Cataract surgery outcomes in pediatric patients with systemic comorbidities

Abstract

Purpose:

The aim of the study was to evaluate the outcomes of cataract surgery in patients of the pediatric age group with systemic comorbidities.

Methods:

Medical records of 54 eyes (30 patients) of the pediatric age group with systemic comorbidities who had undergone cataract surgery in a tertiary-care center were reviewed. The following parameters were recorded: systemic comorbidity; toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV (TORCH) profile, best spectacle-corrected visual acuity (BSCVA), strabismus, nystagmus, and cataract morphology.

Results:

Thirty patients with a mean age of 55 months (9 months–14 years) were included. On average, every child was seen by three physicians, and the mean duration between the first visit to a physician and presentation to our center was 2.23 ± 0.67 years. The various causes for delay in referral include multiple referrals due to a lack of general anesthesia services in 78% of cases, a long waiting list at the referral hospital in 35% of cases, and a lack of awareness at the primary-care physician level in 50% of cases. The mean BSCVA at presentation was 1.4 logMAR (0.3 to 3 logMAR). The most common cataract morphology was that of zonular cataract (31.48%; 17/54). Strabismus and abnormal eye movements were observed in 27.7% (15/54) and 33.3% (18/54) of eyes, respectively. Various systemic associations were periventricular leukomalacia (12/30), Down’s syndrome (6/30), seizure disorder (6/30), cardiac valvular anomalies (6/30), Marfan’s syndrome (4/30), hypothyroidism (4/30), rubella (3/20), cytomegalovirus (3/20), cerebral palsy (2/30), nephrotic syndrome (2/30), Type 1 diabetes mellitus (1/30), microcephaly (1/30), cryptogenic West syndrome (1/30), congenital rubella syndrome (1/30), and Tourette syndrome (1/30). The mean postoperative corrected distance visual acuity (CDVA) at 2-year follow-up improved to 1.0 logMAR (0 to 3 logMAR). No postoperative complications were reported at the final follow-up. Around 70% of the parents reported improvement in their child’s psychomotor skills.

Conclusion:

Intellectually impaired pediatric patients with cataract should be operated upon whenever there is a presence of infrastructure, and unnecessary delay in surgery should be avoided by referring the patient to higher centers. Even though objective improvement in visual acuity was suboptimal, there was definitely an improvement in the psychomotor skills of the patients.

Cataract surgery in the pediatric age group is challenging. The challenges include preoperative evaluation, intraoperative surgical difficulties, and postoperative visual rehabilitation. These challenges, especially, the preoperative assessment and postoperative care, are further doubled while dealing with patients with systemic comorbidities. Systemic abnormalities have been reported to be associated with almost 22% of congenital cataract cases.[1] All pediatric cataract surgeries require general anesthesia (GA), and in the presence of systemic comorbidities, the administration of GA requires a battery of investigations that may delay the surgery. Besides, visual rehabilitation following surgery requires reasonable cognitive skills from the child, which again gets compromised in the presence of systemic comorbidities, especially, neurological. Children with multiple disabilities have the need for varying and complex management involving multiple professional medical teams to effect adequate and appropriate care of not only the ocular condition but also the systemic conditions.

Few of the important barriers in developing countries to early cataract surgery in the pediatric population with systemic comorbidities include lack of awareness among parents, asymptomatic children who regard their poor vision as “normal,” lack of pediatric or anesthetic services in the region, fear or lack of expertise of administering anesthesia, lack of awareness among general practitioners, which means that parents may be wrongly advised to “wait” until the child is older and fear of surgery among parents due to concern about the risk of anesthesia.

Although there is ample literature on the outcomes of cataract surgery in pediatric cases, there are very few studies focusing on cases with systemic comorbidities. In general, the outcomes in such cases are considered poor. In this paper, we report the outcomes of different types of cataract surgery performed on 30 patients of the pediatric age group with systemic comorbidities. To the best of our knowledge, this is the largest case series reported so far.

Methods

This retrospective review included 54 eyes of 30 patients of the pediatric age group (age ≤18 years) with systemic comorbidities (systemic syndromes/metabolic/hereditary/intrauterine infections) who had undergone primary cataract surgery over a period of 5 years with a minimum of 2 years of follow-up at a tertiary eye-care center in north India. The study adhered to the tenets of the Declaration of Helsinki. Approval for the study was obtained from the Institutional Review Board/Ethics Committee. The medical and surgical records were reviewed and the following parameters were recorded: systemic comorbidity, prenatal/antenatal history, type of delivery, birth weight, hemoglobin levels on admission, toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV (TORCH) profile, primary complaint, best spectacle corrected visual acuity (BSCVA), intraocular pressure (IOP), presence of strabismus and abnormal eye movements, cataract morphology and laterality, ultrasound for the posterior segment, visual evoked potential, and ocular biometry. Surgical notes were meticulously reviewed for the type of lens extraction with or without anterior vitrectomy, type of intraocular lens (IOL) implantation, intraoperative difficulties, and complications. Postoperative findings such as corrected distance visual acuity (CDVA), refractive error, findings of examination under anesthesia (EUA), and complications at the last follow-up, such as glaucoma, posterior capsule opacification (PCO), increased IOP, and IOL decentration, were recorded.

All cases had undergone cataract surgery using a standardized technique under GA as published in one of the papers from our center.[2] Intra-lenticular lens aspiration (ILLA) with anterior vitrectomy was performed for ectopia lentis cases only as described in one of our previous papers.[3] All cases were followed up at our lens clinic. At each postoperative visit, all subjects had refraction and examination of the anterior and posterior segments of the eye. Appropriate refractive corrections such as bifocal or aphakic glasses were prescribed for all subjects at the earliest when refraction was feasible. Amblyopia-appropriate patching therapy was advised concurrently based on the laterality and density of the cataract.

