Bilateral dacryoceles associated with bilateral alacrimia with punctal and canalicular agenesis

To report a rare case of a 19 year old female presenting with bilateral dacryocele and punctal and canalicular agenesis without epiphora. After clinical and radiological examination, the patient was found to have bilateral lacrimal gland agenesis as well. No other dental, otological or systemic abnormality suggestive of lacrimo-auricular-dento-digital syndrome was detected. She was managed with bilateral dacryocystectomy. Algorithm for managing such nonconventional lacrimal outflow dysgenesis based on presenting symptoms and co-existing epiphora is proposed. Rationale of preferring lacrimal sac excision instead of dacryocystorhinostomy in this case is also discussed.