Unusual Presentation Of Peter's Anomaly As A Sclerocorneal Tumour: Case Report.

 Our aim is to describe a new manifestation of Peter’s anomaly and its therapeutic management.

Peter’s anomaly is a rare form of Anterior Segment Dysgenesis characterized by corneal opacity, a variable degree of involvement of anterior chamber structures and defects in the posterior layers of the cornea due to failure of neural crest cell migration during embryogenesis. The etiology is not entirely known and does not explain the heterogeneity of existing cases.

This was a case report

A 5-month-old male infant with congenital corneal opacity in both eyes developed, a few weeks after birth, a rapid and progressive tumour-like growth from the sclerocorneal surface in his left eye. An ocular B-mode ultrasound and biopsy of the resected tumour were performed. Histological findings compatible with Peter’s anomaly were evidenced in the tumour mass. Penetrating keratoplasty was performed in the left eye, treated with topical steroids and hypotensive drugs. The patient presented ocular hypertension after keratoplasty, so he underwent transscleral cyclophotocoagulation with diode laser, which reduced the ocular tension figures to normal. An  peripheral optic iridectomy was performed in the fellow eye, obtaining good fixation.

This novel and peculiar presentation of Peter's anomaly as a sclerocorneal tumour not previously described in the literature, suggests that this is an atypical and severe form of this pathology. Despite the complexity of his anomaly, removal of the tumour and penetrating keratoplasty restored the aesthetic appearance of his left eyeball. Peripheral optic iridectomy in the fellow eye provided a clear visual axis by improving his early visual stimulation.