Statistical analysis

Statistical software IBM SPSS 24 was used to analyze the data collected. The independent sample t-test was used to compare means, and categorical data were analyzed using Pearson Chi-squared test. Multiple logistic regression was used to calculate the odd ratios and confidence intervals. A P value of less than 0.05 was taken as statistically significant.

Results

The study analyzed 54 eyes of 30 patients, out of which 24 patients (80%) presented with bilateral cataracts and six patients (20%) had unilateral cataracts. Parents noticed white reflex or leukocoria in 14 patients, whereas diminution of vision was the primary complaint in 12 patients. Inability to grasp objects, not following light, watering, and referral from a pediatrician were among the other few causes, present in one patient each. All patients were specifically screened at presentation for systemic associations by a pediatrician.

Preoperative findings

Table 1 shows the baseline characteristics of the individual eyes of 30 patients. The study included patients of a large age range of 9 months to 14 years, the mean age at surgery being 55 ± 51 months. Males represented two-thirds (20/30) of the subjects. On average, every child was seen by three physicians and the mean duration between the first visit to a physician and presentation to our center was 2.23 ± 0.67 years. The various causes for delay in referral include multiple referrals due to lack of GA services in 78% of cases, a long waiting list at the referral hospital in 35% of cases, and lack of awareness at the primary-care physician level in 50% of cases.

Table 1.

Baseline characteristics of eyes (n=54)

