Early-onset cataracts in pediatric patients: indicators, surgical intervention, and challenges
Muralidhar Ramappa, MD, spoke with Ophthalmology 360 at the 2024 AAO Annual Meeting about a session he was part of, titled “Early-Onset Cataracts in Pediatric Patients: Indicators, Surgical Intervention, and Challenges.”
Muralidhar Ramappa, MD:
The pediatric cataract surgery is quite different than adult surgery. Not only these eyes are much more smaller and have a limited space to work on, these children tend to have a much higher postoperative complication in comparison with the adults. Also, at the time of surgery, each of this child have a long life expectancy, unlike an adult. Not only these eyes are very small, a cornea and sclera elastic, that in turn poses a significant intraoperative challenges to maneuverability. They tend to bleed. They tend to have a more profound anterior chamber inflammation. They are also prone for having postoperative posterior capsular opacification and higher risk of developing a glaucoma. Keeping all this in mind, we have to modify surgical intervention. In these cases, it has to be tailor-based case to case.
It’s important to define whether we are dealing with a monocular or a binocular case. In monocular, most often they have an anterior segment developmental abnormality. Meaning, along with the cataract, there can be a structural abnormality in the eye that can pose a different challenge.
In case of bilateral, where generally, they may have either systemic causes or genetic causes, in a majority, nearly 50% of them may not have an identifiable cause for a bilateral cataract surgery. In certain cases, it could be metabolic conditions; it could be inflammatory, it could be, at certain times, infections. Or even including a fetal alcohol syndrome, particularly, in the US. It can be one of the significant risk factor for a cataract formation in the children.
Me and my colleague, with a video-based presentation, we will be highlighting some of the medical and the surgical pearls, how to deal with these challenging cases. For instance, I would be handling a cataract surgery, particularly in anterior segment developmental anomalies. Apart from a cataract, here, the front part of the cornea, uvea, and including the retina, may have an abnormality.
It is so important to diagnose them very accurately, and have the correct diagnosis and a correct approach in a critical period. Each of this children have a critical period; whether we are dealing with a monocular or binocular, we need to catch them and address, not only keeping their visual access clear, we have to give a correct postoperative refractive correction. Also prompt amblyopia therapy, particularly during a neuroplasticity, in order to maximize their success.
As I mentioned, the child’s eye is quite different, both anatomically and physiologically, than of an adult. As I said, these sizes are not only miniature, they’re highly elastic, and they tend to collapse interoperatively. Since you have less room for a maneuverability, you tend to have injuries to the cornea, lens, injury to the uveal tissue. That can sometimes bleed profusely interoperatively and can produce a significant pigment dispersion, inflammation. Also, the vitreous is highly tenacious. Unlike adults, we may have to do a membranectomy and also anterior vitrectomy. The vitreous is tend to be much more tenacious. This has to be very meticulously planned, in order to prevent the immediate postoperative to posterior capsular opacification.
Also, the fact that these cases have a higher tendency to develop those secondary glaucoma and refractive surprises. Because this is a growing eye, sometimes we may have issues in ascertaining the exact intraocular lens power, therefore end up having either myopic or hyperopic shape. Even that has to be kept in a mind.
As I said, when it comes to bilateral, nearly 50% of them have a genetic basis. Another 50% could be idiopathic. They can also be metabolic syndromes, like a galactosemia.
The toxoplasma, rubella, cytomegalovirus, herpes, syphilis, all can cause a intrauterine growth retardation, along with the cataract, and a significant systemic anomalies. Whereas in a monocular condition, meaning children with a unilateral cataract, they generally have a structural abnormality. The other is normal. An eye which is having a cataract, can have a varying degree of anatomical abnormality. One of the most common would be persistent fetal vasculature. That’s one of the most common causes for unilateral cataract formation. Along with persistent fetal vasculature, they can have a microcornea, microphthalmia. One has to really pick up this in a very early on.
For a uniocular condition, we tend to prefer a surgery. Surgical timing is very, very crucial. Between 6 to 8 weeks for a monocular case. And a binocular, between 8 to 12 weeks is the time. This is a critical period. That actually maximize our functional success. Therefore, it helps us containing the amblyopia.
As I said, pediatric cataract surgery, it’s right from diagnosis. We need to ascertain the root cause for the cataract. Is it a binocular or a monocular? Is it a congenital or a developmental? Does it have any, apart from ophthalmic, does the baby have any systemic association or comorbidities? It is paramount, important to detect them.
We need to preempt. There are certain complications, which is maybe case-wise, and that has to be tackled carefully in a timely manner. Also refining, making modifications in the surgical approaches. It is, again, based on a case to case. Not only keeping the visual access clear and giving a timely refractive correction, and amblyopia is mandatory.
These children needs closed monitoring, in order to pick up some of the complication; it could be PCO, posterior capsular opacification, membrane formations. It could be secondary glaucoma. These are very, very important in the postoperative period.
Also to be able to assess how the child is progressing in terms of glass acceptance, patching, when it is applicable. Periodically, one has to keep changing the glasses until the right age. This is very important, and the commitment is for the lifetime. These children can have complications much later. As I said, apart from a PCO, secondary glaucoma, retinal detachment, and macular abnormality or macular complications, can be seen years later.
Bilateral, 50% of the cases can have it. That is a hereditary cause. Meaning, particularly in developing nations like India, where we tend to have a inbreeding, they tend to marry within a close relationship. Therefore, they end up having some of the autosomal dominant or a recessive condition that can contribute significantly for a cataract. The other 50%, it could be infections, it could be metabolic conditions. A certain miscellaneous also very rare conditions can also manifest bilateral cataracts in children.
