Superior limbic keratoconjunctivitis

Superior limbic keratoconjunctivitis (SLK, Théodore's syndrome[1]) is a disease of the eye[2] characterized by episodes of recurrent inflammation of the superior cornea and limbus, as well as of the superior tarsal and bulbar conjunctiva.[3] It was first described by F. H. Théodore in 1963.[4]

Superior limbic keratoconjunctivitis
SLK develops due to repeated corneal microtrauma of the cornea and limbus
SpecialtyOphthalmology
SymptomsEye redness, burning sensation, tearing
DurationChronic
Risk factorsCorneal microtrauma, hyperthyroidism, hyperparathyroidism
TreatmentTopical corticosteroids, artificial tears, surgery
PrognosisExacerbations decrease with age

Symptoms and signs

Patients present with red eye, burning, tearing, foreign body sensation and mild photophobia.[5] Upon examination, the conjunctiva appears inflamed and thickened, especially at the limbus.

Pathophysiology

The development and pathophysiology of SLK is not well understood, but appears to involve microtrauma of keratoconjunctival surfaces.[6] This mechanical hypothesis is supported by the increased lid apposition of exophthalmic thyroid patients, who are known to have an increased incidence of superior limbic keratoconjunctivitis.[5]

Diagnosis

Treatment

First-line treatments include topical corticosteroids and artificial tears.[7] For non-responsive cases, potential treatments include topical ciclosporin A,[8] vitamin A,[9] autologous serum[10] and injections of triamcinolone.[11] Surgical treatment options include thermocauterization of the bulbar conjunctiva[12] and conjunctival resection,[13] typically under rose bengal (RB) staining to visualize affected areas.[7]

Epidemiology

Superior limbic keratoconjunctivitis tends to occur more often with dry eye syndrome (keratoconjunctivitis sicca), hyperthyroidism and hyperparathyroidism.[14] It is also a rare complication associated with rheumatoid arthritis.[3] Rarely, it may occur as a consequence of upper eyelid blepharoplasty surgery.[15]

References
  1. Matsuo, H. (July 1970). "[Superior limbic keratoconjunctivitis (Theodore's syndrome)]". Ganka. Ophthalmology. 12 (7): 612. ISSN 0016-4488. PMID 5466382.
  2. "Superior limbic keratoconjunctivitis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2017-09-30.
  3. "Superior Limbic Keratoconjunctivitis - EyeWiki". eyewiki.aao.org. Retrieved 2021-07-03.
  4. Theodore, F. H. (January 1963). "Superior limbic keratoconjunctivitis". Eye, Ear, Nose & Throat Monthly. 42: 25–28. ISSN 0014-5491. PMID 13980870.
  5. "Superior Limbic Keratoconjunctivitis (SLK): Background, Etiology and Pathophysiology, Pathophysiology". 2021-06-17. {{cite journal}}: Cite journal requires |journal= (help)
  6. Cher, Ivan (2000). "Superior limbic keratoconjunctivitis: multifactorial mechanical pathogenesis". Clinical & Experimental Ophthalmology. 28 (3): 181–184. doi:10.1046/j.1442-9071.2000.00284.x. ISSN 1442-9071. PMID 10981793. S2CID 23900077.
  7. Yokoi, Norihiko; Komuro, Aoi; Maruyama, Kunio; Tsuzuki, Masakatsu; Miyajima, Seiya; Kinoshita, Shigeru (2003-03-01). "New surgical treatment for superior limbic keratoconjunctivitis and its association with conjunctivochalasis". American Journal of Ophthalmology. 135 (3): 303–308. doi:10.1016/S0002-9394(02)01975-X. ISSN 0002-9394. PMID 12614746.
  8. Perry, Henry D.; Doshi-Carnevale, Sima; Donnenfeld, Eric D.; Kornstein, Howard S. (2003-08-01). "Topical cyclosporine A 0.5% as a possible new treatment for superior limbic keratoconjunctivitis". Ophthalmology. 110 (8): 1578–1581. doi:10.1016/S0161-6420(03)00538-4. ISSN 0161-6420. PMID 12917176.
  9. Ohashi, Yuichi; Watanabe, Hitoshi; Kinoshita, Shigeru; Hosotani, Hisashi; Umemoto, Masayo; Manabe, Reizo (1988-05-01). "Vitamin A Eyedrops for Superior Limbic Keratoconjunctivitis". American Journal of Ophthalmology. 105 (5): 523–527. doi:10.1016/0002-9394(88)90245-0. ISSN 0002-9394. PMID 3285694.
  10. Goto, Eiki; Shimmura, Shigeto; Shimazaki, Jun; Tsubota, Kazuo (November 2001). "Treatment of Superior Limbic Keratoconjunctivitis by Application of Autologous Serum". Cornea. 20 (8): 807–810. doi:10.1097/00003226-200111000-00006. ISSN 0277-3740. PMID 11685056. S2CID 22666102.
  11. Shen, Ying-Cheng; Wang, Chun-Yuan; Tsai, Hin-Yeung; Lee, Yi-Fen (May 2007). "Supratarsal Triamcinolone Injection in the Treatment of Superior Limbic Keratoconjunctivitis". Cornea. 26 (4): 423–426. doi:10.1097/ICO.0b013e318030d230. ISSN 0277-3740. PMID 17457190. S2CID 43314789.
  12. Udell, Ira J.; Kenyon, Kenneth R.; Sawa, Mitsuru; Dohlman, Claes H. (1986-02-01). "Treatment of Superior Limbic Keratoconjunctivitis by Thermocauterization of the Superior Bulbar Conjunctiva". Ophthalmology. 93 (2): 162–166. doi:10.1016/S0161-6420(86)33766-7. ISSN 0161-6420. PMID 3951822.
  13. Passons, Gary A.; Wood, Thomas O. (1984-08-01). "Conjunctival Resection for Superior Limbic Keratoconjunctivitis". Ophthalmology. 91 (8): 966–968. doi:10.1016/S0161-6420(84)34207-5. ISSN 0161-6420. PMID 6493706.
  14. Nelson, J. D. (March 1989). "Superior limbic keratoconjunctivitis (SLK)". Eye. 3 (2): 180–189. doi:10.1038/eye.1989.26. ISSN 1476-5454. PMID 2695351.
  15. Sheu, Mike C.; Schoenfield, Lynn; Jeng, Bennie H. (May 2007). "Development of superior limbic keratoconjunctivitis after upper eyelid blepharoplasty surgery: support for the mechanical theory of its pathogenesis". Cornea. 26 (4): 490–492. doi:10.1097/ICO.0b013e3180303b02. ISSN 0277-3740. PMID 17457203. S2CID 38690433.


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