change in pediatric refractive state requires regular visits to the hospital and frequent replacement of CLs, which may place a heavy financial burden on the child’s family.
- 4.
Allergic Hypersensitivity: The material or disinfectant used with CLs may cause allergic reaction in certain individuals, which can be effectively controlled by the use of anti-allergic drugs. Timely replacement, proper cleaning and disinfection, and appropriate lens care may effectively prevent hypersensitivity reactions.
26.2.2.5 The Different/Various Types of CLs
Commonly used CLs include RGP and soft CLs. With good heat conduction, a desirable moisturizing performance, and high oxygen permeability, RGP lenses are especially suitable for children with higher degrees of astigmatism and irregular corneal surface. Their disadvantages lie in weak elasticity, easy warping, lack of comfort, and a longer period of adaptation.
Soft CLs are flexible, with a desirable elasticity, and they cover the entire cornea; in addition, they have the advantages of being comfortable to wear and require only a short period of adaptation. These are compared with RGP lenses, whose disadvantages are that they are liable to surface deposition and have a short duration of service. Currently the most widely used material in soft CLs is hydroxyethyl methacrylate (HEMA). Plastic polymeric materials (also termed hydrogel) that are soft and hydrophilic are added to this type of lens. As a result, they contain 30–80 % water, which guarantees comfortable wearing. In the last 10 years, the materials used in the manufacture of soft CLs are no longer confined to conventional hydrogel. Instead, various monomers, mostly siloxane-polydimethylsiloxane (PDMS, also called silicone hydrogel), are added to increase oxygen permeability.
For young children, CLs with high oxygen permeability and design for overnight wear should be chosen to reduce frequency of application and removal and thus reduce the opportunity for ocular surface injury. This type of lens allows for continuous use up to 30 days, which is convenient for infants and young children, as well as for parents in handling and care. They also cause less foreign body sensation due to their special hydrophilic treatment. The frequency of replacement of these lenses is typically once or twice a year. Additionally, RGP lenses with added UV filter may reduce ocular damage induced by UV irradiation. It is suggested that a backup set of lenses should be available since RGP lenses are frequently lost when crying and rubbing the eyes. In the early period of wear by older children, RGP lenses should be examined once a month, while those of infants under 1 year should be checked once a week, so as to identify any reaction of ocular tissues and to observe the appearance and condition of the CLs.
Modern design CLs with high oxygen permeability have improved safety for long-term wearing. However, CLs are more demanding on a child’s ocular surface and cooperation, as well as their parents’ understanding and education levels. Therefore, in offering the choice of whether or not to wear CLs, we should consider not only their indications but also practical situations of both the children and their parents.
26.2.3 Intraocular Lenses
IOLs have a lower magnification and have less effect on the peripheral visual field than spectacles. Compared with CLs, IOL implantation eliminates the cumbersome process of the wearing, removal, and care of CLs as well as reduces the risk of infection. It has become the standard practice in correction of pediatric aphakia in most scenarios. For the selection and implantation techniques of IOL, refer to Chaps. 14 and 15, respectively.
26.2.4 Corneal Refractive Surgery
Due to the limitation of corneal thickness, the maximum correction offered by corneal refractive surgery in hypermetropic refractive error (HRE) is +6D. However, the refractive state of the majority of aphakic children falls into the category of hyperopia > +12D, thus making it inadvisable to perform this surgical procedure in pediatric aphakia.
26.3 Treatment of Amblyopia
One of the main reasons for visual impairment caused by pediatric lens disorders is deprivation amblyopia. Studies have shown that disrupted development of visual function from preoperative deprivation persists even in children receiving proper IOL implantation. Therefore, amblyopia treatment is of importance in the treatment of congenital cataract. This section will elaborate on the concept, pathogenesis, and treatment of amblyopia.
26.3.1 Concept of Amblyopia
With a high prevalence worldwide (2–5 %), amblyopia is the first disorder causing monocular visual impairment in children [1, 2]. Amblyopia severely impairs both monocular and binocular visual functions. A study on monocular amblyopia has found that trauma, inflammation, and visual field damage are more likely to occur in the fellow eye [3]. The diagnostic criteria for amblyopia vary in different countries. The classical concept of amblyopia is, a developmental disorder of visual function caused by abnormal visual perception including form deprivation or binocular interaction during visual development [4]. American Academy of Ophthalmology (AAO) has proposed an accurate definition of amblyopia encompassing visual acuity criteria and diagnostic criteria (Table 26.1).
Table 26.1
Diagnostic criteria for amblyopia
Criteria | Clinical manifestations |
---|---|
Monocular amblyopia | |
Fixation properties | Unbalanced fixation |
Preferential looking | Difference of 2 octavesa |
Best corrected visual acuity (BCVA) | Interocular differences in BCVA ≥2 lines |
Binocular amblyopia | |
BCVA | BCVA in either eye is below 20/50 (age ≤3 years) |
BCVA in either eye is below 20/40 (age ≥4 years) |
26.3.2 Pathogenesis of Amblyopia
Amblyopia is typically caused by strabismus, anisometropia, refractive errors, and form deprivation (congenital cataract and ptosis). These anomalies may lead to disrupted development of visual function if they occur during visual development [6].
Visual development may be divided into the critical period, the sensitive period, and the plastic period [7]. In the critical period, the development of visual perceptual functions reaches the same level as that in adults. In the sensitive period, visual disturbance can give rise to functional disorders of vision. While in the plastic period, visual function can be restored after developmental disorders have occurred. Actually, there are no clearly defined time limits for the three periods, and sensitivity to visual stimuli gradually decreases from the critical period toward the plastic period. There are great differences in visual functions among different species, populations, and individuals. Currently there is huge disagreement over the respective duration of these periods. It is generally acknowledged that the critical period occurs at about 5 years to 6 years, the sensitive period at 8 years, and the plastic period at 11–12 years. Previous studies have shown that normal visual perception could no longer be established once beyond the plastic period. However, studies from the authors and other investigators all reveal that there is still certain restoration capacity for impaired visual functions, even in children beyond the plastic period. Therefore, further research is required to confirm whether there is a certain time limit for the plastic period or it has a lifelong duration. But it can be deduced from the three periods of visual development that the sooner amblyopia treatment starts, the better the visual outcomes are. Additionally, treatment should start in a period of greater plasticity, so that visual function can be recovered to a greater degree.