Keywords

Preoperative discussions with caregivers and patients are an essential component of the early phases of care related to childhood cataracts. These conversations are detailed and complex, requiring the physician to set aside appropriate time and participate as both an active educator and listener. The physician will also need to coordinate the interdisciplinary needs of each unique patient. Appropriate expectations should be set, terminology should be introduced, and empathy should be used, particularly in addressing the concerns and needs of each family. The postoperative course after childhood cataract surgery often lasts for decades, so the family must be prepared for long-term care and follow-up. The family should understand that the physician-family-patient relationship will endure both triumphs and challenges requiring short- and long-term support from the medical team. Successful outcomes often require years of individualized vision rehabilitation and lifelong monitoring for complications.

Etiology

Often, the first question families ask is, “Why did my child get a cataract?” Physicians and parents alike have an interest in the answer to this important question. In some situations, the answer is known at the time of diagnosis (e.g., if a child had a family history of childhood cataracts, a congenital infection, or trisomy 21). However, in many instances, there is either no identifiable cause or testing will be recommended to help arrive at a conclusive answer. In the early phases of the physician-family relationship, it is important to outline for the family the broad categories of cataract etiology including idiopathic, inherited, secondary to other intraocular pathology, and secondary to a systemic condition. At this point, the physician may offer the most likely explanation for the cataract based on the history and exam findings. In cases where additional lab work is warranted, we recommend describing the occurrence of cataracts as an “important clue” that may help the ophthalmologist identify an underlying systemic issue that would benefit from early treatment. Chapter 1 (Cataract Epidemiology and Genetics) provides a comprehensive discussion of the specific causative examples of congenital cataracts. Table 7.1 highlights the steps in preoperative assessment for cataract causality.

Table 7.1 Preoperative assessment for cataract causality
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The etiology of congenital cataract can be narrowed according to key clinical features: laterality, family history, syndromic systemic features, exposure history, and associated ocular pathology. A decision tree illustrating how the clinical features and history aid in determining the cause of congenital cataracts is shown in Fig. 7.1. The family, pediatrician, and other subspecialists, such as geneticists, are key participants in this investigation preoperatively. Often, targeted testing at the time of surgery can be arranged by thoughtful collaboration with a multidisciplinary team.

Fig. 7.1
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Decision tree pediatric cataracts

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Following a careful assessment of cataract causality with the assistance of genetics specialists, a patient-specific multidisciplinary team should be assembled as an essential part of appropriate care for a child with cataracts. Table 7.2 outlines cataract etiology according to the associated systemic manifestation with the greatest morbidity. This organization provides an initial framework for the early stages of developing a medical team that will help care for the child. Ophthalmologists should have a low threshold to refer to colleagues with expertise in managing these complex conditions, thereby ensuring that cataract management does not overshadow the child’s global progress.

Table 7.2 Genetic syndromic associations with cataract, grouped according to primary systemic findingsa [3, 4]
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Timing of Pediatric Cataract Surgery

Preoperative discussion regarding the timing of congenital cataract surgery should focus on weighing the risks and benefits of early versus delayed surgical intervention. The optimal age for pediatric cataract surgery is that which reduces the risk of deprivation amblyopia without significantly increasing the risk of secondary postoperative complications. Families should understand that in general, the younger the child is at the time of cataract surgery, the greater the incidence of associated complications. However, delaying surgery increases the risk and severity of amblyopia.

Retrospective and prospective surgical outcome data have helped define a latent period for pediatric cataract surgery beyond which the risk of amblyopia rises significantly [5,6,7,8,9,10,11]. There is consensus that surgery for unilateral congenital cataract should be performed when the child is 4–6 weeks old [10]. Beyond this latent period, visual outcomes and rates of amblyopia have been shown to steadily worsen over time [6, 7, 9, 10]. Surgery at an earlier age significantly increases the risk of secondary glaucoma, which carries severe ocular morbidity.

The surgical timing for children with bilateral cataracts is less well defined. Families should be counseled that poorer visual outcomes have been demonstrated in children who have surgery after 8–10 weeks of age [5, 10, 11]. Surgeries for the two eyes are ideally separated by approximately 1–2 weeks. A longer period between surgeries increases the child’s risk of developing amblyopia in the eye undergoing later surgery. Immediate sequential bilateral cataract surgery should be reserved for cases where general anesthesia exposure must be limited due to patient comorbidities [12]. In these cases, families must fully understand that this approach has the theoretical increased risk of bilateral endophthalmitis and is therefore not the preferred method for otherwise healthy children. In cases where a child is systemically unstable, surgery may need to be delayed until the child can safely undergo anesthesia.

