Graefes Arch Clin Exp Ophthalmol DOI 10.1007/s00417-015-2983-5

PEDIATRICS

Strabismus in infants following congenital cataract surgery Elif Demirkilinc Biler 1 & Duygu Inci Bozbiyik 2 & Onder Uretmen 1 & Suheyla Kose 3

Received: 14 October 2014 / Revised: 20 February 2015 / Accepted: 2 March 2015 # Springer-Verlag Berlin Heidelberg 2015

Abstract Purpose This study aimed to determine the incidence and characteristics of strabismus following congenital cataract surgery in infants. Materials and methods Patients aged 2 Snellen lines. The remaining five patients had at least one amblyogenic risk factor necessitating amblyopia treatment. Secondary IOL implantation was performed in five (15.6 %) patients in group 1 and in two (4.3 %) patients in group 2. Additional surgery for aphakic glaucoma was needed in five (15.6 %) patients in group 1 and in 12 (25.5 %) patients in group 2. There was not a significant relationship between

Graefes Arch Clin Exp Ophthalmol Table 2

Patient characteristics and clinical findings of children with nystagmus

Sex • Male • Female Laterality, n • Unilateral • Bilateral Age at cataract surgery (months) Follow-up period (months) Cataract density, n • Partial • Total Presence of strabismus • Esotropia • Exotropia Development of aphakic glaucoma, n (%) Additional ocular surgery, n (%) a

Pearson chi-square test

b

Mann–Whitney U test

Nystagmus+(n=49)

Nystagmus – (n=30)

P value

30 19

18 12

0.91a

4 45 4.2±2.8 months (1–12 months) 59.6±40.4 (12–168)

10 20 4.3±2.9 months (1–11 month) 35.5±32.1 (12–175)

0.004a

10 39

9 21

0.33a

21 9 23 (46.9) 18 (36.7)

11 6 7 (23.3) 5 (16.7)

0.71a

the need for additional surgery and the incidence of strabismus (Pearson’s chi-square test, p=0.73; chi-square value=0.12) or the type of strabismus (Pearson’s chi-square test, p=0.552; chi-square value=0.36).

Discussion A high incidence of strabismus in patients with congenital cataract has been reported by many researchers; the incidence of strabismus in the general population is estimated to be 1.3– 4.5 % [12–16], whereas the prevalence of strabismus in patients with congenital cataract is 24.2–84 % [3–5, 8, 11, 17]. According to some researchers, the occurrence of strabismus increases following cataract surgery [2, 4, 5]. France [4] reported that strabismus was present preoperatively in 40 % of children at the time of diagnosis, and that it interfered with visual rehabilitation in 86 % of children that underwent surgery for congenital cataract. It has also been reported that strabismus was observed in 21 of 25 children that had congenital cataract surgery during the first 6 months of life [5]. One study reported that 16 (76.1 %) of 21 patients developed strabismus following bilateral cataract surgery performed before the age of 12 months [7]. In the present study, the incidence of strabismus was higher than in the general population. Among the 79 patients in the present study that underwent congenital cataract extraction before the age of 12 months, 47 (59.5 %) developed strabismus during the post-surgery follow-up period.

0.97b 0.001b

0.04a 0.57a

Hiles and Sheridan [3] reported that early cataract surgery and vigorous optical rehabilitation did not appear to ensure protection against strabismus. It was also reported that despite early surgical intervention and optimal postoperative management, nystagmus and strabismus were observed in a large majority of patients [11]. Magli et al. [18] reported that age at the time of cataract diagnosis and age at the time of surgery were not significantly associated with strabismus. On the other hand, Bothun et al. [19] reported that strabismus was less common in infants that underwent surgery before 49 days of age. Binocular vision first appears at the age of 3 months [21]. It is probable that infants with visually significant congenital cataracts that receive prompt treatment within these sensitive periods have a lower risk of developing amblyopia or binocular abnormalities. In the present study, all surgeries were performed before the age of 12 months, but the incidence of post-operative strabismus was still higher than in the general population, in agreement with the literature. Although the incidence of strabismus was lower in the present study’s patients that underwent surgery before the age of 3 months, it did not differ significantly according to age at the time of surgery. However, we feel that statistics failed to find the difference and that the study might be somewhat under-powered. Although not statistically significant, the lower incidence of strabismus in the present study’s patients that underwent surgery before the age of 3 months might have been associated with the fact that binocular reflexes are not yet established at age 3 months, as previously reported [20, 21]. Thus, if surgery is performed before the establishment

