Role of scleral tectonic graft in saving the vision of a child with apert syndrome

Abstract

Apert syndrome (AP) is a rare form of acrocehpalosyndactyly with autosomal-dominant transmission. The most common ocular features of patients with AP include proptosis, shallow orbits, and hypertelorism; they can have exotropia and antimongoloid slant of the palpebral fissures. The aim of this report was to study the role of scleral tectonic patch graft (STPG) in managing the corneal exposure due to AP. This was the clinical case report of a 9-year-old girl with AP, presented with right eye corneal perforation. She was treated with homologous STPG instead of tectonic corneal graft with good improvement of her visual acuity (VA). The outcome of the patient VA after 1 year was the improvement of the vision in the affected eye from the hand movement to 6/12, which was considered very satisfactory to the child and her family. This case report revealed that tectonic scleral patch graft is a sight-saving procedure in condition where the scleral tissue is readily available rather than the corneal tissue that needs the presence of cornel banks.