ESCRS - PO0240 - Clinical And In Vivo Confocal Microscopy Findings In Mgus - A Case Report And Literature Review

Clinical And In Vivo Confocal Microscopy Findings In Mgus - A Case Report And Literature Review

Published 2023 - 41st Congress of the ESCRS

Reference: PO0240 | DOI: 10.82333/q951-p010

Authors: Valentino De Ruvo* 1 , alfonso strianese 1 , chiara quisisana 1 , Francesco Pozzo Giuffrida 2 , Fondazione IRCCS Ca' Granda,university of milan,milan,Italy, Francesco Viola 2 , Fondazione IRCCS Ca' Granda,university of milan,milan,Italy, Saverio Luccarelli 2 , Fondazione IRCCS Ca' Granda,university of milan,milan,Italy, Luca Rossetti 1 , Paolo Fogagnolo 1

1Department of Ophthalmology of San Paolo Hospital,university of milan,milan,Italy, 2Department of Ophthalmology of Policlinico di Milano Ospedale Maggiore

The purpose of this study was to describe a case of  paraproteinemic keratopathy (PPK) in a patient with monoclonal gammopathy of undetermined significance (MGUS) and to review literature about in vivo confocal microscopy (IVCM) in PPK in MGUS and other paraproteinemias.

Patients with MGUS there may be corneal involvement. While the paraproteinemic corneal crystals have been widely described from a histological point of view, no review about IVCM description of those deposits is present in the current literature. By the moment that to obtain histological data is necessary an invasive corneal biopsy is our opinion that it would be of great interest in current clinical practice to identify an IVCM diagnostic pattern, being IVCM a non invasive diagnostic tool.

A 56 years old man was assessed using a slit lamp and IVCM with Heidelberg HRT Rostock II. We analyzed 11 articles about IVCM in PPK in MGUS and other paraproteinemias highlighting IVCM features compatible with our findings allowing us to generalize a first diagnostic IVCM pattern for PPK.

The patient showed bilateral diffuse, symmetric, bilateral, non-polychromatic, punctiform crystals in all layers of the cornea. The remaining part of the ophthalmic clinical assessment was normal. Esthesiometry was normal in all quadrants.

Anterior segment OCT confirmed diffused hyperreflectivity of the epithelium and corneal stroma.

IVCM was then executed, showing polymorphic hyperreflective crystals in all layers of corneal epithelium and anterior stroma, subbasal plexus nerves beading, cytoplasmatic hyperreflectivity of the keratocytes of the posterior stroma without any endothelial involvement.

Despite the small number of studies  and case reports currently published on IVCM in PPK, we can highlight some cardinal IVCM features of PPK.

In conclusion, despite the small number of studies  and case reports currently published on IVCM in PPK, we can highlight some cardinal IVCM features of PPK among all types of MG: 

  • Corneal stroma is affected in nearly all cases (>97%) by crystal deposition that can be both extracellular and intracellular
  • Bowman layer involvement with subbasal nerve beading and accentuated tortuosity due to the deposition of crystals along the nerve is usually present
  • Epithelium is affected in about 10% of cases with intracellular and extracellular deposits (mainly granular or needle-like)
  • The endothelium can be affected, but its involvement is the rarest (<4%)

We suggest the IVCM diagnostic patterns that may be used to identify PPK without corneal biopsy.