Bilateral cloudy cornea: is the usual suspect congenital hereditary endothelial dystrophy or stromal dystrophy?

Area of Science:

• Ophthalmology
• Pediatric Ophthalmology
• Corneal Surgery

Background:

• Congenital cloudy cornea presents a significant challenge for visual development in children.
• Traditional penetrating keratoplasty (PK) in pediatric patients often yields suboptimal prognoses.
• Accurate diagnosis is essential for guiding surgical intervention in pediatric corneal opacity.

Observation:

• A 4-year-old girl presented with bilateral cloudy corneas and blurred vision.
• Initial penetrating keratoplasty (PK) was performed under the diagnosis of congenital hereditary endothelial dystrophy.
• Pathological examination of the explanted corneal tissue revealed healthy Descemet's membrane (DM) and endothelium, indicating congenital hereditary stromal dystrophy.

Findings:

• Deep anterior lamellar keratoplasty (DALK) was subsequently performed on the left eye.
• The DM remained transparent during follow-up after DALK.
• This case highlights a diagnostic discrepancy between initial clinical assessment and post-operative pathology.

Implications:

• Alternative keratoplasty techniques like DALK and Descemet's stripping automated endothelial keratoplasty (DSAEK) offer promising alternatives to PK in pediatric cases.
• Thorough pathological examination of host corneal tissue is vital for optimizing surgical strategy and prognosis in children with congenital corneal clouding.
• Visual rehabilitation in pediatric congenital corneal opacity relies on precise diagnosis and tailored surgical approaches.