Current management of infantile cataracts

Abstract

Infantile cataracts remain one of the most treatable causes of lifelong visual impairment. While the chance of improving vision for children with infantile cataracts has never been better, significant global and socioeconomic disparities still exist in their early management. Recent epidemiological studies reveal a stable prevalence of infantile cataracts in high-income countries and highlight challenges in determining the prevalence of infantile cataracts in low-income countries. Detailed descriptions of cataract morphology may inform us as to etiology, provide guidance with regards to surgical approach, and have prognostic value. Molecular genetics is providing new insights into the hereditary bases and potential systemic associations of infantile cataracts. For visually significant infantile cataracts requiring surgery to clear the visual axis, surgical techniques continue to evolve based on the experiences and research efforts of skilled teams worldwide. The most common complications of cataract surgery performed in infancy are visual axis opacification and, in about a third of patients, the long-term development of glaucoma. Children with unilateral cataracts generally see well given the presence of a healthy fellow eye. Better visual outcomes in operated eyes, however, are achieved in the setting of early presentation, bilateral infantile cataracts, absence of nystagmus or strabismus, and consistent amblyopia therapy. While intraocular lenses for infants less than 6 months can result in good visual outcomes, contact lenses may be preferred in situations in which they are available and practical. Many studies have demonstrated the benefits of early surgery for infantile cataract. We must strive for the continued evolution of technologies and strategies that have the potential to further improve these outcomes.

Keywords: Infantile cataract; congenital cataract.

Fig. 1 –

Anterior polar cataract.

Fig. 2 –

Fetal nuclear congenital cataracts in 2 patients.

Fig. 3 –

Persistent fetal vasculature.

Fig. 4 –

Longitudinal view on preoperative B-scan ultrasound reveals persistence of hyaloid stalk.

Fig. 5 –

Posterior plaque of persistent fetal vasculature with elongated ciliary processes.

Fig. 6 –

Microphthalmos of the left eye in a patient with persistent fetal vasculature of the left eye.

Fig. 7 –

Posterior lentiglobus.

Fig. 8 –

Congenital cataracts in the right (A) and left and (B) eyes of a 4- week old boy. There was no family history of congenital cataracts. His cataracts were membranous at the time of cataract surgery and Lowe syndrome was suspected. However, genetic testing revealed a pathogenic mutation (c.97C>T) in the first exon of the major intrinsic protein of the ocular lens fiber membrane (MIP) gene which has been shown to be associated with autosomal dominant congenital cataracts and a normal OCRL gene. The bottom left and right photos show similar looking congenital cataracts in the right eye (C) and left eye (D) of an infant with Lowe syndrome.

Fig. 9 –

(A) Large, pre-existing posterior capsular defect in patient with posterior polar type cataract. (B) Care taken to avoid excessive enlargement of defect during lens removal can permit placement of an intraocular lens in the capsular bag.

Fig. 10 –

Different outcomes resulting from variations in postoperative management (A) Sixteen-year old diagnosed with a nuclear cataract in her left eye as an infant. She underwent a left lensectomy at age 6 weeks and was fit with a silicone elastomer contact lens postoperatively. Her right eye was patched part-time until age 7 years (1 hour/day/per month of life until age 8 months; 6 hours/day until age 13 months; 5 hours/day until age 2 ½ years; 4 hours/day until age 3 years; 2 hours/day until age 4 years; 4 hours/every other day until age 5 years; 4–5 hours/day twice a week until age 7 years). At 7 years of age, patching was discontinued. Her best-corrected visual acuity was 20/20 in both eyes wearing a +18 D contact lens on her aphakic left eye. She was orthotropic and had 40 s/arc of stereopsis. (B) Nine-year old diagnosed with a nuclear cataract in his left eye as an infant. He underwent a left lensectomy at age 4 weeks. Postoperatively he wore a silicone elastomer contact lens on his aphakic left eye. His right eye was patched for all but 2 of his waking hours until age 4 years when patching was tapered and then stopped at age 7 years. His visual acuity was 20/20 in both eyes. However, he remained esotropic after one strabismus surgery and he had no stereopsis. (C) Thirty-five year old woman diagnosed with a congenital cataract in her left eye as an infant. Cataract surgery was deferred until she was age 3 years and no optical correction was prescribed for her aphakia postoperatively. Her best-corrected visual acuity was 20/20 in her right eye and counting fingers in her left eye. She has undergone 4 strabismus surgeries on her left eye.