Congenital alacrima in Pierre Robin sequence

Cornea. 2004 Aug;23(6):632-4. doi: 10.1097/01.ico.0000121700.74077.31.


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.

Publication types

  • Case Reports

MeSH terms

  • Corneal Edema / diagnosis
  • Corneal Edema / etiology
  • Corneal Ulcer / diagnosis
  • Corneal Ulcer / etiology
  • Eye Abnormalities / complications*
  • Eye Abnormalities / diagnostic imaging
  • Female
  • Humans
  • Infant
  • Lacrimal Apparatus / abnormalities*
  • Lacrimal Apparatus / diagnostic imaging
  • Pierre Robin Syndrome / complications*
  • Pierre Robin Syndrome / diagnostic imaging
  • Tomography, X-Ray Computed