Anterior segment dysgenesis in a child with factor VII deficiency

Abstract

Purpose:

To describe the first case of anterior segment dysgenesis associated with factor VII congenital deficit (hypoproconvertinemia).

Method:

A 2-month-old child with factor VII deficiency was referred to our clinic because of corneal opacities. The child was born to consanguineous parents and severe factor VII deficiency was diagnosed on the third day of life because of gastrointestinal bleeding.

Result:

Ocular examination under anesthesia showed bilateral corneal opacities with adherent iris strands and peripheral anterior synechiae. The intra-ocular pressure was normal in both eyes and there were neither signs of cataracts nor glaucomatous optic nerve damage.

Conclusion:

This observation provide new information on the possible ocular findings in patients with hypoproconvertinemia. Based on this report, we suggest that careful coagulation screening should be considered in children who possess idiopathic anterior segment dysgenesis aiming at identifying the possible coagulation disorder.

Keywords:

• anterior segment dysgenesis
• factor VII deficit
• hypoproconvertinemia
• corneal opacity
• intracranial hemorrhage

Disclosure

Research grants: none.

Financial support: none.

Competing interests: the authors declare that they have no competing interests.