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

Kapoor, Anasua Ganguly, M.D., F.I.C.O.*,†; Mishra, Dilip, M.D.; VS, Vijitha, M.S., F.I.C.O.*,†; Ali, Mohammad Javed, F.R.C.S., Ph.D

Ophthalmic Plastic & Reconstructive Surgery: May/June 2019 - Volume 35 - Issue 3 - p e88
doi: 10.1097/IOP.0000000000001317
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*Ophthalmic Plastic Surgery and Ocular Oncology Services, LV Prasad Eye Institute, Vijayawada, Andhra Pradesh, India

Govindram Seksaria Institute of Dacryology, L.V. Prasad Eye Institute, Vijayawada, Andhra Pradesh, India

Ophthalmic Pathology Service, L.V.Prasad Eye Institute, Hyderabad, Telengana, India.

Accepted for publication December 13, 2018.

Dr. Ali receives royalties from Springer, Germany for his treatise “Principles and Practice of Lacrimal Surgery” and “Atlas of Lacrimal Drainage Disorders”. The remaining authors have no financial or conflicts of interest to disclose.

Address correspondence and reprint requests to Anasua Ganguly Kapoor, M.D., F.I.C.O, Govindram Seksaria Institute of Dacryology, L.V. Prasad Eye Institute, Consultant Ophthalmic Plastic Surgery & Ocular Oncology Services, Vijayawada, Krishna-521137, Andhra Pradesh, India. E-mail:

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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.





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