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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
Insights
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.
Purpose:
To report the presence of congenital alacrima in a patient with Pierre Robin (PR) sequence.
Methods:
A 6-month-old child with diagnosed PR sequence presented to us with dysfunctional lacrimation present since birth. The child subsequently developed bilateral corneal ulcers. This unusual presentation and its management are described in detail, and other ocular associations of the PR sequence are discussed.
Results:
Continuous tear supplementation and appropriate treatment with antibiotics controlled the microbial infection. However, the long-term prognosis for visual rehabilitation remained poor in this patient because of the presence of central corneal opacities, persistent dry eye, which would preclude future optical keratoplasty, and possible sensory deprivation amblyopia.
Conclusions:
Alacrima can cause significant ocular morbidity, because persistent ocular surface dryness can be very difficult to manage. Lifelong tear supplementation may be necessary. The association or coexistence of congenital alacrima and the Pierre Robin sequence has not been described before this report.
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