A 19-year-old male with congenital hereditary endothelial dystrophy (CHED) presented with severe bilateral corneal clouding precluding any view of the intraocular structures. He underwent modified Descemet's stripping automated endothelial keratoplasty (DSAEK) technique including a suture pull-through technique to prevent lens damage. Surgery resulted in progressive clearing of the cornea and d...

CASE REPORT A 31-year-old woman who had undergone surgery for a congenital cataract as a newborn developed bilateral malignant glaucoma, which was refractory to medical-surgical treatment. The patient currently has terminal glaucoma and severe band keratopathy. For aesthetic purposes, we performed intrastromal keratopigmentation surgery using ink for skin tattoos supplied in sterile single-dose...

BACKGROUND Sclerocornea, a congenital corneal pathology characterized by bilateral scleralization of the cornea, which can be found in few cases with posterior fossa malformationshemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe (PHACES) syndrome. Presence of vascularization in peripheral cornea and smaller diameter of recipient cornea corre...

PURPOSE To localize a gene causing a newly described autosomal dominant anterior segment dysgenesis characterized by corneal endothelial dystrophy, iris hypoplasia, congenital cataracts, and corneal stromal thinning (EDICT syndrome). DESIGN Experimental study. METHODS A set of microsatellite markers spanning the 22 human autosomes was used to perform linkage analysis on affected and unaffec...

OBJECTIVE It is to study the pattern of corneal diseases (including injuries) in paediatric population in a tertiary eye care center. MATERIALS AND METHODS A descriptive, cross sectional study was carried out in a tertiary eye care center which included 176 cases. RESULTS Out of 176 cases studied, 6 cases (3.4%) presented with congenital causes, 4 cases (2.27%) were of dystrophy and ectasia...

CASE REPORT A 33-year-old woman with superficial and deep bilateral corneal vascularization and keratoconjunctivitis sicca, keratoerythema and neurosensory deafness, was diagnosed with keratitis-ichthyosis-deafness (KID) syndrome. DISCUSSION KID syndrome is a congenital ectodermal dysplasia characterized by the association of vascularizing keratitis, hyperkeratotic skin lesions and sensorineu...

Norrie's disease, a congenital progressive oculo-acoustico-cerebral degenerative condition, is a sex linked recessive disorder. Previously described as atrophia oculi congeneti, it is associated with bilateral pseudotumour of the retina, lens, and corneal opacities, and phthisis bulbi. Some patients develop progressive deterioration of mental function and hearing. We report a Sri Lankan family ...