Giant Dacryocele in a Setting of Bilateral Congenital Alacrimia With Punctal and Canalicular Agenesis

Abstract

IMAGE LEGEND Giant dacryoceles with proximal lacrimal agenesis is an uncommon entity. A 51-year-old male presented with a painless mass arising from the medial aspect of the OD which has been slowly progressive since childhood. There was no associated epiphora. Examination revealed a large cystic lesion involving the right lacrimal sac region and was obstructing the visual axis (Fig. 1A). All the four puncta were absent, and the canalicular area showed atrophic changes (Fig. 1B,C). Clinically, there was no evidence of any lacrimal gland in the superotemporal fornix (Fig. 1D,E). Best corrected visual acuity was 20/20 OU with normal ocular findings. CT showed giant right dacryo cele involving the lacrimal sac region and extending along the length of the nasolacrimal duct to the inferior meatus of the nose with complete absence of lacrimal glands on both sides (Fig. 1F). Patient was treated with right dacryocystectomy. Intraoperatively a giant sac (Fig. 2A), dilatation of bony nasolacrimal duct opening (Fig. 2B), and absence of canalicular opening of the sac were noted. Histopathology showed wide and irregular lumen lined by pseudostratified columnar epithelium with goblet cells (Fig. 2C,D). Bilateral dacryocele with punctal and canalicular agenesis and alacrimia form a part of lacrimo-auricular-dento-digital syndrome or Levy Hollister syndrome. However, the patient did not have any other abnormality and hence could either represent an isolated anomaly involving the entire lacrimal system or a forme-fruste variant of the lacrimo-auricular-dento-digital syndrome. He did not have dry mouth ruling out the diagnosis of aplasia of lacrimal and major salivary glands in this case.FIG. 1FIG. 2