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Ocular manifestations of congenital insensitivity to pain: a long-term follow-up
Baker Elsana1,2, Libe Gradstein3,2, Ahed Imtirat3,2
1Ophthalmology Department, Soroka University Medical Center, Clalit Health Services, Beer Sheva, Israel bakerelsana1@gmail.com.
Insights
Children with congenital insensitivity to pain (CIP) and congenital insensitivity to pain with anhidrosis (CIPA) often develop corneal scarring. Preserved corneal sensation is linked to better vision in these patients.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Background:
- Congenital insensitivity to pain (CIP) and congenital insensitivity to pain with anhidrosis (CIPA) are rare genetic disorders characterized by the inability to feel pain.
- Ocular manifestations, particularly corneal complications, are significant concerns in these conditions.
Purpose of the Study:
- To delineate the spectrum of ocular manifestations in children diagnosed with CIPA or CIP.
- To investigate the relationship between corneal sensitivity, corneal opacities, and visual acuity in these patient groups.
Main Methods:
- Retrospective review of eye examination records for 39 children with CIPA or CIP.
- Assessment of ocular surface findings, corneal sensitivity (blink reflex), and visual acuity (VA).
- Statistical analysis to compare CIPA and CIP groups and explore correlations.
Main Results:
- Corneal opacities were more frequent in CIP (57%) than in CIPA (23%).
- A preserved blink reflex was observed in 52% of CIPA eyes versus 33% of CIP eyes.
- A preserved blink reflex correlated positively with better visual acuity (≥20/25) and negatively with corneal opacities.
Conclusions:
- Children with CIP and CIPA are susceptible to corneal scarring and vision impairment.
- CIP patients may experience more severe ocular surface disease, potentially linked to reduced corneal sensation.
- Regular ophthalmic monitoring is crucial for early detection and management of ocular complications to prevent vision loss.
Aim:
To describe ocular manifestations in children with congenital insensitivity to pain with and without anhidrosis (CIPA and CIP).
Methods:
We reviewed records of eye examinations of 39 children diagnosed with CIPA or CIP. We collected clinical data, with particular attention to ocular surface findings. Corneal sensitivity was tested by presence of a blink reflex upon touching the cornea. Statistical analysis assessed differences in manifestations between the two conditions, and relationships among corneal sensitivity, presence of corneal opacities and visual acuity (VA).
Results:
CIPA was diagnosed in 32 children and CIP in 7. The median follow-up periods were 50 months (CIPA group) and 94 months (CIP group). Corneal opacities were present in 23% of CIPA eyes and in 57% of CIP eyes. A blink reflex was positive in 52% of CIPA eyes and in 33% of CIP eyes. We recorded VA ≥20/25 in 36% of CIPA eyes, whereas all patients with CIP had VA ≤20/30. For the whole cohort, we found a negative correlation between a preserved blink reflex and the presence of corneal opacities, and a positive correlation between a preserved blink reflex and VA ≥20/25.
Conclusion:
Children with congenital insensitivity to pain are prone to develop corneal scarring. Patients with CIP tend to have more severe ocular surface disease than those with CIPA, probably due to more prevalent loss of corneal sensation. In both groups, a preserved blink reflex correlated with good vision. Affected children should have close follow-up to promptly treat ocular surface disease and prevent vision loss.
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