Patient no.	Age at surgery (years)/Sex	Eye	Systemic comorbidity	Prenatal and antenatal history		Type of delivery	Birth weight (gm) and NICU stay		Hemoglobin levels (g/dl)		TORCH profile	Primary complaint and age of presentation	Visual acuity (logMAR)
1	1.8/F	OD	PVL	Full term		NVD	2500, no		8.9		Negative	Leukocoria at 1 year	UC
1	1.8/F	OS	PVL	Full term		NVD	2500, no		8.9		Negative	Leukocoria at 1 year	UC
2	0.7/F	OD	PVL, epilepsy, Rubella IgG + , cerebral palsy	Preterm, premature rupture of membranes, delayed cry		NVD	1000, yes		11.5		Negative	Leukocoria at 1 month	FL
2	0.7/F	OS	PVL, epilepsy, Rubella IgG + , Hypothyroid, cerebral palsy	Preterm, premature rupture of membranes, delayed cry		NVD	1000, yes		11.5		Negative	Leukocoria at 1 month	FL
3	10/F	OD	Marfan’s syndrome, mitral valve prolapse, mitral and tricuspid regurgitation, aortic root dilatation	Full term		LSCS	NA		13		Negative	Diminution of vision at 5 years	0.47
4	12/M	OS	Down’s syndrome, hypothyroidism	Full term		NVD	NA		12		Negative	Leukocoria at 11 years	NFL
5	1.6/M	OD	Down’s syndrome, operated VSD and PDA ligation, PVL	Full term		NVD	NA		10.8		Negative	Inability to grasp objects at 1.3 months	FL
5	1.6/M	OS	Down’s syndrome, operated VSD and PDA ligation, PVL	Full term		NVD	NA		10.8		Negative	Inability to grasp objects at 1.3 months	FL
6	0.7/F	OS	Down’s syndrome, PVL, ASD, VSD	Full term		NVD	NA		9.6		Negative	Leukocoria at 3 days	RO
7	6.1/M	OD	Down’s syndrome, PVL, VSD, ASD, PDA, hypothyroid	Full term		NVD	NA		11.4		Negative	DOV at 5 years	2
7	6.1/M	OS	Down’s syndrome, PVL, VSD, ASD, PDA, hypothyroid	Full term		NVD	NA		11.4		Negative	DOV at 5 years	2
8	0.5/M	OD	Neonatal seizures, sepsis	Full term		NVD	1600, yes		8.2		Toxoplasma and CMV Ig G positive	Leukocoria at 2 months	NFL
8	0.5/M	OS	Neonatal seizures, sepsis	Full term		NVD	1600, yes		8.2		Toxoplasma and CMV Ig G positive	Leukocoria at 2 months	NFL
9	0.3/M	OD	Rubella and anti CMV Ig G +	Preterm, premature rupture of membranes		LSCS	NA, yes		9.3		Rubella and CMV Ig G positive	Leukocoria at birth	FL
9	0.3/M	OS	Rubella and anti CMV Ig G +	Preterm, premature rupture of membranes		LSCS	NA, yes		9.3		Rubella and CMV Ig G positive	Leukocoria at birth	FL
10	7.1/M	OD	Marfan’s syndrome	Preterm		NVD	1200, yes		9.6		Negative	DOV at 6 years	0.77
10	7.1/M	OS	Marfan’s syndrome	Preterm		NVD	1200, yes		9.6		Negative	DOV at 6 years	0.77
11	13.3/F	OD	Type 1 diabetes mellitus, focal neurological deficit	Full term		NVD	NA		14.2		Negative	DOV at 12 years	1.47
11	13.3/F	OS	Type 1 diabetes mellitus, focal neurological deficit	Full term		NVD	NA		14.2		Negative	DOV at 12 years	0.3
12	1.7/M	OD	PVL, microcephaly	Full term, delayed cry		NVD	NA		8.6		Negative	Leukocoria at 1 year	NFL
12	1.7/M	OS	PVL, microcephaly	Full term, delayed cry		NVD	NA		8.6		Negative	Leukocoria at 1 year	NFL
13	7/M	OD	Nephrotic syndrome, seizure disorder, hypothyroid	Full term		LSCS	NA		NA		Negative	Leukocoria at 6.5 years	0.3
13	7/M	OS	Nephrotic syndrome, seizure disorder, hypothyroid	Full term		LSCS	NA		NA		Negative	Leukocoria at 6.5 years	3
14	0.08/F	OD	Down’s syndrome, ASD, rubella and anti CMV Ig G +	Preterm, oligohydramnios		LSCS	1900, yes		11.2		Rubella Ig G positive	Leukocoria at birth	UC
14	0.08/F	OS	Down’s syndrome, ASD, rubella and anti CMV Ig G +	Preterm, oligohydramnios		LSCS	1900, yes		11.2		Rubella Ig G positive	Leukocoria at birth	UC
15	0.1/M	OS	Down’s syndrome	Full term		LSCS	2750, yes		8.2		Negative	Leukocoria at birth	NFL
16	1.3/F	OD	Seizure disorder	Full term, multiple pregnancies		LSCS	1350, yes		9		Negative	Leukocoria at 1 year	NFL
16	1.3/F	OS	Seizure disorder	Full term, multiple pregnancies		LSCS	1350, yes		9		Negative	Leukocoria at 1 year	FL
17	14/M	OD	PVL	Full term		NVD	1500, no		13.2		Negative	DOV at 13.5 years	2
17	14/M	OS	PVL	Full term		NVD	1500, no		13.2		Negative	DOV at 13.5 years	2
18	9/M	OD	PVL	Full term, delayed cry		NVD	NA, yes		9.6		Negative	Leukocoria at birth	2
18	9/M	OS	PVL	Full term, delayed cry		NVD	NA, yes		9.6		Negative	Leukocoria at birth	2
19	3.2/M	OS	Cryptogenic West syndrome	Full term		NVD	2500, no		11.1		Negative	Leukocoria at 3 years	UC
20	4.4/M	OD	PVL	Full term		NVD	NA		9.0		Negative	Leukocoria at 3.5 years	UC
21	8.1/F	OD	Marfan’s syndrome	Full term		NVD	NA		10.4		Negative	DOV at 6.5 years	0.6
21	8.1/F	OS	Marfan’s syndrome	Full term		NVD	NA		10.4		Negative	DOV at 6.5 years	0.6
22	0.1/M	OD	Congenital rubella syndrome	Full term, pustular eruptions at 2 months of gestation, delayed cry		LSCS	NA, yes		10		Rubella IgG positive	Leukocoria at birth	FO
22	0.1/M	OS	Congenital rubella syndrome	Full term, pustular eruptions at 2 months of gestation, delayed cry		LSCS	NA, yes		10		Rubella IgG positive	Leukocoria at birth	NFO
23	6.1/M	OD	PVL	Full term		NVD	NA, yes		12		Negative	DOV since childhood	0.6
23	6.1/M	OS	PVL	Full term		NVD	NA, yes		12		Negative	DOV since childhood	1.77
24	7/M	OD	Marfan’s syndrome	Full term		NVD	NA		9		Negative	Not following light since birth	3
24	7/M	OS	Marfan’s syndrome	Full term		NVD	NA		9		Negative	Not following light since birth	3
25	1/M	OD	PVL, cerebral palsy, CMV Ig G +	Full term, obstructed labor		LSCS	4500, yes		8.8		CMV Ig G positive	Watering since birth	UC
25	1/M	OS	PVL, cerebral palsy, CMV Ig G +	Full term, obstructed labor		LSCS	4500, yes		12		CMV Ig G positive	Watering since birth	UC
26	0.5/F	OD	PVL, seizure disorder	Full term, multiple pregnancies, delayed cry		LSCS	NA, yes		7		Negative	On pediatric referral	FO
26	0.5/F	OS	PVL, seizure disorder	Full-term, multiple pregnancies, delayed cry		LSCS	NA, yes		7		Negative	On pediatric referral	FO
27	1.6/M	OD	Infantile spasm, PVL, seizure disorder	Full term		NVD	NA, yes		8.6		Negative	Leukocoria since birth	FL
27	1.6/M	OS	Infantile spasm, PVL, seizure disorder	Full term		NVD	NA, yes		8.6		Negative	Leukocoria since birth	FL
28	8.5/M	OD	Nephrotic syndrome	Full term		NVD	NA		11.5		Negative	Leukocoria at 5.5 years	0.47
28	8.5/M	OS	Nephrotic syndrome	Full term		NVD	NA		11.5		Negative	Leukocoria at 5.5 years	0.3
29	1.5/F	OD	PVL, seizure disorder	Preterm		NVD	NA, yes		10.2		Negative	Leukocoria at 1 year	FL
