Tectonic Corneal Transplant in the Management of Congenital Anterior Staphyloma

To describe management and outcomes in cases of severe congenital anterior staphyloma (CAS).

We reviewed the medical records of patients treated for CAS at the Children's Hospital of UPMC, Pittsburgh, between January 2022 and December 2023. Our analysis included demographic data, clinical observations, genetic testing results, histopathology analysis, electrophysiology testing, details of surgical procedures, and outcomes. B-scan ultrasound, ultrasound biomicroscopy, and anterior segment optical coherence tomography studies were reviewed.

Eight patients, including 2 with bilateral disease, underwent penetrating keratoplasty (PKP). The average age at presentation was 13.75 ± 13.06 weeks (median 10.50 wk, range 1-44 wk), and patients were followed for 423.13 ± 166.19 days (median 411.50 d, range 166-658 d). The average age at the time of PKP was 30.10 ± 13.23 weeks (median 27.21 wk, range 10-53 wk). Post-PKP complications occurred in 9 eyes, including increased intraocular pressure in 7 eyes, persistent graft epithelial defects in 4 eyes, cyclitic retro-corneal membranes in 3 eyes, and graft stromal neovascularization, bacterial keratitis, tractional retinal detachment, and corneal perforation in 1 eye each. At the end of follow-up period, 4 eyes had clear grafts, 3 eyes displayed partially opacified grafts, and 3 presented with opacified grafts. All patients achieved satisfactory intraocular pressure control. No signs of sympathetic ophthalmia were observed in the unaffected eye.

Tectonic PKP can be considered an alternative to infant enucleation or evisceration when counseling parents of infants with CAS. Anterior segment optical coherence tomography findings in these cases confirm histological corneal findings in our series.