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Anterior segment dysgenesis in a child with factor VII deficiency

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Authors: Francis Beby, Sandrine Meunier, Pierre Cochat, Olivier Roche, Florent Aptel, et al

Published Date December 2007 Volume 2007:1(3) Pages 335 - 337
DOI: http://dx.doi.org/10.2147/OPTH.S

Francis Beby1, Sandrine Meunier2, Pierre Cochat3, Olivier Roche4, Florent Aptel1, Carole Burillon1, Philippe Denis1

1Department of Ophthalmology, Edouard Herriot Hospital, Lyon, France; 2Haemophilia Care Center, Department of Hemostasis, Edouard Herriot Hospital, Lyon, France; 3Department of Pediatrics, Edouard Herriot Hospital, Lyon, France; 4Department of Ophthalmology, Necker-Enfants Malades Hospital, Paris, France

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








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