Idiopathic Canalicular Inflammatory Disease: New Disease Description of Clinical Patterns, Investigations, Management, and Outcomes

Purpose: The objective of this perspective is to present a separate disease description of "idiopathic canalicular inflammatory disease" and outline the diagnostic criteria and early experiences with its investigations and management. Methods: Retrospective case series of 44 canaliculi of 22 eyes of 11 patients presenting at a tertiary care Dacryology service over a period of 2 years with typical clinical patterns of inflammatory canaliculitis and its outcomes were studied. All the patients underwent microbiological work-up with culture and sensitivity, dacryoendoscopy imaging, serial Fourier domain ocular coherence tomography, and collagen vascular profiles. Stages in the evolution of the disease were studied. All patients were treated initially with topical steroids followed by punctal dilatation and placement of mini-monoka stents. Five patients in addition had a small biopsy from the inflamed portion of the vertical canaliculus. Stents were extubated at 6 weeks. Results: Forty-four canaliculi were diagnosed to have idiopathic canalicular inflammatory disease during the study period. There was a female preponderance (81.8%, 9/11) and the mean age at presentation was 57 years. All patients presented with unilateral epiphora without any discharge, pain, or swelling. Collagen vascular profiles and screening for autoimmune diseases were negative. Clinical picture ranged from stages 1 to 5, consisting of edema, progressive centripetal vascularization, pouting of vascularized mucosa, membrane formation, and progressive scarring. The presentation begins in 1 eye and usually involves the other eye at a mean of 6 months. Ocular coherence tomography and dacryoendoscopy were of adjunctive value in the diagnosis. Histopathological examination was suggestive of a chronic inflammation. All patients had relentless progression to end-stage disease, although delayed significantly by steroids and monoka intubation. Conclusion: Idiopathic canalicular inflammatory disease has a distinct and typical clinical behavior and the current study proposed diagnostic features and disease staging. The use of topical and systemic immunosuppressive agents needs to be explored to formulate effective protocols for its management. Accepted for publication December 8, 2017. Mohammad Javed Ali received support from the Alexander von Humboldt Foundation for his research and he also receives royalties from Springer for the textbook "Principles and Practice of Lacrimal Surgery' and Atlas "Atlas of Lacrimal Drainage Disorders". The authors have no conflicts of interest to disclose. Address correspondence and reprint requests to Mohammad Javed Ali, F.R.C.S., Govindram Seksaria Institute of Dacryology, L.V.Prasad Eye Institute, Road No. 2, Banjara Hills, Hyderabad-34, India. E-mail: drjaved007@gmail.com © 2018 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.