Ahead of print

Case report

Utility of in Vivo Corneal Confocal Microscopy in Atypical MEN2B Findings. A Case Report

Full Text links

Summary

Purpose: To describe atypical biomicroscopical and histological changes in corneal and conjunctival structures in multiple endocrine neoplasia type 2 (MEN2b) and bring attention to common characteristics and atypical features.

Methods: Retrospective case series.

Results: Two patients, female, and male, with previously confirmed MEN2B diagnosis were examined at our clinic to evaluate corneal and conjunctival pathologies using in vivo corneal confocal microscopy (IVCM). The female patient showed all hallmark signs of MEN2b features despite a recent unilateral herpetic infection. The male was examined at a very late stage of the disorder and showed only partial features of typical ocular MEN2b manifestations. Two notable deviations were observed: an opaque corneal mass of the right eye and absence of prominent corneal nerves in both eyes IVCM conjunctival neuroma scans correlated with scans of the corneal mass, ascertaining its histological nature.

Conclusions: This case series is, to our knowledge, the first to describe the absence of prominent corneal nerves in MEN2b. It also highlights the utility of IVCM in superficial lesion analysis. Its non-invasive nature is of great benefit to the patient.

References

  1. Mirzayev I, Gündüz AK, Ersöz CC, et al. Anterior segment optical coherence tomography, in vivo confocal microscopy, histopathologic, and immunohistochemical findings in a patient with multiple endocrine neoplasia type 2b. Ophthalmic Genet. 2020;41:1-6. doi:10.1080/13816810.2020.1795891
  2. Lam D, Villaret J, Kim PN, et al. In Vivo Confocal Microscopy of Prominent Conjunctival and Corneal Nerves in Multiple Endocrine Neoplasia Type 2B. Cornea. 2019;38:1453-1455. doi:10.1097/ico.0000000000002028
  3. Eter N, Klingmüller D, Höppner W, et al. Typical ocular findings in a pa­ tient with multiple endocrine neoplasia type 2b syndrome. Graefe’s Arch Clin Exp Ophthalmol. 2001;239:391-394. doi:10.1007/s004170000245
  4. Jacobs JM, Hawes MJ. From Eyelid Bumps to Thyroid Lumps: Report of a MEN type IIb Family and Review of the Literature. Ophthalmic Plast Rec. 2001;17:195-201. doi:10.1097/00002341-200105000-00009
  5. Yanoff M, Sharaby ML. Multiple Endocrine Neoplasia Type IIB. Arch Ophthalmol-chic. 1996;114:228. doi:10.1001/archopht.1996.01100130222027
  6. Robertson DM, Sizemore GW, Gordon H. Thickened corneal nerves as a manifestation of multiple endocrine neoplasia. Transactions Sect Ophthalmol Am Acad Ophthalmol Otolaryngology. 1975;79:772-787.
  7. Malhotra C, Jain A, Thapa B, et al. In vivo confocal microscopic architecture of corneal nerves in a case of multiple endocrine neoplasia type 2b. Middle East Afr J Ophthalmol. 2016;23:326. doi:10.4103/0974-9233.194094