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Congenital alacrima in Pierre Robin sequence
Sudesh K Arya1, Zia Chaudhuri, Rajeev Jain
1Government Medical College, Sector 32, Chandigarh 160047, India. aryasudesh@yahoo.co.in
Cornea
|July 17, 2004
Summary
Congenital alacrima, the absence of tears, was observed in a patient with Pierre Robin sequence. This rare combination can lead to severe eye problems and requires lifelong management.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Background:
- Pierre Robin sequence is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
- Ocular manifestations in Pierre Robin sequence are not uncommon, but congenital alacrima is a previously undescribed association.
Observation:
- A 6-month-old infant diagnosed with Pierre Robin sequence presented with congenital alacrima (absence of tear production) since birth.
- The patient developed bilateral corneal ulcers, necessitating prompt medical intervention.
Findings:
- Management involved continuous tear supplementation and antibiotic treatment to control corneal infections.
- Despite treatment, the patient's long-term visual prognosis was poor due to central corneal opacities, persistent dry eye, and potential sensory deprivation amblyopia.
Implications:
- Congenital alacrima can lead to significant ocular morbidity and visual impairment.
- This case highlights the importance of comprehensive eye examinations in patients with Pierre Robin sequence.
- Lifelong management with artificial tears is crucial for patients with alacrima to prevent ocular surface damage.