30	11.3/M	OD	Tourette's syndrome	Full term		NVD	NA		NA		Negative	DOV 10 years	3
30	11.3/M	OS	Tourette's syndrome	Full term		NVD	NA		NA		Negative	DOV 10 years	3
Patient no.	Diagnosis		Abnormal eye movements	Squint	Morphology of cataract	USG for posterior segment			Visual evoke potential (µV/ms)	Axial length (mm)		Keratometry (D)	Corneal diameter (mm)
1	OU congenital cataract with nystagmus		Nystagmoid	Absent	Zonular cataract with anterior capsular plaque	anechoic			NA	20.39		47.3	10.5
1	OU congenital cataract with nystagmus		Nystagmoid	Absent	Zonular cataract with anterior capsular plaque	anechoic			NA	20.39		46.3	10.5
2	OU congenital cataract		Nystagmus	Absent	Zonular cataract	anechoic			11.9/101	18		47.6	10
2	OU congenital cataract		Nystagmus	Absent	Zonular cataract	anechoic			15.6/96	18		47.1	10
3	OU Ectopia lentis		Absent	Absent	Temporal 8 clock hours subluxated clear lens	anechoic			NA	26.4		43.1	11.7
4	OS developmental cataract with Retinal detachment on USG		Absent	Absent	Total cataract	Retinal detachment			NA	21.62		46.3	12
5	OU congenital cataract with RCS		Absent	Esotropia	Total cataract	anechoic			NA	23.9		46.9	12
5	OU congenital cataract with RCS		Absent	Absent	Zonular cataract	anechoic			NA	23.41		46.7	12
6	OS congenital cataract with LCS with amblyopia		Absent	Esotropia	Total cataract	anechoic		20/136		20.97		49.1	12
7	OU developmental cataract		nystagmus	Esotropia	Zonular cataract	anechoic		NA		23.99		44.7	11
7	OU developmental cataract		nystagmus	Esotropia	Zonular cataract	anechoic		NA		23.5		44.2	11
8	OU developmental cataract		nystagmoid	Absent	Zonular cataract	anechoic		No significant response		19.55		45.5	10.5
8	OU developmental cataract		nystagmoid	Absent	Zonular cataract	anechoic		No significant response		19.54		46.2	10.5
9	OU congenital cataract		Absent	Absent	Total cataract	anechoic		14/109		18.1		48	10.5
9	OU congenital cataract		Absent	Absent	Total cataract	anechoic		24/109		18.07		47.7	10.5
10	OU ectopia lentis		Absent	Absent	IN subluxated lens from 9-1 o’clock	anechoic		NA		22.04		44.7	11
10	OU ectopia lentis		Absent	Absent	Superior subluxated lens 2-10 o’clock	anechoic		NA		21.91		44.3	11
11	OU PSC		Absent	Absent	PSC	anechoic		NA		22.85		43.1	11.5
11	OU PSC		Absent	Absent	PSC	anechoic		NA		22.8		43.3	11.5
12	OU microphthalmos with developmental cataract		nystagmoid	Absent	Posterior capsule plaque	anechoic		2/156		17.54		47.1	9.5
12	OU microphthalmos with developmental cataract		nystagmoid	Absent	Posterior capsule plaque	anechoic		3.1/124		17.7		48.87	9.5
13	OU steroid-induced cataract		Absent	Absent	Total cataract	anechoic		NA		21.86		45.6	10.5
13	OU steroid-induced cataract		Absent	Absent	Total cataract	anechoic		NA		22.04		45.2	10.5
14	OU congenital cataract		Absent	Absent	PPC	anechoic		12.5/172		17.7		43.4	10
14	OU congenital cataract		Absent	Absent	PPC	anechoic		20/172		17.81		43.6	10
15	OS congenital cataract		Absent	Absent	Total cataract	anechoic			16.5/123	18.9		42.4	11
16	OU congenital cataract with RCS		Absent	Esotropia	Total cataract	anechoic			NA	20.15		48.8	11
16	OU congenital cataract with RCS		Absent	Absent	Total cataract	anechoic			NA	20.4		49	11
17	OU developmental cataract with RCS		Absent	Esotropia	Zonular cataract	anechoic			11/95	28.77		45.2	11
17	OU developmental cataract with RCS		Absent	Absent	Zonular cataract	anechoic			20/104	25.96		46.1	11.5
18	OU developmental cataract with LCS with nystagmoid movements		nystagmoid	Absent	Zonular cataract	anechoic			17/89	26.41		43.7	11.5
18	OU developmental cataract with LCS with nystagmoid movements		nystagmoid	Esotropia	Zonular cataract	anechoic			20/80	26.72		45.5	11
19	OS cataract with Retinal detachment on USG		Absent	Absent	Total cataract	Retinal detachment			9/117	20.5		43	10.5
20	OU High myopia OD total cataract with RDS, OS optic atrophy		Absent	Exotropia	Total cataract	anechoic			10/111	26.59		46.2	10.5
21	OU ectopia lentis		Absent	Exotropia	Temporal subluxated lens 12-7 o’clock	anechoic			NA	21.91		42.6	12.38
21	OU ectopia lentis		Absent	Exotropia	Temporal subluxated lens 6-12 o’ clock	anechoic			NA	22		42.3	12.4
22	OU congenital cataract		Absent	Absent	Membranous cataract	anechoic			NA	17.76		44.6	9.5
22	OU congenital cataract		Absent	Absent	Membranous cataract	anechoic			NA	17.73		43.7	9.5
23	OU developmental cataract		Absent	Absent	Zonular cataract	anechoic			20/96	22.93		43.8	11
23	OU developmental cataract		Absent	Exotropia	Zonular cataract	anechoic			8.5/95	22.68		42.8	11
24	OU total cataract with nystagmus with ACS		Nystagmus	Esotropia	Total cataract	anechoic			12.1/86	23.17		43.7	11
24	OU total cataract with nystagmus with ACS		Nystagmus	Esotropia	Total cataract	anechoic			25.7/86	23.9		42.7	11
25	OU PCG with operated trab with congenital cataract with nystagmus		nystagmus	Absent	Partially absorbed membranous cataract	Optic nerve head cupping			NA	20.53		40.9	14
25	OU PCG with operated trab with congenital cataract with nystagmus		Nystagmus	Absent	Partially absorbed membranous cataract	Optic nerve head cupping			NA	21.5		42.4	13
26	OU congenital cataract		Absent	Absent	Total cataract	anechoic			20/146	19.9		46.2	9
26	OU congenital cataract		Absent	Absent	Total cataract	anechoic			20/136	20.09		44.6	9
27	OU congenital cataract		Nystagmoid	Absent	Anterior polar cataract	anechoic			14.9/106	17.91		45.9	10
27	OU congenital cataract		Nystagmoid	Absent	Anterior polar cataract	anechoic			11.7/115	17.92		43.7	10
28	OU developmental cataract		Absent	Absent	Zonular cataract	anechoic			7.4/83	25.98		40	10.5
28	OU developmental cataract		Absent	Absent	Zonular cataract	anechoic			14/79	25.84		39.5	10.5
29	OU developmental cataract		Absent	Absent	Total cataract	anechoic			6.5/140	17.72		49.3	11
29	OU developmental cataract		Absent	Absent	Total cataract	anechoic			NA	17.48		50.5	11
30	OU advanced keratoconus with healed hydrops with subluxated cataractous lens		Nystagmoid	Exotropia	Superior subluxated cataractous lens	anechoic			No significant response	21.1		57.12	11.5
30	OU advanced keratoconus with healed hydrops with subluxated cataractous lens		Nystagmoid	Exotropia	Superior subluxated cataractous lens	anechoic			No significant response	21.9		67.1	11.5

