View abstract

Cet abstract a été assigné à session OB Poster session > in O'Bistro

Titre

Congenital Corneal Staphyloma: Dramatic Ophthalmological Findings in a Newborn

But	To describe the clinical spectrum of congenital corneal staphyloma.
Méthodes	A complete ophthalmological examination with additional B-scan ultrasonography of both eyes. Evisceration of the right eye was performed on the day of birth. We referred the patient for a general pediatric investigation with ultrasound of the abdomen and brain. A blood sample was taken for mutation analysis of the PITX2, FOXC1 and the beta1,3-glucosyltransferase genes.
Résultats	A newborn girl was referred with an amorphous remnant of an intra-uterine perforated right eye and a buphalmic left eye with opacified cornea. Ultrasound of the left eye revealed a deep anterior chamber with adhesions of the peripheral iris to the posterior segment of the cornea. Histopathological examination of the remnants of the right eye revealed the characteristics of a congenital corneal staphyloma. No mutation was identified in the PITX2, FOXC1 and beta1,3-glucosyltransferase genes. Systemic examination was normal except for a hypoplastic right kidney with normal corticomedullary differentiation. At the age of 10 months a large diameter keratolimbal graft was performed on the left eye. Systemic immunosuppressive therapy was started to prevent a graft failure, but after three months phtysis was inevitable.
Conclusion	This case represents a bilateral congenital corneal staphyloma, confirmed by histopathological examination of both corneas. Management of these infants is challenging with poor visual outcome in nearly all cases.

Auteur 1

Nom	VERSCHOOTEN
Initiales	R
Institut	Department of Ophthalmology
Ville	Leuven

Auteur 2

Nom	Foets
Initiales	B
Institut	Department of Ophthalmology
Ville	Leuven

Auteur 3

Nom	de Ravel
Initiales	T
Institut	Human Genetics
Ville	Leuven

Auteur 4

Nom	Van Ginderdeuren
Initiales	R
Institut	Department of Ophthalmology
Ville	Leuven

Auteur 5

Nom	Lombaerts
Initiales	R
Institut	Pediatrics
Ville	Leuven

Auteur 6

Nom	Casteels
Initiales	I
Institut	Department of Ophthalmology
Ville	Leuven