Risks of Pediatric Cataract Surgery

Informed consent for pediatric cataract surgery should be discussed on multiple occasions prior to the procedure. Some families are referred to the ophthalmologist with a known diagnosis of cataract and the expectation of surgery, while for others, a cataract is a brand new diagnosis. In either scenario, all families will need a great deal of additional information to be adequately informed. Discussion of the potential benefits of cataract surgery is likely to be readily accepted and understood. However, for children who present at a later age, especially those with unilateral cataract, the risk of limited visual recovery and guarded visual prognosis should be explained in detail. Advantages of lens removal for such patients include enhanced contrast and color, improved peripheral vision, prevention of complications related to end-stage cataracts (phacomorphic glaucoma or phacolytic glaucoma), and improved view of the optic nerve and retina for both ocular health monitoring and cycloplegic refraction. For patients who present in a timely fashion, the potential benefits are even greater, but the risks of perioperative and postoperative complications for all children cannot be understated. This part of the discussion with families typically requires more time as it raises additional concerns and questions that need to be addressed. Table 7.3 outlines the many risks that the pediatric ophthalmologist must anticipate at the time of surgery and at every postoperative visit. While this list is too long to discuss each item in detail during the informed consent process, parents and families should be aware of and understand the very common risks of glaucoma, strabismus, refractive error, and amblyopia following pediatric cataract extraction. The rare but devastating risks of infection, retinal detachment, and anesthesia-related surgical risks must also be described in sufficient detail. Table 7.4 highlights the risk of significant complications related to pediatric cataract surgery.

Table 7.3 Operative risks related to pediatric cataract surgery
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Table 7.4 Rates of significant complications associated with pediatric cataract surgery
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Case 1

A 4-month-old boy presents to the emergency department for shaking eyes and failure to thrive. His ocular exam is notable for blink to light visual acuity, dense bilateral lamellar cataracts (Fig. 7.2), and an otherwise normal anterior segment. Fundus examination could be performed through a crescent of clear red reflex around the cataract with a widely dilated pupil and was normal.

Fig. 7.2
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External photo with ruler. Preoperative corneal assessment during examination under anesthesia demonstrates corneal diameters of 11 mm and 10 mm, respectively

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Comment

At this time, discussion with our patient’s parents centered on the causation of his cataracts and the possibility that they represented an “important clue” into his failure to thrive. A genetics and comprehensive inpatient pediatric admission were recommended to assist in his workup. We discussed the timing and risks of surgery, as discussed above. Due to his presenting nystagmus, visual prognosis was felt to be more uncertain. Nonetheless, it was advised to proceed with surgery. Because of his overall good state of health, surgeries were performed 1 week apart.

Two weeks after presentation, the patient successfully underwent bilateral lensectomy and posterior capsulotomy with anterior vitrectomy. He was given aphakic contact lenses. There was resolution of the nystagmus, and he appeared to intermittently fix and follow with each eye. His eye pressure was 11 in each eye with iCare tonometry, fundus examination was normal, and cycloplegic refraction was +20.0 in each eye. At this point, his systemic workup was unrevealing, and he began gaining weight appropriately.

Two years later, the patient was able to reliably read single Allen pictures in aphakic contact lenses, and his vision was 20/30 in each eye. Unfortunately, he had developed elevated eye pressures, enlarged corneal diameters, corneal clouding, and a myopic shift bilaterally but worse in the right eye, leading to the diagnosis of glaucoma following cataract surgery. He required surgical treatment for his right eye and topical antihypertensives in his left. He also underwent two strabismus surgeries for esotropia.

Comment

The patient did well with improved early visual functioning and excellent acuity 2 years after his surgery. This case demonstrates how good visual outcomes can be achieved with prompt intervention, even if referral is delayed and the patient has signs of damage to the visual pathways (nystagmus) prior to lensectomy. Ongoing discussions with his parents continued to focus on the multiple nuances of recovery and care following cataract surgery.

We had decided on aphakic correction with contact lenses (see below for discussion of intraocular lens (IOL) implantation). The needs for long-term care included a discussion of the risks of amblyopia, strabismus, and glaucoma, among others. While patient age at the time of cataract surgery is a key risk factor for development of secondary glaucoma, delayed surgery (even a significant delay of 3 months) does not universally prevent secondary glaucoma. Due to excellent, consistent follow-up, we were able to diagnosis elevated eye pressure early, optimize our patient’s outcome, and minimize risks of vision-threatening complications.

This patient’s care was enhanced by consistent communication and collaboration with his pediatrician and our affiliated genetics team. Genetic testing identified a rare deletion previously reported in other individuals with congenital cataracts. This genetic finding offered a presumed genetic diagnosis but did not confirm the association with and etiology for his cataracts. Continued multidisciplinary involvement, including genetics subspecialists and genetic counselors, is an essential component of his ongoing ophthalmic care.