ACS=alternate convergent squint; ASD=atrial septal defect; BE=both eyes; CMV=cytomegalovirus; D=diopters; DOV=diminution of vision; FL=follows light; FO=follows object; IN=infero-nasal; LCS=left convergent squint; logMAR=logarithm of minimum angle of resolution; LSCS: lower segment cesarean section; mm=millimeters; NA=not available; NFL=not follow light; NFO=not follow object; NVD: normal vaginal delivery; OD=right eye; OS=left eye; OU=both eyes; PCG=primary congenital glaucoma; PDA=patent ductus arteriosus; PPC=posterior polar cataract; PSC=posterior subcapsular cataract; PVL=periventricular leukomalacia; RCS=right convergent squint; RO=resists occlusion; Trab=trabeculectomy; TORCH=toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV; UC=uncooperative; USG=ultrasound; VSD=ventricular septal defect; μV=microvolt; ms=milliseconds

BSCVA was measurable quantitively in 23 eyes (13 patients), and at presentation, it was 1.40 logarithm of the minimum angle of resolution (logMAR) (range 0.3 to 3 logMAR), whereas qualitative data on VA was available for 31 eyes. Fixation was central and maintained in 48.1% of eyes (26/54), whereas it was poor in 24.1% (13/54).

The mean maximum corneal diameter was 10.8 ± 0.95 mm (range: 9.0 to 14.0 mm). Microcornea (corneal diameter <9 mm in newborns and <10 mm after >2 years old) was not noted in any patient. Strabismus was observed in 27.7% of eyes (15/54), out of which nine eyes had esotropia and six had exotropia. Nystagmus and nystagmoid movements were documented in 14.8% (8/54) and 18.5% (10/54) of patients, respectively. EUA was performed to record the essential parameters for intraocular lens (IOL) power calculation in infants and young and mentally impaired children, whereas optical biometry was used in older children. The mean axial length documented using the indentation method was 21.37 ± 2.96 mm (range: 17.48 to 28.77 mm). The mean keratometry noted using a handheld autokeratometer without a speculum was 45.72 ± 4.14 diopters (D) (range: 39.50 to 67.10 D).

The most common cataract morphology was that of zonular cataract (17 eyes), followed by total cataract (16 eyes), ectopia lentis with clear lens (5 eyes), ectopia lentis with cataractous lens (2 eyes), membranous cataract (4 eyes), and two eyes each of anterior capsular plaque, posterior capsular plaque, posterior subcapsular cataract, anterior polar cataract, and posterior polar cataract.

Fundoscopy could be performed in 13 patients (43%) preoperatively; in the rest of the cases, it was performed postoperatively. Preoperative retinal detachment, Leber’s congenital amaurosis, and primary congenital glaucoma were present in two eyes each. Because a majority of the patients had pre-existing multisystem disorder, the various systemic associations were periventricular leukomalacia (40%; 12/30), Down’s syndrome (20%; 6/30), seizure disorder (20%; 6/30), cardiac valvular anomalies (20%; 6/30), Marfan’s syndrome (13.3%; 4/30), hypothyroidism (13.3%; 4/30), rubella (10%; 3/30), cytomegalovirus IgG positive (10%; 3/30), cerebral palsy (6.6%; 2/30), nephrotic syndrome (6.6%; 2/30), Type 1 diabetes mellitus (3.3%; 1/30), microcephaly (3.3%; 1/30), cryptogenic West syndrome (3.3%; 1/30), congenital rubella syndrome (3.3%; 1/30), and Tourette syndrome (3.3%; 1/30). Seventeen patients (56.6%) were diagnosed with microcytic, hypochromic iron deficiency anemia on admission as age-based hemoglobin levels were less than 2 SD below the mean.

Six patients were born at preterm, that is, less than 37 weeks (two patients of premature rupture of membranes, one patient of multiple pregnancies, one patient of rubella infection, one patient of oligohydramnios, and one patient of preterm labor with an unidentifiable cause). Six patients had a history of delayed crying at birth with the 5-minute appearance, pulse, grimace, activity, and respiration (APGAR) score being less than 7 out of 10. History of cesarean section was given by 9 out of 30 patients, and six patients provided a history of low birth weight (three low birth weight, two very low birth weight, and one extremely low birth weight). History of neonatal intensive care unit (NICU) stay was present in almost 50% of patients (14/30). A family history of congenital or developmental cataract was present in three patients. The TORCH profile on admission revealed Rubella IgG positivity in three patients, cytomegalovirus (CMV) IgG in two patients, and toxoplasma IgG in one patient.

