05 October 2021
3 minutes to read
Pediatric Keratoconus is often an undiagnosed condition that in many cases results in untreated.
As this patient population has a vulnerable developing visual system, delayed treatment can have lifelong visual consequences.
With the rapid rate of progression found in keratoconus in children compared to keratoconus in adults, initiation of treatment is imperative. Management options have changed over the past decade with the success of corneal collagen crosslinking (CXL) and, therefore, it is important to understand the role of each treatment option available for our children with keratoconus.
Contact lenses are a non-surgical option for pediatric keratoconus, with standard gas permeable (GP) lenses and scleral lenses offering great visual success in children. Although GP contact lenses can provide visual assistance to our pediatric patients, they are not on their own considered a long-term treatment option.
This conservative modality does not stop disease progression but may provide long term visual benefit when used in conjunction with CXL.
Data in the pediatric population are limited, but the available cases show that intracorneal ring segment implantations (ICRS) are well tolerated with an adequate visual outcome at baseline (Olivo-Payne et al.). Although this appears to be promising information, ICRS implantation is not a commonly used treatment in pediatric patients due to the aggressive progressive nature of pediatric keratoconus, associated frequent eye rubbing, and unpredictable results (Venugopal et al.). These results make ICRS a short-term solution and not a viable long-term treatment modality.
Studies evaluating the combination of ICRS implantation with CXL show stable topographic results and good visual results. With over 20% of keratoconus patients intolerant to contact lenses (Olivo-Payne et al.), This dual treatment modality may be an option to consider in the future for a specific group of patients. Since the data is minimal, more studies are needed to understand long-term success in children.
Overall, ICRS alone does not stop the progression of keratoconus and should not be considered the standard of care for pediatric keratoconus.
CXL is a technique that uses ultraviolet A (UV-A) light and riboflavin to increase the biomechanical stiffness of the cornea and stop the progression of ectasia in patients with keratoconus. The original protocol removes the epithelium (epi-off) before the application of riboflavin and UV-A light. Long-term topographic stabilization has been well documented in the pediatric patient population, along with noted visual improvement (Caporossi et al.). Currently, the Glaukos iLink procedure is the only CXL system approved by the FDA in the United States.
Another CXL method explored abstaining from epithelial removal (epi-on) prior to application of riboflavin and UV-A light. This method has been studied to potentially reduce postoperative pain and infection. Unfortunately, the current CXL epi-on technique does not allow adequate penetration of riboflavin, resulting in decreased uptake of UV-A in the stroma. Therefore, it does not provide long-term stability for pediatric keratoconus (Venugopal et al.).
Researchers are studying adjustments to riboflavin, UV irradiation, and oxygen uptake, which may make the modified epi-on procedure a viable option for our pediatric patients in the future.
Corneal transplants for keratoconus in the pediatric population represent 15-20% of all corneal transplants (Olivo-Payne et al.). These procedures carry a multitude of perioperative, intraoperative and postoperative risks. Additionally, a younger age at the time of transplantation has been associated with a higher risk of rejection, leading to an increased need for multiple transplants and a poor visual prognosis (Venugopal et al.).
While this is a possibility, and sometimes the only option, for advanced stage keratoconus, it certainly comes with high risks and does not guarantee a successful visual outcome.
In summary, once the diagnosis of keratoconus is made, it becomes essential to stop the progression of this condition to avoid a deterioration of the long-standing visual result of these children. While the management options for these children can be overwhelming, CXL is the only treatment for pediatric keratoconus that has been shown to halt disease progression and prevent prolonged reduction in visual acuity. a child.
- Caporossi A, et al. J Ophthalmol. 2011; 2011: 608041. doi.org/10.1155/2011/60804.
- Olivo-Payne A, et al. Clin Ophthalmol. 2019; 13: 1183-1191.
- Venugopal A, et al. Indian Ophthalmol J. February 2021; 69 (2): 214-225.