Primary Pseudophakia Versus Aphakia

The discussion of intraocular lens (IOL) implantation should review the risks and benefits specific to this unique surgical situation. Although IOLs have been found to be safe and effective in children, the Infant Aphakia Treatment Study (IATS) did not demonstrate a visual benefit to primary IOL implantation over primary aphakia with contact lens placement in children with unilateral congenital cataracts. In the IATS, there was an increased rate of adverse events and higher rate of additional surgeries in the IOL compared with the aphakic group [31,32,33]. Disadvantages of primary IOL implantation that should be discussed include increased ocular inflammation, scarring, and the potential need for additional surgery including lens power changes [28, 32, 34]. Table 7.5 details specific risks related to IOL implantation in children.

Table 7.5 Risks specific to intraocular lens (IOL) implantation
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It should be explained that the majority of the growth of the eye occurs during the first 2 years of life; however, the eye continues to grow and elongate throughout the second decade. This makes selecting an IOL power to minimize refractive error in young adulthood a challenge due to our limited accuracy in predicting the growth trajectory of each child’s eye [34, 35]. Various refractive options should be discussed including aiming for a hyperopic correction to avoid a large amount of myopia later in life versus aiming for emmetropia with the use of a piggyback lens or refractive surgery when the axial length has stabilized [36]. Multifocal IOLs may be considered for older children [35, 37].

If the patient is to be placed in aphakic contact lenses, the management of contact lenses should be discussed in detail preoperatively. Active involvement and participation from the patient’s caretaker are critical for success with contact lenses [38]. The caretaker will need to be comfortable with insertion, removal, and cleaning of contact lenses. Families should be counseled preoperatively that adherence to patching and contact lenses is essential for prevention of amblyopia. Disadvantages of contact lenses include the cost associated with frequent replacement due to mucous buildup and change in refractive power [39]. If the physician or family suspects that there are barriers to successful management of aphakic contact lenses that put the patient at risk for poor vision, the physician may in turn select IOL implantation or aphakic spectacles [40].

Long-Term Management

It is important to counsel families that regular ophthalmologic care will extend far beyond the time of the initial cataract surgery and the immediate postoperative period. Preparing families for long-term surveillance allows the ophthalmologist to follow closely for pathology associated with pediatric cataracts and cataract surgery. Amblyopia, secondary glaucoma, and strabismus are three of the most common long-term complications associated with pediatric cataracts. A complete and thorough discussion of these risks should take place. Families should understand that frequent follow-up appointments for patching, refraction, adjustment of spectacles and/or contact lenses, and monitoring of intraocular pressure are an important part of the long-term management and treatment of pediatric cataracts.

Amblyopia

Families should be aware that cataract extraction does not guarantee full visual recovery. Children with cataracts are at high risk for amblyopia [41]. Early cataract surgery reduces the risk of amblyopia; however, postoperative amblyopia therapy is equally as important. Families should be counseled that patching and optical correction, including glasses and aphakic contact lenses, will be needed, with frequent adjustment to account for refractive shifts as the eye grows. The IATS demonstrated that higher socioeconomic status, lower parental stress, and younger age of the child were associated with improved adherence to postoperative patching regimens [42]. These potential factors may help identify parents who require additional guidance in the postoperative period to optimize long-term patient outcomes. It is also important to utilize social support or additional services when needed to help families adhere fully to the prescribed visual rehabilitation regimen.

Glaucoma

Secondary glaucoma is a potential and serious complication that should be discussed with families prior to pediatric cataract surgery. Glaucoma occurs in 15–30% of cases and may be detected as early as 2 weeks or as late as 5 years following cataract surgery [29, 43,44,45,46]. It is often insidious in onset without the typical hallmark findings of primary congenital glaucoma (buphthalmos, epiphora, corneal clouding). Therefore, close intraocular pressure and optic nerve monitoring by the ophthalmologist are required for detection of glaucoma long after the initial surgery and at every clinical visit postoperatively [46]. Glaucoma after cataract surgery is more common in children with microphthalmia and microcornea and in those who undergo cataract surgery at a younger age, although it may occur in children without these risk factors. Additional risk factors for developing glaucoma include persistent fetal vasculature, prolonged postoperative inflammation, and the need for multiple operations [46, 47]. As previously mentioned, families should be counseled that the most readily modifiable risk factor for developing secondary glaucoma following cataract extraction is the age at which the child undergoes cataract surgery. Delaying cataract surgery – especially unilateral cataract surgery – significantly increases the risk of amblyopia. Therefore, a degree of postoperative glaucoma risk is most often accepted by the surgeon in order to reduce the risk of amblyopia development.

