Trigeminal Trophic Syndrome Associated with Intense Itching, Eye Rubbing and Ipsilateral Keratoconus after Trigeminal Decompression Surgery: A Case Report

Introduction

Keratoconus (KC) is the most common of the corneal ectasias and is characterized by an inferotemporal, paracentral thinning with corresponding steepening [1]. It is considered a bilateral, highly asymmetrical disease [1], [2]. The existence of unilateral KC has been debated in the literature, as some patients with “unilateral” disease have had their “normal” eye progress to KC through time, while others did not [2]. Additionally, “normal” eyes of KC patients have shown statistically significant differences in their corneal topography against non-KC controls [2]. While the exact pathophysiology regarding the development of KC remains to be elucidated, several risk factors have been identified [1]. Eye rubbing has been consistently identified as a risk factor for KC, with a recent metanalysis reporting an odds ratio of 3.09 (95% confidence interval: 2.17 – 4.00) for the development of KC in patients with eye rubbing [3]. Eye rubbing can be associated with different clinical scenarios such as itching in atopic and allergic conjunctivitis, compulsive behaviors, emotional stress, and accommodative asthenopia [4]. An extremely uncommon cause of itching and subsequent eye rubbing is the neuropathic perceptions caused by damage to the trigeminal nerve [5]. These perceptions can be reported as pain, numbness, tingling, prickling, or intense itching [5], [6]. Trigeminal nerve damage has been previously associated with corneal and facial skin ulcerations and referred to as trigeminal trophic syndrome (TTS) [6]. More rarely, a concomitant appearance of an unrelenting neuropathic itch in TTS has been reported [7]. To the authorsʼ best knowledge, TTS has not been previously associated with intense itching, eye rubbing, and ipsilateral KC. Therefore, it is the objective of the present paper to report on a case with the aforementioned findings.

Publication History

Received: 20 April 2024

Accepted: 09 August 2024

Accepted Manuscript online:
20 August 2024

Article published online:
10 October 2024

• References

• 1 Santodomingo-Rubido J, Carracedo G, Suzaki A. et al. Keratoconus: An updated review. Cont Lens Anterior Eye 2022; 45: 101559
• 2 Değirmenci C, Palamar M, İsmayilova N. et al. Topographic evaluation of unilateral keratoconus patients. Turk J Ophthalmol 2019; 49: 117-122
• 3 Hashemi H. et al. The prevalence and risk factors for keratoconus: a systematic review and meta-analysis. Cornea 2020; 39: 263-270
• 4 Najmi H, Mobarki Y, Mania K. et al. The correlation between keratoconus and eye rubbing: a review. Int J Ophthalmol 2019; 12: 1775
• 5 Garza I. The trigeminal trophic syndrome: an unusual cause of face pain, dysaesthesias, anaesthesia and skin/soft tissue lesions. Cephalalgia 2008; 28: 980-985
• 6 McVeigh KA, Adams M, Harrad R. et al. Periocular manifestations of trigeminal trophic syndrome: A case series and literature review. Orbit 2018; 37: 32-35
• 7 Bradburn KH, Elston D, Murphey AW. et al. Trigeminal Trophic Syndrome–A Unique Clinical Presentation of a Rare Condition. Ear Nose Throat J 2019; 98: 606-608
• 8 Poddar S, Mondal H, Podder I. Aetiology, pathogenesis and management of neuropathic itch: A narrative review with recent updates. Indian J Dermatol Venereol Leprol 2023; 90: 5-18
• 9 Huang CC, Yang W, Guo C. et al. Anatomical and functional dichotomy of ocular itch and pain. Nat Med 2018; 24: 1268-1276
• 10 Kuruvilla M, Kalangara J, Lee FE. Neuropathic pain and itch mechanisms underlying allergic conjunctivitis. J Investig Allergol Clin Immunol 2019; 29: 349-356
• 11 Ertan A, Muftuoglu O. Keratoconus clinical findings according to different age and gender groups. Cornea 2008; 27: 1109-1113
• 12 Henriquez MA, Hadid M, Izquierdo jr. L. A Systematic Review of Subclinical Keratoconus and Forme Fruste Keratoconus. J Refract Surg 2020; 36: 270-279