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This abstract is assigned to session OB Poster session > in O'Bistro

Title

Congenital Corneal Staphyloma: Dramatic Ophthalmological Findings in a Newborn

Purpose	To describe the clinical spectrum of congenital corneal staphyloma.
Methods	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.
Results	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.

Author 1

Last name	VERSCHOOTEN
Initials	R
Department	Department of Ophthalmology
City	Leuven

Author 2

Last name	Foets
Initials	B
Department	Department of Ophthalmology
City	Leuven

Author 3

Last name	de Ravel
Initials	T
Department	Human Genetics
City	Leuven

Author 4

Last name	Van Ginderdeuren
Initials	R
Department	Department of Ophthalmology
City	Leuven

Author 5

Last name	Lombaerts
Initials	R
Department	Pediatrics
City	Leuven

Author 6

Last name	Casteels
Initials	I
Department	Department of Ophthalmology
City	Leuven