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Published on: April 7, 2014
Congenital corneal anesthesia
Muralidhar Ramappa1, Sunita Chaurasia1, Subhabrata Chakrabarti2
1Cornea and Anterior Segment Services, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India.
Insights
Congenital corneal anesthesia presents with diverse symptoms, including epithelial defects and ulcers. Early diagnosis and tarsorrhaphy are vital for preserving vision in affected children.
Area of Science:
- Ophthalmology
- Neurology
- Genetics
Background:
- Congenital corneal anesthesia is a rare condition affecting corneal sensation from birth.
- It can lead to severe ocular surface disease and vision impairment.
- Understanding its varied presentations is crucial for timely intervention.
Purpose of the Study:
- To describe the clinical spectrum of congenital corneal anesthesia in children.
- To identify associated neurological and systemic findings.
- To evaluate treatment outcomes for ocular complications.
Main Methods:
- Retrospective review of medical records of 40 children diagnosed with congenital corneal anesthesia.
- Data collected included demographics, clinical characteristics, and ocular findings.
- Analysis of treatment interventions and visual outcomes.
Main Results:
- The study included 40 patients, with 70% having bilateral involvement.
- Common corneal lesions included epithelial defects and sterile ulcers.
- Associated findings included generalized insensitivity to pain and anhidrosis in 25% of cases.
- Tarsorrhaphy led to ulcer resolution in most cases, but some eyes had poor visual outcomes after keratoplasty.
Conclusions:
- Congenital corneal anesthesia requires accurate diagnosis and comprehensive neurological assessment.
- Prompt management with tear substitutes and tarsorrhaphy is essential for visual rehabilitation.
- Long-term sequelae can be minimized with early and appropriate care.
Purpose:
To report the various clinical presentations of congenital corneal anesthesia.
Methods:
The medical records of children diagnosed with congenital corneal anesthesia between January 2006 and June 2012 at the L.V. Prasad Eye Institute, Hyderabad, India, were retrospectively reviewed for demographics and clinical characteristics.
Results:
A total of 40 patients (29 males) were included. Median age at presentation was 12 months (interquartile range, 6-60 months). Bilateral involvement was noted in 28 cases (70%). Generalized pain insensitivity with anhidrosis was documented in 10 cases (25%). The spectrum of corneal lesions included geographic epithelial defects (32 eyes), sterile ulcers (15 eyes), sterile ulcers with hypopyon (11 eyes), secondary bacterial keratitis (2 eyes), superficial/ deep corneal vascularisation (5 eyes). Generalized anesthesia with self-mutilating behavior was recognized in 10 cases. In all cases, sterile corneal ulcers resolved on average 30 days after permanent 2/3-width paramedian tarsorrhapy. One eye progressed to the stage of anterior staphyloma and subsequently underwent auto evisceration. Four eyes that underwent keratoplasty for visual rehabilitation had poor outcomes.
Conclusions:
Accurate diagnosis, recognition of risk factors, and thorough neurological assessment is important for lessening long-term sequelae of congenital corneal anesthesia. Judicious use of tear substitutes and expedited tarsorrhaphy is crucial to restoration of useful vision in these children.
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