A method to preserve limbus during penetrating keratoplasty for a case of presumed PHACES syndrome with sclerocornea

BACKGROUND Sclerocornea, a congenital corneal pathology characterized by bilateral scleralization of the cornea, which can be found in few cases with posterior fossa malformationshemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe (PHACES) syndrome. Presence of vascularization in peripheral cornea and smaller diameter of recipient cornea correlate to poor outcome of penetrating keratoplasty (PKP) in sclerocornea. Here we report a method to preserve limbus during PKP for small, irregular, and scleralized cornea. METHODS A 12-year-old boy with multiple congenital anomalies diagnosed as PHACES syndrome suffered from bilateral total sclerocornea and poor visual acuity. Due to the fact that the left eye cornea was small (6.5 mm × 10 mm), lamellar dissection and posterior recession of inferior limbus was first performed and followed by a 6 mm trephination and PKP with a 6.5 mm graft for left eye. At the same time, lens aspiration and release of peripheral anterior synechia were performed. RESULTS After 6 years of follow-up, the cornea remained clear, and there has been no sign of inflammation and conjunctivalization. The patient maintained useful vision of 20/400 in left eye. CONCLUSION The stabilization of corneal surface is possible after PKP for sclerocornea if the limbus can be preserved during the operation, and epithelium can remain corneal in phenotype preventing pannas growth.