Alternatives to Penetrating Keratoplasty

It is important to consider alternatives to penetrating keratoplasty, particular those techniques that are less invasive but nevertheless effective [5, 8, 12, 20, 27]. For example, in some infants with a clear peripheral cornea and crystalline lens, an optical iridectomy may result in an acceptable visual outcome [46]. This is an effective surgical intervention, particularly if follow-up is likely to be difficult or unreliable, and may be the safer option in some cases [25, 29, 46].

Rotational autografts may be suitable in selected cases where a corneal opacity involving the pupillary area is rotated to leave a clear central visual axis [2, 31, 39].

In adults, lamellar techniques are now preferred over penetrating keratoplasty to selectively replace diseased tissue, thereby leaving healthy tissue untouched. For instance, endothelial keratoplasty is currently the preferred surgical technique for isolated endothelial disease such as Fuchs’ endothelial keratoplasty and pseudophakic bullous keratopathy. The advantages of Descemet stripping endothelial keratoplasty (DSEK) over PK are numerous and include faster visual rehabilitation, less induced refractive error, a tectonically stronger eye, and less suture-related complications [12]. There have been a few case reports of successful DSEK in children with CHED [20, 27].

DSEK surgery is also technically more challenging in the pediatric age group due to their smaller anterior chamber dimensions and risk of cataract formation if iatrogenic damage to the crystalline lens occurs [12]. Furthermore, there is more difficult postoperative management in children secondary to compliance issues with posturing, etc. Nevertheless, DSEK may be an option for a selected group of patients, that is, those children with isolated endothelial disease [20, 27].

Similarly, deep anterior lamellar keratoplasty (DALK) may be preferred to PK in children with isolated stromal pathology, e.g., corneal scarring secondary to infectious keratitis, mucopolysaccharides, central dermoids, and keratoconus [23]. DALK is technically more challenging but is advantageous as it avoids an “open sky” procedure and essentially eliminates the risk of lens extrusion or suprachoroidal hemorrhage. There are different surgical techniques of performing DALK and include manual deep lamellar dissection, “big-bubble,” and viscodissection.

The Boston keratoprosthesis has been performed in pediatric patients as an alternative to penetrating keratoplasty. The advantages include rapid visual rehabilitation, elimination of allograft rejection, and earlier initiation of amblyopia therapy [5]. It may have a role in children with poor prognosis for PK such as multiple allograft failures secondary to immune rejection [5, 8, 12]. However, disadvantages include the need for a bandage contact lens, difficulty in monitoring intraocular pressure as well as the indefinite use of antibiotics [32]. Complications include retroprosthetic membrane, stromal thinning or melting, implant extrusion, sterile vitritis, and infectious endophthalmitis [32]. The longterm complications are of particular concern as these patients will have their prosthesis for a very long duration.

Summary for the Clinician

•An optical iridectomy is minimally invasive and may result in a good visual outcome in selected cases.

•Lamellar surgery including DSEK and DALK as well as the Boston keratoprosthesis has been performed successfully in pediatric patients.

Conclusion

Infant keratoplasty is challenging in many ways. Careful patient selection, meticulous preoperative planning and surgical technique, early refractive correction, and amblyopia therapy as well as regular follow-up are necessary to achieve best visual outcomes. This requires a team approach with close collaboration between the corneal surgeon, pediatric ophthalmologist, support staff as well as family members. The impact of achieving functional vision for children with severe corneal opacity cannot be overemphasized. It is associated with significant improvements in behavior, communication, and ambulation, as well as having tremendous benefits for the family members.

Conflict of Interest None of the authors have any financial/conflicting interests to disclose.

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