Pediatric Keratoplasty

doi:10.1016/j.survophthal.2008.12.011

Survey of Ophthalmology

Volume 54, Issue 2, March–April 2009, Pages 245-271

https://doi.org/10.1016/j.survophthal.2008.12.011 Get rights and content

Abstract

Penetrating keratoplasty in children is a highly challenging and demanding procedure associated with a high risk of graft failure or failure of amblyopia therapy in clear grafts. Nonetheless, keratoplasty remains the surgery of choice for the management of pediatric corneal stromal opacities or edema. Allograft rejection, graft infection, corneal neovascularization, glaucoma, trauma to the anterior segment, vitreous pathology, and additional surgical interventions, especially those related to glaucoma management, are important risk factors. Successful penetrating keratoplasty in children requires careful preoperative evaluation and selection of patients follow-up by well-motivated parents, an expert corneal transplant surgeon, and a devoted pediatric ophthalmologist.

Introduction

Congenital eye disorders, although infrequent, are important causes of childhood blindness. Visual deprivation due to corneal opacification can lead to long-term changes in the central nervous system.¹⁶¹ In order to achieve optimal visual results and avoid visual-deprivation amblyopia, corneal transplantation must be performed in the early months of life. Penetrating keratoplasty in children has a higher rate of graft failure and a poorer visual prognosis than adult keratoplasty. Improved understanding of intraoperative and postoperative problems has resulted in more successful pediatric corneal grafts.

Pediatric keratoplasty was performed infrequently prior to the mid 1970s¹⁸ and was recommended only in pediatric patients with bilateral corneal involvement.¹⁵⁹ Technical advances, however, have now lowered the age at which keratoplasty is performed and indications have increased with improvement in surgical techniques and therapies.

Special problems in corneal transplantation in children include difficult preoperative evaluation; intraoperative problems such as low scleral rigidity, increased fibrin reaction, and positive vitreous pressure; the need for frequent examinations under anesthesia for postoperative follow-up evaluations; frequent loosening of sutures necessitating replacement/early removal; increased risk of rejection and infections; and the difficulties with repeated refractive error assessments, and reversal of amblyopia. Even with increased anatomic success of pediatric corneal grafts, visual rehabilitation remains a concern.

In developing nations an increasing number of grafts are performed for infectious keratitis, post-infectious keratitis corneo-iridic scars, or keratomalacia. Measures to maintain clear graft and successful visual rehabilitation following keratoplastyare required to achieve a successful anatomical and functional outcome. This review of corneal grafting in infants and children evaluates the various indications, as well as factors affecting graft prognosis, technique, special problems, and outcome of pediatric keratoplasty.

Section snippets

Indications

Pediatric corneal opacities have been classified into three categories:133, 134 congenital, traumatic, and acquired non-traumatic. Indications for pediatric corneal transplantation (Table 1) vary widely; the proportion of keratoplasties performed for congenital indications range from 14–64%, for acquired non-traumatic conditions they range from 19–80%, and for acquired traumatic conditions they range from 6–29%.1, 31, 32, 33, 34, 95, 134 Al-Ghamdi et al⁵ propose a newer classification that

Technique

Pediatric keratoplasty was considered previously to be contraindicated because of its technical challenges due to a low scleral rigidity and forward displacement of lens–iris diaphragm.13, 47, 103, 115 Although pediatric keratoplasty is now considered to be a safe and effective procedure, specific problems do exist in the management of children who undergo corneal transplantation.

Postoperative Management

Postoperative management of pediatric corneal grafts demands dedicated follow-up evaluations under anesthesia, monitoring of postoperative medications for frequency alterations, appropriate management of sutures, close watch for rejection, frequent correction of refractive errors, initiation of amblyopia therapy, and ensuring compliance to long-term amblyopia therapy. Hence, the need to emphasize on the biphasic approach of 1) maintaining a clear graft, and 2) reversing amblyopia, is of

Complications

Acquired corneal scars, later corneal decompensation in older children, and phakic eyes have the best prognosis. Corneal perforations, active inflammation or infection, and infants with multiple ocular anomalies have the poorest prognosis. Children undergoing combined procedures have been found to have a less favorable result than those undergoing a single- or two-staged procedure.³¹ Complications such as cataract development, secondary glaucoma, epithelial defects, band keratopathy, retinal

Outcome of Pediatric Keratoplasty

Comparison of graft survival outcomes among the reported studies is difficult due to the heterogeneity of the involved conditions, varying size of the study group, and varying periods of follow-up (Table 2).

Poor results in corneal grafting in congenital, central corneal opacities prompts surgeons to avoid penetrating keratoplasty in patients with unilateral, congenital corneal opacities.61, 96, 126, 134, 159, 164 Irreversible amblyopia, glaucoma, other structural abnormalities of the anterior

Conclusion

Early penetrating keratoplasty in infants with congenital corneal opacities is required to prevent amblyopia and allow normal development of vision. In the past corneal grafting in children for congenital corneal opacities was delayed until early childhood because of the difficulty in operating upon infant eyes. Recent studies stress on the need for early grafting in order to prevent amblyopia. Technical advancements have made corneal grafting possible at a younger age group. Clear graft rates

Method of Literature Search

The PubMed database was searched electronically for the years 1950–2007 with the keywords penetrating keratoplasty in children, pediatric keratoplasty, corneal transplantation.

Manual cross reference search: Some articles that were not found by our Medline search were taken from the references from other articles and books. English abstracts available on PubMed were included in certain important non-English language articles on pediatric penetrating keratoplasty. Inclusion of the articles was

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