Management of Cataracts in Pediatric Patients with Developmental Delay at a Tertiary Care Pediatric Hospital

Background: Childhood cataract causing visual impairment can compound developmental delay (DD) if left untreated. Current literature in children with DD is limited; thus, we evaluated cataract etiology, challenges, and treatment compliance in this group. Purpose: To report the presentation and challenges associated with cataract management in children with developmental delay (DD) at a tertiary care pediatric hospital. Methods: Retrospective review of 100 patients (173 eyes) presenting with cataracts and DD from February 2014 to December 2017. Results: 100 patients (173 eyes) were included. 27 patients had unilateral cataracts and 73 bilateral. The average age was 120.55 months (SD 63.77, range 5.87 - 243.16); the average follow-up pe-riod was 57.7 months (SD 139.14, range 1.03 - 1412.30). 61% of patients (55% eyes) underwent medical management for cataracts due to: cataract was not visually significant (66% eyes), parent deferred surgery (11% eyes), self-abusive behavior (14% eyes), and medical conditions that limited visual recovery (9% eyes). 32% of patients were unable to perform objective visual acuity by age 5. Patients with self-abusive behavior were more likely to present with or develop retinal detachment (RD) (35%) compared to those without self-abusive


Introduction
There are approximately 1.4 million blind children worldwide, with an additional 500,000 children becoming blind annually. Of these cases, childhood cataracts contribute to 14% of these cases and are most associated with congenital rubella infection and inherited developmental disorders [1]. In general, children with developmental delay (DD) have a higher frequency of ocular conditions, including cataracts, and should be screened appropriately [2] [3] [4]. If left untreated, cataracts can negatively affect psychosocial, educational, and visual development in patients who carry the diagnosis of DD [5]. Unlike cataract management in adult patients, cataract management in children incurs additional challenges, such as a need for general anesthesia, limitations in examination and treatment due to cooperation, presence of amblyopia, and changes in refractive error over time [6] [7].
Pediatric patients with DD and cataracts present providers with unique challenges. Contributing factors that challenge cataract management in children with DD include the need to interpret nonverbal cues, the presence of comorbid medical conditions limiting visual diagnosis and recovery, and the potential for self-injurious behavior. Current literature correlating etiology, type of cataract, complications, and compliance with treatment in children with DD are limited.
In the United States, 1 of every 6 children aged 3 -17 has DD, and this number has been increasing over the last decade [8]. The purpose of this study is to report the etiology and characteristics of cataracts in patients with DD, explore contributing factors that challenge cataract management in children with DD, and provide direction for providers managing cataracts in these children, based on a population study at a tertiary-care center. Best corrected visual acuity was recorded from the first and most recent visit.

Methods
Those with no light perception or who could not perform objective visual acuity measurement by age 5 were noted. In those patients who were able to cooperate for visual acuity examination, the Snellen or HOTV method was used.
Compliance with drops, glasses, contacts, or patching was recorded. Noncompliance with eye drops was noted in any patient who did not receive all doses of drops in the recommended course. Glasses compliance was recorded via parental history. If the patient wore glasses less than 50% of the recommended time per day for two separate visits, noncompliance with glasses wear was recorded. Failure to patch was also assessed via the patient's legal guardians, and was deemed unsuccessful in those who did not patch greater than 50% of the recommended time for two separate clinic visits.

Etiology and Morphology
Morphology and etiology of cataract are shown in Figure 1 and Figure 2, respectively. 36% (62/173) of the cataracts were congenital. Morphology of cataract is further characterized in Figure 3

Examination, Complications, and Treatment
Difficulty with the examination, complications after surgery, and treatment adherence issues are summarized for providers in Table 3. Objective visual acuity

Result Recommendation
Objective visual acuity was not achievable due to poor cooperation in 32% of patients over the age of 5. Poorer cooperation is more likely in nonverbal patients than verbal patients.
Providers should be aware of this challenge. The red reflex exam and retinoscopy to determine the visual significance of cataracts is a useful tool in these instances.
39% of eyes in patients with DD developed a complication, with aphakic glaucoma and visual axis opacification being the most common occurrences.
Counsel families regarding the risk of these postoperative occurrences and need for intervention. Exam under anesthesia may be required in some circumstances.
Visual axis opacification is more likely in patients with IOLs than those who are aphakic.
Visual axis opacification is a known occurrence after pediatric cataract surgery. Counsel families regarding the risk of these postoperative occurrences.
Patching is universally difficult in both verbal and nonverbal children with DD.
Counsel families to anticipate this challenge and be given options on alternative patch modalities.
Verbal patients are more compliant with glasses treatment than nonverbal patients.
Counsel families of nonverbal patients to anticipate this challenge. In some situations, an alternative method of refractive correction may be considered.
Comorbid diagnoses of RD and cataract are more likely when the patient has self-injurious behaviors.
Maintain a higher suspicion for RD in patients with DD and self-injurious behavior presenting with complete cataracts.
was not achievable due to poor cooperation in 32% of patients over the age of 5 (25/78 patients), despite the average age of these poorly compliant patients being 145 months (12 years). Poor cooperation for the exam was noted to occur more frequently in nonverbal patients with 83% (43/52) of nonverbal patients classified as uncooperative compared to 23% (11/48) of verbal patients found to be uncooperative (P < 0.0001). The incidence of RD in patients with DD at the time of cataract evaluation was found to be higher in those who displayed self-injurious behavior compared to those without self-injurious behaviors (P = 0.0028). Only 6% (5/83) of patients without self-abusive behavior presented with comorbid RD at the time of cataract evaluation, while 35% (6/17) of patients with self-abusive tendencies presented with both RD and cataracts. Of the patients with self-abusive behavior, 10/34 eyes had no light perception at the time of presentation, and 2/34 eyes could only blink to light.
Complications after surgery occurred in 39% of eyes that were managed surgically (30/78). 18% (14/78 eyes) of eyes developed aphakic glaucoma, 17% (13/78 eyes) developed VAO, 1% (1/78 eyes) developed RD after surgery due to self-injurious behavior, and 3% (2/78 eyes) required removal of the IOL due to anterior chamber inflammation secondary to noncompliance with post-operative drops. Those with IOL placement after cataract extraction were more likely to develop VAO; 27% (9/33) of eyes with IOL developed VAO compared to 9% (4/45) of aphakic eyes (P = 0.0313). Open Journal of Ophthalmology Amblyopia was diagnosed in 31% of patients (31/100 patients), and 71% of these (22/31 patients) were recommended to undergo patching treatment. 59% (13/22) of patients were not compliant with patching. No statistical difference was found in compliance with patching between verbal patients (8% eyes) compared to nonverbal patients (10% eyes). Other recommended treatments included glasses or contacts in 56% (96/173) of eyes. Compliance with glasses treatment in verbal patients with DD (97% with good compliance) was better than nonverbal patients with DD (37% with good compliance) (P < 0.0001).