Intraoperative findings

Table 2 shows the intraoperative findings of each case. Lens aspiration with posterior continuous curvilinear capsulorhexis (PCCC) and anterior vitrectomy (AV) alone was performed in 42.5% of eyes (23/54), whereas intraocular lens implantation was performed in the primary sitting in 57.5% of eyes (31/54). A single-piece, acrylic, hydrophobic IOL (ZCB00, Abbott Medical Optics, Santa Ana, CA, USA) was implanted in the bag for 40.7% eyes (22/54) and three-piece IOL (AcrySof MA60AC, Alcon laboratories, Fortworth, TX) in the sulcus with optic capture in 16.6% of eyes (9/54) [Fig. 1a-1d]. ILLA with intrascleral haptic fixation of IOL was performed in three eyes of ectopia lentis [Fig. 1e and f], whereas four eyes were left aphakic intraoperatively, and two cases of poorly dilating pupil and one case each of anterior capsular fibrosis, posterior capsule plaque, zonular dialysis, posterior synechiae, and posterior capsular dehiscence were noted. Complications such as intraoperative extension of posterior capsulorhexis margin and iatrogenic iris sphincter damage were noted in one case each.

Table 2.

Intraoperative and postoperative findings

Surgery	Intraocular lens status	Intraoperative difficulties/complications	Intraoperative posterior segment evaluation	Visual acuity (logMAR) POD 1	IOP POD 1	Refraction POD 1 (Diopters)	CDVA (logMAR) at the last follow-up	Refraction at last follow-up (Diopters)
LA + PCCC + AV + PCIOL	Single-piece FHA	Nil	Temporal disc pallor	UC	normal	+5.5	NA	NA
LA + PCCC + AV + PCIOL	Single-piece FHA	Nil	Temporal disc pallor	UC	normal	+5.5	NA	NA
LA + PCCC + AV	Aphakic	Nil	Temporal disc pallor	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	Temporal disc pallor	FL	normal	NA	NA	NA
LA + SFIOL	Three-piece FHA	Nil	Tessellated fundus	0.17	16	+1.75	NA	+1.75
Preparatory LA + PCIOL	Single-piece FHA	Nil	Retinal detachment	FL	normal	NA	NA	NA
LA + PCCC + AV + PCIOL	Single-piece FHA	Nil	WNL	FL	normal	+5	NA	+5
LA + PCCC + AV + PCIOL	Single-piece FHA	SN extension of PCCC	WNL	FL	normal	+5	NA	+5
LA + PCCC + AV + PCIOL in sulcus	Three-piece FHA	PC defect	WNL	UC	normal	NA	NA	NA
LA + PCIOL	Single-piece FHA	Nil	WNL	0.60	14	+0.75	UC	−2
LA + PCIOL	Three-piece FHA	Nil	WNL	0.60	14	+0.75	UC	−2.75
LA + PCCC + AV	Aphakic	Nil	WNL	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	WNL	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	Salt and pepper retinopathy	FL	normal	+19	NA	+19
LA + PCCC + AV	Aphakic	Nil	Salt and pepper retinopathy	FL	normal	+19	NA	+19
ILLA + SFIOL	Three-piece FHA	Nil	Tessellated fundus	0.60	12	+1.25	0.3	−2.75
ILLA + SFIOL	Three-piece FHA	Nil	Tessellated fundus	0.77	14	+1.25	0.3	−2.5
LA + PCIOL	Single-piece FHA	Nil	WNL	0	14	+1	NA	+1
LA + PCIOL	Single-piece FHA	Nil	WNL	0	16	+1.5	NA	+1.5
LA + PCCC + AV	Aphakic	Nil	Temporal disc pallor	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Anterior and posterior synechiae, shallow AC, zonular laxity, retrolental membrane	Temporal disc pallor	FL	normal	NA	NA	NA
LA + PCIOL	Single-piece FHA	Nil	WNL	0.17	16	NA	0	-1.5
LA + PCIOL	Single-piece FHA	Nil	WNL	0	14	NA	0	-2.5
LA + PCCC + AV	Aphakic	Nil	WNL	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	WNL	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Anterior capsule fibrosis (9-11 o clock)	WNL	UC	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	WNL	UC	normal	NA	UC	+10.75
LA + PCCC + AV	Aphakic	Superior iatrogenic iris sphincter tear	WNL	UC	normal	NA	UC	+11.75
LA + PCIOL	Three-piece FHA	Nil	WNL	0.30	14	+4	NA	+4
LA + PCIOL	Three-piece FHA	Nil	WNL	0.47	12	+3.5	NA	+3.5
LA + PCIOL	Single-piece FHA	Nil	WNL	2	10	+2	NA	+2
LA + PCIOL	Single-piece FHA	Nil	WNL	2	10	+1.25	NA	+1.25
LA + PCIOL	Three-piece FHA	Nil	Retinal detachment	FL	normal	NA	NA	NA
LA + PCIOL	Single-piece FHA	Nil	WNL	UC	normal	NA	NA	NA
ILLA + AV	Aphakic	Nil	WNL	0.30	10	NA	NA	NA
ILLA + AV	Aphakic	Nil	WNL	1	12	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	Salt and pepper retinopathy	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	Salt and pepper retinopathy	FL	normal	NA	NA	NA
LA + PCIOL	Single-piece FHA	Nil	WNL	0.60	14	−1.25	0.3	−1.25
LA + PCIOL	Single-piece FHA	Nil	WNL	1.77	12	−1.75	1.17	−3.75
LA + PCCC + AV + PCIOL	Three-piece FHA	Nil	WNL	3	16	NA	NA	NA
LA + PCCC + AV + PCIOL	Three-piece FHA	Nil	WNL	3	14	NA	NA	NA
LA + PCCC + AV + PCIOL	Three-piece FHA	Nil	CDR: 0.8:1, NRR thin, FR sharp	UC	normal	NA	NFL	−7
LA + PCCC + AV + PCIOL	Three-piece FHA	Nil	WNL	UC	normal	NA	NFL	+3.75
LA + PCCC + AV	Aphakic	Nil	WNL	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Nil	WNL	FL	normal	NA	NA	NA
LA + PCCC + AV	Aphakic	Poorly dilating pupil	WNL	FL	normal	NA	UC	+23.75
LA + PCCC + AV	Aphakic	Poorly dilating pupil	WNL	FL	normal	NA	UC	+20.5
LA + PCIOL	Single-piece FHA	Nil	WNL	0.30	12	+2.25	NA	+2.25
LA + PCIOL	Single-piece FHA	Nil	WNL	0.17	14	+2.25	NA	+2.25
LA + PCCC + AV	Aphakic	Nil	Optic disc pallor	UC	normal	NA	UC	+22
LA + PCCC + AV	Aphakic	Nil	Optic disc pallor	UC	normal	NA	UC	+21
LA	Aphakic	Nil	Bony spicules like pigmentation in mid-periphery, optic disc pallor, CR degeneration	3	12	+8	3	+8

