Bilateral Lacrimal Gland Disease: From Docile to Deadly

Abstract

 

Purpose:

 

Bilateral lacrimal gland (LG) disease represents a rare presentation with prognoses ranging from innocuous to life-threatening. The literature contains only isolated case reports describing this entity. Here, we present 62 such patients with pathology resulting from inflammatory, infectious, neoplastic, and other etiologies. Clinical presentation, radiography, pathology, & treatment outcomes were assessed.

 

Methods:

 

Retrospective review of 62 cases presenting with bilateral LG disease.

 

Results:

 

Included are 14 males and 48 females with mean age of 47 years (range 10-82, Table 1). In all cases, LG enlargement is clinically evident (Fig 2A), with the disease process either confined to the LG (Fig 2B), or showing orbital extension (Fig 2C). Categories of disease include inflammatory (n=34, 55%), lymphoproliferative (n=16, 26%), prolapsed LG (n=6, 10%), dacryops (n=5, 9%), and extramedullary hematopoiesis (n=1, 2%). Specific diagnoses include idiopathic orbital inflammation (pseudotumor, n=18, 29%), lymphoid tumor (n=16, 26%), sarcoidosis (n=8, 13%), Rosai-Dorfman disease (n=2, 3%), Erdheim-Chester disease (2, 3%), Sjogren’s Disease (2, 3%) and less common entities. Clinical features and outcomes were evaluated.

 

Conclusions:

 

Our findings indicate that bilateral LG disease is most commonly a manifestation of inflammatory and lymphoproliferative diseases. We believe this to be the largest such case series to date and suggest that evaluation and management of bilateral LG disease should focus on the most prevalent diagnoses: pseudotumor, lymphoma, and sarcoidosis.  

 

 

Keywords: lacrimal gland • imaging/image analysis: clinical • orbit