Strabismus

The majority of children who undergo cataract surgery will develop strabismus, and it is often present at the time of cataract diagnosis [48]. In some instances, parents may have noticed their child’s ocular misalignment prior to ophthalmologic evaluation. It is important to counsel parents that almost half of children with pediatric cataracts will require strabismus surgery following cataract extraction [49]. Families should be counseled that success with strabismus surgery in this population is lower compared to children without cataracts and the rate of reoperation may be higher. This is due to amblyopia disrupting binocular functioning required to help drive ocular alignment.

Long-Term Visual Prognosis

Parents will want to know what can be done to ensure that their child has the best possible outcome following cataract surgery. A host of factors impact a child’s long-term visual prognosis including, but not limited to, the age of cataract onset and timing of cataract extraction, the density and laterality of the cataract (unilateral versus bilateral), associated ocular and systemic pathology, and the quality of postoperative visual rehabilitation. Children who are of kindergarten age or older when they develop cataracts, and infants who have early surgery for bilateral cataracts, have a superior visual prognosis compared to those with early-onset unilateral cataracts. Major studies such as the IATS can be referenced to explain that almost half the children in the latter group achieved vision better than 20/200 following cataract surgery and the majority acquired some depth perception [6,7,8]. Close surveillance, possible chronic intraocular pressure-lowering therapy, and, often, additional eye surgeries are often required. Therefore, long-term treatment and support must be offered well after the initial cataract surgery is completed in order to optimize and preserve the child’s visual functioning.

Health Literacy and Informed Consent

It is important for physicians to gauge the family’s health literacy in order to guide preoperative discussions and to target patient information materials and written consent forms appropriately. Families with limited education levels or literacy skills pose a challenge in preoperative counseling and obtaining informed consent for pediatric cataract surgery. Written and verbal discussions with families should take into account that 50% of the US population cannot read above an eighth-grade level. Even patients with the highest levels of education may have limited health literacy, hindering their ability to understand health information and make appropriate health-related decisions. The National Institutes of Health and American Medical Association recommend that patient educational resources be written at or below an eighth-grade reading level so that the information is accessible to most patients. While the risks and nuances of long-term management of pediatric cataracts may be complex, the surgeon should make every effort to use clear, concise , and simple language in counseling patients and their families [50, 51].

Appendix 1 contains a sample consent form that can be used during the preoperative discussion with families in planning for pediatric cataract surgery. This form is modified from the adult cataract consent form available on the Ophthalmic Mutual Insurance Company web page and is validated through readability indexes to be written at or below an eighth-grade level.

Case 2

An 11-month-old baby boy with trisomy 21 was referred by his pediatrician for dull red reflexes. He presented to the ophthalmology clinic with his parents, who live in a rural area 2 hours away. His mother and father are 17 years old, did not attend high school, and have a history of substance abuse. The patient was found to have visually significant cataracts in both eyes (Fig. 7.3). After thorough discussion with the family regarding the risks and benefits of surgery, the decision was made to pursue bilateral sequential lensectomy, without implantation of an intraocular lens.

Fig. 7.3
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Microscope view bilaterally. Preoperative lens assessment during examination under anesthesia demonstrates bilateral dense lamellar cataracts

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Immediate postoperative appointments were uneventful, and the postsurgical course was without complications. Two weeks later, the patient failed to return for follow-up appointments. A pediatric clinical social worker was then recruited to assist in contacting social services, and transportation was provided for appointments. The patient’s grandmother was noted to be an involved caretaker, and she was encouraged to attend all clinic appointments along with the parents. The family was educated on the importance of timely follow-up and the risks of vision loss. Given the complex social dynamic and inconsistent follow-up history, aphakic spectacles were prescribed instead of contact lenses.

After 3 years of follow-up, the patient was 4 years old and demonstrated very good vision. He continued to return for follow-up appointments with the assistance of social services. He is doing well with aphakic spectacles and may be a good candidate for secondary IOL implantation in the future.

Comment

The surgeon should identify any potential barriers to postoperative management and timely follow-up prior to cataract surgery. This may include difficulty obtaining medications, contact lenses, glasses, or attending postoperative appointments. Early involvement of social support services should not be overlooked in its ability to assist in these vital components of post-cataract surgery rehabilitation. For situations where primary caregivers are unable to adequately absorb all duties of the postoperative care, other family members can be recruited. Understanding family dynamics and identifying risk factors can aid in the decision to use aphakic spectacles versus contact lenses or to implant a secondary intraocular lens.