Neurotrophic Keratopathy Clinical Presentation

  • Author: Robert H Graham, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 14, 2012
 

History

A careful medical and surgical history should be obtained. Inquire about the following:

  • Previous surgical or traumatic injury to the trigeminal nerve, ocular surgery, or laser treatment, which may have damaged the ciliary nerves
  • Previous herpetic eye disease or a history of herpes zoster ophthalmicus[6]
  • Diabetes mellitus[7]
  • Use of topical medications, including potential abuse of topical anesthetics or nonsteroidal anti-inflammatory drugs (NSAIDs)

Also inquire about the use of contact lenses or exposure to chemical fumes.

Next

Physical

Cranial nerve examination

A cranial nerve examination can help localize the cause of corneal hypesthesia. Pupillary abnormality may indicate pathology of the intraconal orbit or cavernous sinus or may reveal an Adie pupil. Dysfunction of cranial nerves III, IV, and VI may indicate an aneurysm or cavernous sinus pathology. Dysfunction of cranial nerves VII and VIII may indicate acoustic neuroma or injury from its resection.

External examination

Cranial nerve VII function should be assessed not only for its value in localizing the cause of hypesthesia but also for its prognostic value. Poor lid closure promotes exposure and can hasten progression. The presence of scars from surgery, chemical burns, or thermal burns can provide clues as to the cause of the hypesthesia. Ectropion, lagophthalmos, or thyroid ophthalmopathy increase the risk of progression.

Ocular surface examination

The function of the tear film should be carefully examined for its impact on the management of neurotrophic keratopathy.[8, 9]

Corneal sensitivity should be assessed. To do so, a piece of twisted cotton or the corner of a tissue is used. A Cochet-Bonnet esthesiometer is a device that can give a quantitative measurement of corneal sensitivity. It consists of a nylon filament, which can be extended from the device to different lengths and touched to the cornea until it bends or the patient responds. The small diameter of the instrument allows accurate testing of different areas of the cornea. The shorter the length of filament required, the less sensitive the cornea. In one study, only those patients with readings of 2 cm or less developed epithelial sloughing and ulceration.

Slit-lamp examination may show indications of the underlying cause of corneal hypesthesia. These include herpetic epithelial disease, stromal scarring from previous infection, lattice or granular stromal dystrophy, and enlarged or beaded corneal nerves from leprosy.

Mackie classification for neurotrophic keratopathy

Stage 1 is as follows:

  • Rose bengal staining of the inferior palpebral conjunctiva
  • Decreased tear break-up time
  • Increased mucous viscosity
  • Punctate epithelial fluorescein stainin

Stage 2 is as follows:

  • Epithelial defect, usually oval and in the superior cornea
  • Defect surrounded by a rim of loose epithelium
  • Edges may become smooth and rolled
  • Stromal swelling with folds in the Descemet membrane
  • Sometimes associated with anterior chamber inflammatory
  • action

Stage 3 is as follows:

  • Stromal lysis/melting
  • May result in perforation

Anterior segment examination

This may reveal iris atrophy from a prior herpetic infection or an anterior chamber inflammatory reaction.

Dilated funduscopy

Optic nerve swelling or pallor may indicate an orbital lesion or a retroorbital lesion. Diabetic retinopathy could indicate the likelihood of diabetic neuropathy. Laser scars from panretinal photocoagulation may indicate ciliary nerve damage.

Previous
Next

Causes

The causes of neurotrophic keratopathy are conditions that decrease corneal sensitivity. The most common of these are herpetic infections of the cornea, surgery for trigeminal neuralgia, and surgery for acoustic neuroma.

Infectious causes are as follows:

Fifth nerve palsy causes are as follows:

  • Surgery for trigeminal neuralgia
  • Neoplasia (acoustic neuroma)
  • Aneurysms
  • Facial trauma
  • Congenital
  • Familial dysautonomia (Riley-Day syndrome)
  • Goldenhar-Gorlin syndrome
  • Möbius syndrome
  • Familial corneal hypesthesia

Topical medication causes are as follows:

  • Anesthetics
  • Timolol
  • Betaxolol
  • Sulfacetamide
  • Diclofenac sodium
  • Ketorolac

Corneal dystrophies include the following:

  • Lattice
  • Granular

Systemic disease causes are as follows:

  • Diabetes mellitus[7]
  • Vitamin A deficiency
  • Multiple sclerosis

Iatrogenic causes are as follows:

  • Contact lens wear
  • Trauma to ciliary nerves by laser treatment and surgery
  • Corneal incisions
  • Laser in situ keratomileusis (LASIK)[10]

Toxic causes are as follows:

  • Chemical burns
  • Carbon disulfide exposure
  • Hydrogen sulfide exposure

Miscellaneous causes are as follows:

  • Increasing age
  • Dark eye color
  • Adie syndrome
Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Robert H Graham, MD  Senior Associate Consultant, Department of Ophthalmology, Mayo Clinic, Scottsdale, Arizona

Robert H Graham, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and Arizona Ophthalmological Society

Disclosure: WebMD/eMedicine Salary Employment

Coauthor(s)

Mark A Hendrix, MD  Consulting Staff, Department of Ophthalmology, Suburban Hospital, Shady Grove Hospital

Mark A Hendrix, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen D Plager, MD, FACS  Chief, Department of Ophthalmology, Dominican Hospital; Assistant Clinical Professor, Department of Ophthalmology, Stanford University Hospital

Stephen D Plager, MD, FACS is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and California Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Institute

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; RPS Ownership interest Other; EyeGate Pharma Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting; Merck Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Bonini S, Rama P, Olzi D, Lambiase A. Neurotrophic keratitis. Eye. Nov 2003;17(8):989-95. [Medline].

  2. Cohen EJ, Rapuano CJ. Neurotrophic keratopathy. In: Friedberg MA, Rapuano CJ, eds. Wills Eye Hospital office and emergency room diagnosis and treatment of eye. 54-55.

  3. Groos EB. Neurotrophic keratitis. In: Krachmer JH, ed. Cornea: Fundamentals of Corneal and External Disease. Mosby: St. Louis; 1997:1339-62.

  4. Okada Y, Reinach PS, Kitano A, Shirai K, Kao WW, Saika S. Neurotrophic keratopathy; its pathophysiology and treatment. Histol Histopathol. Jun 2010;25(6):771-80. [Medline].

  5. Fogle JA, Kenyon KR, Foster CS. Tissue adhesive arrests stromal melting in the human cornea. Am J Ophthalmol. Jun 1980;89(6):795-802. [Medline].

  6. Pavan-Langston D, Dohlman CH. Boston keratoprosthesis treatment of herpes zoster neurotrophic keratopathy. Ophthalmology. Feb 2008;115(2 Suppl):S21-3. [Medline].

  7. Lockwood A, Hope-Ross M, Chell P. Neurotrophic keratopathy and diabetes mellitus. Eye. Jul 2006;20(7):837-9. [Medline].

  8. Allen VD, Malinovsky V. Management of neurotrophic keratopathy. Cont Lens Anterior Eye. Sep 2003;26(3):161-5. [Medline].

  9. Lambiase A, Rama P, Aloe L, Bonini S. Management of neurotrophic keratopathy. Curr Opin Ophthalmol. Aug 1999;10(4):270-6. [Medline].

  10. Latvala T, Linna T, Tervo T. Corneal nerve recovery after photorefractive keratectomy and laser in situ keratomileusis. Int Ophthalmol Clin. Fall 1996;36(4):21-7. [Medline].

  11. Mackie IA. