Pediatric Corneal Transplantation

Increased understanding of the special problems associated with pediatric grafts, advances in preoperative diagnostic techniques and in surgical methods, and improved postoperative management have enhanced the visual prognosis for children who have undergone corneal transplantation. Further, improvements in pediatric anesthesia and the recognition that development of amblyopia is a major impediment to useful vision have led to earlier surgical intervention. The success rate of pediatric corneal transplantation depends on the extent of coexisting ocular abnormalities. For example, one of the most common indications for pediatric keratoplasty is Peters anomaly. For type I disease, in which there is a central corneal opacity and a normal anterior segment, the survival rate for a clear graft was 83%–90% in 1 large series (mean follow-up time of 78 months), depending on the age of the patient at the time of surgery. By contrast, in another large series of patients with either type I or type II Peters anomaly, the outcomes were significantly worse: only 56% of grafts remained clear at 6 months; 44%, at 3 years. This was attributed to the inclusion of patients with type II disease, in which the anterior segment findings are more severe and characteristically include adhesions among the cornea, iris, and lens; corneal neovascularization; glaucoma; cataract; and corneal staphyloma. Therefore, more extensive surgery was required, and not surprisingly, the survival rate of the graft decreased.

Critical to a successful outcome is the family’s (or caregiver’s) dedication to following a rigorous postoperative regimen, which includes repeated examinations under anesthesia and adherence to the medication regimen. Postoperative glaucoma, strabismus, self-induced trauma, and immune rejection are common. As part of obtaining informed consent from the family, the physician must discuss the many difficult issues associated with the surgery, including the complicated postoperative course, loss of time from work (with associated loss of income), the extensive ongoing care required for the child, disruption of home life, and less time to devote to other dependents.

Corneal grafting in children younger than 2 years is associated with rapid neovascularization, especially along the sutures. As the wound heals, erosions may occur along the sutures, leading to eye rubbing, epithelial defects, mucus accumulation, and possible infection. Suture erosion has been reported to occur as early as 2 weeks postoperatively in infants and necessitates urgent and frequent examination under anesthesia in the operating room to evaluate the transplant and remove sutures until all have been removed. In addition, early fitting with a contact lens (as early as the time of PK) and ocular occlusive therapy are necessary to stem development of amblyopia in children with monocular aphakia.

Surgeons have become aware of DALK as an option for certain pediatric patients with stromal scarring without any other corneal pathology. For disease that is primarily endothelial, such as congenital hereditary endothelial dystrophy, EK has been reported to provide good outcomes, as observed in a small series of 8 patients (15 eyes).

Busin M, Beltz J, Scorcia V. Descemet-stripping automated endothelial keratoplasty for congenital hereditary endothelial dystrophy. Arch Ophthalmol. 2011;129(9):1140–1146.

Nischal KK. Pediatric keratoplasty. In: Mannis MJ, Holland EJ, eds. Cornea. Vol 2. 4th ed. Philadelphia: Elsevier; 2017:1382–1398.

Rao KV, Fernandes M, Gangopadhyay N, Vemuganti GK, Krishnaiah S, Sangwan VS. Outcome of penetrating keratoplasty for Peters anomaly. Cornea. 2008;27(7):749–753.

Zaidman GW, Flanagan JK, Furey CC. Long-term visual prognosis in children after corneal transplant surgery for Peters anomaly type I. Am J Ophthalmol. 2007;144(1):104–108.

Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.