AC=anterior chamber; AV=anterior vitrectomy; CDR=cup disc ratio; CR=chorio-retinal; FHA=foldable hydrophobic acrylic; FL=follows light; FR=foveal reflex; ILLA=intra-lenticular lens aspiration; IOP=intraocular pressure; LA=lens aspiration; NA=not available; NFL=not follow light; NRR=neuro-retinal rim; PCCC=posterior continuous curvilinear capsulorhexis; PCIOL=posterior chamber intraocular lens; POD=postoperative day; SFIOL=scleral fixated intraocular lens; SN=supero-nasal; UC=uncooperative

Figure 1.

(a) An 8-year-old child with zonular cataract. (b) Lens aspiration with single-piece, acrylic, hydrophobic IOL implantation was performed. (c) A 1 year old child with congenital rubella syndrome presenting with membranous cataract and posterior synechiae. (d) Membranectomy and implantation of three-piece IOL in the sulcus with optic capture was performed. (e) A 9-year-old child with Marfans’s syndrome diagnosed with ectopia lentis in both eyes. (f) ILLA was completed with intrascleral haptic fixation of IOL (SFIOL)

Postoperative findings

Table 2 also shows the postoperative outcomes in all cases. Post-operation, on day 1, CDVA was measurable quantitively in 23 eyes of 13 patients, and the mean CDVA improved to 1.03 logMAR (range 0 to 3 logMAR). Although the objective gain was minimal, in most cases, the parents reported some subjective improvements like responding to parents’ facial gestures (64%), smiling at toys (56%), responding to hand gestures (45%), and ability to recognize parents and other family members (87%). Two eyes of primary congenital glaucoma with operated trabeculectomy presented with a raised IOP of 26 mm Hg in one eye postoperatively at 2 months and was managed with medical therapy. Postoperative CDVA was measurable quantitively in 23 eyes (13 patients), the mean of which at a 2-year follow-up improved to 1.00 logMAR (range 0 to 3 logMAR). Refraction was possible in 31 eyes at a 2-year follow-up with a mean spherical equivalent (SE) of 5.51 ± 8.72 D (range -7 to + 24 D). At the final review, 27.7% of eyes (15/54) were noted to have strabismus, the same as that preoperatively. No cases of PCO or secondary glaucoma or IOL decentration were reported at the final follow-up. None of the subjects required additional intervention following cataract surgery.

On average, every child was seen by three physicians, and the mean duration between the first visit to a physician and presentation to our center was 2.23 ± 0.67 years. The various causes for delay in referral include multiple referrals due to lack of GA services in 78% of cases, a long waiting list at the referral hospital in 35% of cases, and lack of awareness at the primary-care physician level in 50% of cases.

Discussion

Severe vision impairment in syndromic patients or those with intellectual impairment associated with untreated cataract has a significant adverse impact on health-related quality of life, especially on mental well-being. Those patients in whom there is significant visual gain following cataract surgery are expected to be more confident, cope with future challenges, and socialize with greater ease. To our knowledge, we present here the outcomes of cataract surgery in the largest series of pediatric patients with systemic comorbidities at our center.

Our mean age at surgery was closer to the results from Ethiopia and Iran than other countries.[4,5] The reasons could be late diagnosis and late surgery, less severity or peripheral lens opacity with acceptable visual acuity, unavailable subspecialty of pediatric ophthalmologists in rural regions, poor economic status, or a combination of these etiologies. In our study, there was no significant difference between the age of presentation and the age at the time of cataract surgery (results not shown). Being a tertiary-care center, most of the cases were operated at the earliest following the first presentation. This was relevant because many patients had white cataracts and poor vision during their first visit to our hospital. Children with Marfan’s syndrome belonged to a comparatively older age group as ectopia lentis prompts children and their families to seek medical care after a decade or so because of the absence of leukocoria. Our study had unequal gender distribution with a male: female ratio of 2:1, similar to other Asian studies, which may explain the gender bias in our society or a higher prevalence of systemic diseases in male patients.[6,7] Cesarean section and low birth weight have been reported to increase the risk of congenital cataracts with a 10.6-fold higher risk of developing a bilateral cataract, though our study was unable to reveal similar findings.[8] The majority of our subjects had bilateral cataracts, which can be very well described by the predominance of genetic diseases, metabolic diseases, and maternal infections in our study population. This has been consistent with the previously reported literature as well.[5,9] The presence of nystagmus and nystagmoid movements in our case series was found to be much higher (33.3%) than in studies that have reported 14.2% and 0.09%.[5,9] This enormous variability in data can be explained by the higher percentage of patients with Down’s syndrome in our cohort which is known to be associated with nystagmus in 18–30% of cases.[10]

