Ligneous conjunctivitis in a Dandy-Walker syndrome: A rare case report

Area of Science:

• Ophthalmology
• Genetics
• Pediatrics

Background:

• Ligneous conjunctivitis (LC) is a rare, chronic inflammatory condition characterized by thick, woody exudates on the conjunctiva.
• It is often associated with plasminogen deficiency, impairing local fibrinolytic activity and leading to the accumulation of fibrin-rich membranes.
• This condition primarily affects children and can be refractory to conventional treatments.

Observation:

• This case report describes a pediatric patient presenting with recurrent ligneous conjunctivitis.
• The patient also exhibited progressive congenital hydrocephalus, diagnosed as Dandy-Walker syndrome due to aqueductal stenosis.
• The co-occurrence of these two distinct conditions in a single patient is highly unusual.

Findings:

• The clinical presentation confirmed the simultaneous presence of ligneous conjunctivitis and Dandy-Walker syndrome.
• Diagnostic workup supported the link between impaired fibrinolysis (plasminogen deficiency) and the conjunctival membranes.
• The hydrocephalus was attributed to aqueductal stenosis, a known feature within the Dandy-Walker spectrum.

Implications:

• This case highlights a rare association between a fibrinolytic disorder and a congenital neurological malformation.
• Understanding this co-occurrence may offer insights into shared or interacting pathophysiological pathways.
• Prompt surgical intervention for hydrocephalus was crucial for managing the neurological condition in this pediatric patient.