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Self-inflicted corneal injuries in children with congenital corneal anaesthesia
Insights
Self-inflicted eye scratching caused severe corneal ulcers in boys with congenital corneal anesthesia. Arm splints enabled rapid healing, highlighting a preventable cause of eye injury.
Area of Science:
- Ophthalmology
- Pediatrics
- Genetics
Background:
- Congenital corneal anesthesia can lead to corneal ulceration.
- Self-inflicted injury is a potential, yet often unrecognized, cause of corneal ulcers in affected children.
Observation:
- Four young boys with CCA presented with severe, non-healing corneal ulcers.
- Observation revealed that the children were repeatedly scratching their own eyes, exacerbating the corneal damage.
- This self-inflicted injury was the primary cause of the refractory corneal ulceration.
Findings:
- Application of arm splints to prevent eye-scratching resulted in rapid healing of the corneal ulcers in all four patients.
- Recurrences were noted in two cases but resolved quickly with re-application of splints.
- While most children had normal development, two exhibited neurological signs suggestive of central nervous system malformations.
Implications:
- Self-inflicted eye injury is a critical, previously unrecognized cause of corneal ulceration in children with CCA.
- Early identification of eye-scratching behavior and prompt intervention with splinting are crucial for preventing vision loss.
- Routine corneal sensation testing and consideration of self-inflicted injury are recommended for children with refractory corneal ulcers, even with CCA.
Abstract:
Severe corneal ulceration related to self-inflicted injury in the presence of congenital corneal anaesthesia is described in four boys under 2 1/2 years of age. The ulcers had failed to heal until it was recognised that the children were scratching their own eyes. The application of arm splints allowed rapid healing. Although corneal ulceration is a recognised complication of congenital corneal anaesthesia, this preventable cause of the ulceration has not previously been recognised. In two cases there were isolated recurrences which healed quickly with the reapplication of splints. All four children had good vision initially, and, although there were no overt gross development abnormalities, two had neurological signs on detailed investigation suggesting cerebellar or brain stem malformation and one had unilateral anophthalmos, talipes equinovarus, and patent ductus arteriosus. All the children showed normal intellectual development. Whether the eye scratching behaviour was the primary cause of the ulceration or merely an aggravating factor, the identification of this abnormal behaviour is important in any child with idiopathic corneal ulceration, as even in the presence of congenital corneal anaesthesia the eyes heal quickly with effective splinting of the elbows. It is therefore important to test sensation of the cornea and face and to consider the possibility of self-inflicted injury in children with refractory corneal ulceration, as in our cases there were no other consistent diagnostic features.