The most common morphological variant of cataract in our series was zonular cataract, whereas studies from Iran and South Korea have found posterior subcapsular cataract and nuclear cataract as the most common types, respectively.[5,11] Young children with early onset severe vision impairment can experience delayed motor, language, emotional, social, and cognitive development, with lifelong consequences. Because the visual acuity in children would be dependent on the child’s attention span and cooperation, we chose to use postoperative refraction (objective measurement) at a 2-year follow-up as our outcome parameter. Quantitative assessment of VA was feasible in 42.5% of eyes. As per the WHO criteria, manifest blindness is defined as VA ranging from 1/60 to just perception of light in terms of Snellen’s. Our case series did reveal the preoperative mean BSCVA to be 1.40 logMAR (range 0.3 to 3 logMAR) which perfectly fits into the definition of manifest blindness. Nevertheless, an overall improvement in the mean postoperative CDVA at a 2-year follow-up was present (1.00 logMAR or Snellen’s equivalent of 6/60), and we were unable to assess the exact role of an amblyopic component in our patients. There is a huge possibility that the majority of the patients were unable to attain good vision postoperatively due to amblyopia. Also, as mentioned previously, it may be difficult to accurately assess the visual function and visual acuity in these patients due to subnormal mental intelligence. This highlights the fact that the associated systemic and ocular comorbidity definitely hampers the final visual outcomes. We found a low prevalence of associated ocular comorbidities in our study, and some of these such as retinal detachment, advanced optic nerve head cupping, and Leber’s congenital amaurosis were directly responsible for poor visual outcomes.

Systemic associations related to cardiac or endocrine or neurological systems might often delay surgery in these patients as they require an extensive pre-anesthetic check-up before administering GA. In fact, a major factor for delayed presentation in our series was the lack of an adequate anesthesia facility at the primary-care setup and the inability of the parents to bring the patients to the referral center, even after counseling by the primary-care physician due to socio-economic issues. Anesthetic evaluation of such syndromic patients has to have a meticulous search for associated conditions, the involvement of other organ systems, and airway abnormalities. Furthermore, bilateral cataract surgeries were performed in the same sitting in cases with bilateral white cataracts to avoid the risk of a second exposure to GA and expedite visual rehabilitation in our patients.

We observed a high rate (71.4%) of subluxation of the lens and strabismus (57.1%) and a low rate of myopic astigmatism in patients with Marfan’s syndrome. Visual rehabilitation with scleral fixated IOL and posterior chamber in the bag IOL implantation in the primary sitting was possible in 42.8% and 28.5% of cases, respectively. A review including nine patients with subluxation of the lens secondary to Marfan syndrome showed significant improvement of VA (0.5 ± 0.3 logMAR at the baseline to 0.2 ± 0.2 logMAR after surgery) in lensectomy with secondary artisan IOL implantation.[12]

There is a paucity of literature regarding outcomes of cataract surgery in patients with hypoxic ischemic insult, seizure disorder, hypothyroidism, and other rare syndromes. However, with hypothyroidism and seizure disorder, it must be kept in mind that complications during anesthesia, such as circulatory depression, faster inhalation induction, potentiation of anesthetic agents, neuromuscular blockade, and hypothermia, can be expected. In most such cases, the outcomes are expected to be poor. In our series, we did observe a hesitancy by some secondary-care centers to operate upon such cases in view of nil or extremely guarded prognosis and an approach toward easy referral to other centers. However, as mentioned in the Results section, parents reported subjective improvement in most (70%) of our cases. Thus, we believe that such cases should be operated upon, whenever the infrastructure is there, and unnecessary delay in surgery should be avoided by referring the patient to other centers. In addition to neurological abnormalities, amblyopia could be a contributing factor in such cases and by avoiding the delay in surgery, the visual outcomes of cataract surgery could be improved.

In previous studies on congenital or infantile cataracts, 3–7% of cases were associated with Down’s syndrome.[13,14,15,16] The reason behind early cataractogenesis has been attributed to factors such as susceptibility to oxidative stress and the build-up of amyloid precursor protein and amyloid-β peptides in the lens.[14,15] It has also been suggested that individuals with Down’s syndrome may have a wide variation in visual acuity due to differential characteristics between the eye and the brain and other associated neuropathologies.[16]

Because congenital rubella syndrome is a multisystem disorder, other associated ocular (salt and pepper retinopathy), as well as systemic disorders (neurological, hearing, cardiovascular, and speech abnormalities), can affect visual outcomes after cataract surgery. Two out of three rubella-positive cases had the presence of salt and pepper retinopathy in our study. The incidence of TORCH infections is high in the Indian subcontinent, and up to 20% of cases may be seropositive.

The limitations of the current study are its retrospective nature because of which data regarding anesthetic-related challenges could not be obtained. Secondly, we were unable to evaluate the exact role of the amblyopic component in our patients. Lastly, the psychological impact of the surgery and visual rehabilitative measures (spectacle aid and amblyopia therapy) on parents could not be assessed. In spite of all these confines, we believe that this study would possibly add some benefit to the existing literature.

With increasing safety and efficacy of cataract surgery in the present world, screening and early treatment for visually significant cataracts is beneficial in this population. Recommendations for early cataract surgery necessitate anesthetizing infants with diagnosed medical conditions. Careful preoperative assessment and planning are a prerequisite for a safe anesthetic outcome. Simultaneous bilateral cataract surgery may be considered in this group of patients to enhance their quality of life. The American Academy of Pediatrics recommends that an eye exam should be carried out in these children by 6 months of age with follow-up exams once per year or more frequently if indicated by the ophthalmologist. We recommend that this should be followed in developing countries too where the primary focus of public health is senile cataract and pediatric cataract has not received its due attention.

Conclusion

To our knowledge, this is the first study aimed at highlighting the delayed presentation for cataract surgery in the pediatric age group with systemic comorbidities and subjective improvement in psychomotor skills in the postoperative period. Understanding the reasons for delayed presentation and/or surgery will provide valuable insights to reduce the time gap between the onset of vision-impairing cataract and surgery. Reducing these gaps will improve the visual outcomes of childhood cataract surgery and thus contribute to achieving one of the main priorities of VISION 2020: The Right to Sight Initiative.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.