Congenital Corneal Opacity/ Corneal Clouding causes – STUMPED
The mnemonic STUMPED is a good aid to remember the differential diagnosis of a neonate with a cloudy cornea.
- Tears in Descemets (trauma)
- Peter’s anomaly
- Edema: Congenital hereditary endothelial dystrophy (CHED)
Sclerocornea : caused by the abnormal development of the tissues of the anterior segment of the eye. It usually occurs bilaterally, but can occur unilaterally. Patients might present with varying degrees of vascularized, peripheral, white cornea rim that merges with the sclera, eliminating the limbus. The central cornea is usually normal, whereas in Peters anomaly the central cornea is opaque.
Trauma : Birth trauma (eg, forceps-induced obstetric trauma) can result in breaks in the inner layer of the cornea (Descemet membrane), leading to corneal clouding and edema. This clouding of the cornea is different from Primary congnital glauocma owing to associated trauma to periorbital soft tissues, normal intraocular pressures, absence of corneal enlargement, and a deep anterior chamber.
Ulcers – Bacterial or viral infections (eg, herpes simplex, rubella) of the cornea can cause a cloudy cornea in a newborn.
Metabolic disorders : Several lysosomal metabolic disorders and glycogen storage diseases can cause corneal clouding.
Peter’s anomaly : An uncommon disorder caused by abnormal development of the anterior segment of the eye, resulting in central or complete clouding of the cornea. It typically also involves the lens, causing the formation of cataracts.
Endothelial dystrophy (eg, congenital hereditary endothelial dystrophy) : Congenital hereditary endothelial dystrophy is either autosomal dominant or recessive. The recessive form is usually present at birth and nonprogressive, with infants experiencing intense cornea edema. On the other hand, the dominantly inherited form is usually milder in presentation, with parents of affected infants noticing corneal haziness, tearing, and sensitivity to light.
Dermoids : Limbal dermoids are benign congenital tumours that contain ectopic tissue and are most often located on the inferior temporal area of the corneal limbus. In rare instances, they present exclusively within the cornea or remain limited to the conjunctiva