Occult Nasolacrimal Duct Obstruction Secondary to IgG4-Related Ophthalmic Disease

Abstract

A 68-year-old man with known history of IgG4-related disease presented with a 1-year history of mucoid discharge OD and progressive proptosis OS. MRI showed diffuse enlargement of the left lacrimal gland along with prolonged infiltrative changes involving the left extraocular muscles, supraorbital nerve, and infraorbital nerve. No identifiable masses were noted in either nasolacrimal drainage system on MRI. Due to clinical evidence of nasolacrimal duct obstruction, a right external dacryocystorhinostomy was performed along with biopsies of the right lacrimal sac and left lacrimal gland. Immunohistochemical stains identified IgG4-positive plasma cells in the biopsy of the right lacrimal sac indicative of secondary acquired nasolacrimal duct obstruction despite the absence of mass forming lesions on exam and nasolacrimal imaging. This report is the first to describe nasolacrimal drainage system IgG4-related ophthalmic disease involvement in an otherwise clinically and radiographically normal appearing tear drainage system.

Autoimmune disease of head and neck, imaging, and clinical review

“…[46][47][48] Most nasolacrimal lesions present with high T2 signal intensity, so low T2 signal with bone erosion suggests granulomatosis with polyangiitis. 49 Imaging of the nasolacrimal drainage system includes MRI, CT, ultrasound, and nuclear scintigraphy. CT is the initial imaging of choice and shows enlargement of the lacrimal sac with pressure effect on orbital structure.…”

“…51 Differential diagnosis should include pituitary adenoma in adults and Langerhans cell histiocytosis and germinoma in adolescents. [49][50][51] Lymphocytic types are more frequent in women and often associate with late pregnancy and postpartum hypophysitis. 53 Hypophysitis may present with anterior or posterior hypophysis symptoms.…”

“…MRI demonstrates enlargement of the pituitary gland with uniform enhancement. 49,51 Chronic cases may present with pituitary gland atrophy and empty Sella making diagnosis difficult. 49,51 On MRI, Lymphocytic type is seen as a T1 isointense mass in shape of dumbbell or triangle.…”

“…49,51 Chronic cases may present with pituitary gland atrophy and empty Sella making diagnosis difficult. 49,51 On MRI, Lymphocytic type is seen as a T1 isointense mass in shape of dumbbell or triangle. It has often suprasellar extension.…”

“…46–48 Most nasolacrimal lesions present with high T2 signal intensity, so low T2 signal with bone erosion suggests granulomatosis with polyangiitis. 49…”