A Review of Corneal Endotheliitis and Endotheliopathy: Differential Diagnosis, Evaluation, and Treatment

Abstract

The corneal endothelium plays an integral role in regulating corneal hydration and clarity. Endotheliitis, defined as inflammation of the corneal endothelium, may disrupt endothelial function and cause subsequent visual changes. Corneal endotheliitis is characterized by corneal edema, the presence of keratic precipitates, anterior chamber inflammation, and occasionally limbal injection, neovascularization, and co-existing or superimposed uveitis. The disorder is classified into four subgroups: linear, sectoral, disciform, and diffuse. Its etiology is extensive and, although commonly viral, may be medication-related, procedural, fungal, zoological, environmental, or systemic. Not all cases of endothelial dysfunction leading to corneal edema are inflammatory in nature. Therefore, it is imperative that practitioners consider a broad differential for patients presenting with possible endotheliitis, as well as familiarize themselves with appropriate diagnostic and therapeutic modalities.

Keywords: Bacterial endotheliitis; Corneal edema; Endothelial dysfunction; Endotheliitis; Endotheliopathy; Keratic precipitates; Polymegathism; Pseudoguttata; Viral endotheliitis.

Fig. 1

Anterior segment photography demonstrating different patterns of endotheliitis. a Linear—fine KP (white arrow) in a linear pattern and overlying corneal edema. b Sectoral—HSV endotheliitis presenting with sectoral corneal edema (white arrows). c Disciform—CMV endotheliitis presenting in a disciform pattern (white arrow) with overlying edema. d Diffuse—endotheliitis presenting with diffuse KP (white arrow). KP keratic precipitates (Courtesy of Dr. Majid Moshirfar)

Fig. 2

Anterior segment photograph of a patient with EBV presenting with a subtle stromal reaction, KP of differing sizes and overlying corneal edema and haze (Courtesy of Dr. Majid Moshirfar, MD)

Fig. 3

Anterior segment photograph of amantadine-associated corneal edema with Descemet’s folds (Courtesy of Dr. Dean Ouano, MD)

Fig. 4

Anterior segment photograph of endotheliitis secondary to tarantula hair exposure (Courtesy of James Gilman, Moran Eye Center)

Fig. 5

Specular microscopy of a patient with CMV. The corneal endothelium shows polymorphism and polymegethism in the lower left corner as well as pseudoguttata (Courtesy of Dr. Majid Moshirfar)

Fig. 6

Algorithm for Diagnosis and Treatment of Endotheliitis