Graefes Arch Clin Exp Ophthalmol

of binocular reflexes, patients might be protected from strabismus, maintaining binocularity and orthophoria. Parks and Hiles [22] reported that there was a strong correlation between cataract type and the risk of strabismus; however, there was not a significant association observed between cataract density and strabismus in some other studies [12, 18]. In the present study, cataract type and density were not significantly associated with strabismus. In addition, a gender predilection for strabismus was not noted. Some researchers observed a greater predisposition to strabismus in unilateral cataract cases than in bilateral cases [4, 10, 12, 17, 18]; however, Hiles and Sheridan [3] reported that the occurrence of strabismus was equal in unilateral and bilateral cataract cases. In the present study, there was a significant difference in the occurrence of strabismus according to cataract laterality (85.7 % in unilateral cases and 53.8 % in bilateral cases), as reported in earlier studies. A decrease in or deprivation of visual stimulus in one eye in cases of unilateral cataracts during the critical period of visual maturation before surgery, and the presence of significant anisometropia or aniseikonia, which may result from aphakia correction following cataract extraction, may lead to ocular deviation [17]. Additionally, the high risk of amblyopia due to non-compliance with strict occlusion therapy in unilateral cases can play a role in the subsequent development of sensorial strabismus. France [4] observed that unilateral congenital cataract patients had esotropia or exotropia in about equal proportions; however, in bilateral cases esotropia was dominant. In other studies, esotropia was more common in congenital cataract cases [3, 8, 17, 23, 19]. On the other hand, Weisberg [12] and Park [7] reported that exotropia was more common than esotropia in their cases. In the present study, esotropia was more common in both unilateral (66.7 %) and bilateral (68.6 %) congenital cataract patients. This could be due to increasing convergence mechanisms that develop at an early age [4, 8]. In the present study, age at the time of cataract extraction did not differ significantly between the esotropic and exotropic patients, as reported by Parks [7]. Mean time between surgery and the appearance of strabismus in the present study was 13.3 months (range: 1– 60 months), which is similar to the mean 6–12 months post cataract surgery reported by Spanou [17]. Another study reported that the mean time from cataract extraction and diagnosis of strabismus was 32.5 months in patients with exotropia and 35.6 months in patients with esotropia [7]. In the present study, 70.2 % of the 47 cases of strabismus occurred during the first 12 months post cataract surgery; however, if mean survival time of 66.3 months follow-up had been available for all patients, the estimated cumulative percentage of patients with strabismus would be 66.9 % according to Kaplan–Meier survival analysis. Thus, a careful long-term follow-up is necessary for detecting strabismus post cataract surgery.

Many children treated for dense cataracts also develop nystagmus with or without strabismus [20, 24]. Nystagmus was reported to be more prevalent among children with bilateral cataracts than in children with unilateral cataracts [24–26]. In the present study, the incidence of strabismus was higher in bilateral cataract cases, and the relationship between cataract laterality and the presence of nystagmus was significant. Age at the time of surgery and duration of visual deprivation have also been proposed as risk factors for the development of nystagmus [9, 20, 25, 27]. Birch et al. [28] reported that infantile onset of cataract and visual deprivation >6 weeks were associated with a significantly higher risk of nystagmus, and that the duration of deprivation was an important factor for adverse ocular motor outcomes. Gelbart [29] reported that none of his cataract patients without nystagmus preoperatively developed it postoperatively; however, in the present study nystagmus developed in 26 cases preoperatively, and occurred in an additional 23 patients post-operatively—despite early surgery. Although the amplitude of nystagmus decreased during follow-up in some of the present study’s cases, it never resolved completely. The present study has a number of limitations. First, it was a non-randomized retrospective study, and although none of children had strabismus pre-operatively, it is possible that we might have missed cases with small angles of strabismus preoperatively. Moreover, without binocular tests there is a possibility that cases with small angles of strabismus postoperatively were also missed, which might have led to misdiagnosis of the time of onset, and possibly even misassignment to a group. Secondly, stereoacuity was not assessed in a majority of the patients. Very few patients were able to cooperate sufficiently for binocularity testing at the final follow-up visit; therefore, findings related to binocularity were not obtained. On the other hand, all the patients underwent complete strabismological evaluation at each follow-up visit by two different ophthalmologists, and via this method underestimation of the number of strabismic patients was minimized. Lastly, we did not group the patients according to their characterictics further and did not perform subgroup analysis of cataract type, so it is possible that this study was underpowered to find differences and interactions across the subgroups. In conclusion, management of both monocular and binocular congenital cataracts remains a clinical challenge. The present findings confirm the high frequency of strabismus in congenital cataract patients, even if they undergo surgery at a very young age. The presence of strabismus in these patients represents a significant problem, because it constitutes an additional amblyopia-producing factor that negatively affects postoperative treatment [30]. As strabismus and nystagmus occur more frequently in children undergoing cataract surgery than in the general population, careful long-term follow-up is necessary in these children for monitoring of the development of strabismus.

Graefes Arch Clin Exp Ophthalmol Conflict of interest No conflicting relationship exists for any author and there is no financial support or interest related to the material in the manuscript.

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Strabismus in infants following congenital cataract surgery.

This study aimed to determine the incidence and characteristics of strabismus following congenital cataract surgery in infants...
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