Discussion
There has been a significant increase in the prevalence of DD over the last decade, and as a result, a necessity to improve targeted interventions [8]. A multispecialty approach is necessary to care for and determine resources needed for children with DD, with ophthalmology being particularly important due to the high prevalence of ophthalmic conditions in this group [10]. To our knowledge, this is the first study to delineate the etiology and morphology of cataracts in patients with DD as well as explore the challenges in managing these patients.
It has been demonstrated that children with DD have ophthalmologic abnormalities at a higher prevalence than the general population [10]. Many children with DD have been found to have significant ophthalmic pathology even when there is no suspicion of eye-related problems [2]. Formal recommendations for ophthalmic screening in children with DD are not well-established, but many providers recommend performing an eye examination between 1 to 5 years [2] [4] [11]. As expected, patients with DD undergoing surgery for RD, receiving radiation to the head, or using steroids are at increased risk for PSCs and should be monitored accordingly. This is consistent with existing data in non-DD populations, suggesting that PSC development in patients with these risk factors is independent of DD status [12] [13].
Cataract management and extraction in nonverbal pediatric patients with DD is generally successful, however, providers should prepare for limitations when managing these patients. The red reflex exam and cycloplegic retinoscopy, if tolerated, are most helpful in evaluating the visual significance of cataracts in nonverbal patients, as many cannot participate in a slit lamp examination in the clinic. In situations where clinic exams cannot be completed, exams under anesthesia may need to be utilized [14]. Furthermore, there is a higher incidence of difficulty wearing glasses, patching, and cooperating during clinical examination in nonverbal patients compared to verbal patients with DD. Providers should be aware that targeted counseling and additional efforts may be required to ensure adherence to examination and treatment.
Ocular self-injury, while uncommon, is an important consideration when evaluating patients with DD, as trauma is a common cause of cataracts [15]. Reports of self-inflicted blunt head trauma as a mechanism for cataract development also correlate with a higher incidence of vitreous hemorrhage, RD, and Open Journal of Ophthalmology self-enucleation at presentation in patients with DD [15] [16] [17]. Even without visible injury to the globe or eyelid, dense cataracts have been reported in patients with chronic headbanging behavior [17]. In our patient population, children with self-injurious behavior were more likely to present with RD and with a white cataract. This calls for provider awareness and targeted screening for self-injurious behavior and its associated morbidities when eliciting patient histories.
Surgical outcomes are generally favorable in patients with DD, but as expected, VAO is a frequent and expected complication particularly when IOLs are placed. VAO is more common in the pediatric population, occurring in up to 40% of patients undergoing cataract surgery [18]. Providers should educate patients and their families that YAG laser or surgical management may be necessary due to the development of VAO when an IOL is placed. On the other hand, our data suggest that aphakic glaucoma may be a more prevalent surgical complication in children with DD (18% of eyes) compared to recent rates published in the general pediatric population (6% -13%) [19] [20]. Studies from the early 1990s report rates of aphakic glaucoma as high as 41% in pediatric patients, but advances in surgical techniques may play a role in the lower rates today [21].
Higher rates of complications in children with DD are consistent with existing literature, which states surgical complications in children with Trisomy 21 occur in 20% -60% of patients compared to 0% -28% of the general pediatric population. However, existing studies were limited by small sample size [3]. This difference in surgical outcomes between children with DD and the general pediatric population could be secondary to more medically complex cases, susceptible anatomy, or difficult post-surgical course. Higher rates of cataract surgery complications in children with DD correlate with the increased morbidity for this group from surgical interventions outside of ophthalmology as well [3].
Our study is limited by the fact that it is a single-center study and used a retrospective design; however, our diverse cohort in age, race, and ethnicity make our results more representative of pediatric patients with DD and cataracts. Future research on patterns of cataract etiology and management in specific forms of DD could be useful.
While the severity of DD cannot always be measured quantitatively, factors such as language ability and self-injurious behavior may be useful predictors of ease of visual examination, cataract surgery outcomes, and treatment compliance.
Difficulty assessing objective visual acuity, poor patching compliance, self-abusive behavior, and poor compliance in glasses wear are potential challenges that may limit the ability to manage patients with DD. Educating providers regarding these challenges can help establish interventions for patients with DD and prepare both patients and providers for the difficulties in management.