Dacryocystorhinostomy Precipitating Keratoconjunctivitis Sicca in Aplasia of Lacrimal and Major Salivary Glands (ALSG)

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We report a 16-month-old girl referred for bilateral epiphora and sticky eyes since birth. Examination revealed a refluxible left lacrimal sac mucocele, agenesis of the left lower punctum, and agenesis of both puncta on the right side. Complete bony obstruction was noted on probing of the left nasolacrimal duct. At 4 years of age, she underwent left external dacryocystorhinostomy (DCR) with silicone intubation because of chronic dacryocystitis. Her epiphora and stickiness improved significantly in the first postoperative year, but she subsequently developed dryness of the left eye, dry mouth, and dental caries. CT and MRI scans revealed the absence of the lacrimal and salivary glands. The clinical signs and symptoms improved with plugging the left upper punctum and topical lubricants.

Aplasia of the lacrimal and salivary glands may present with symptoms of congenital lacrimal obstruction, and failure to make an early diagnosis will result in inappropriate lacrimal surgery and dry eye.

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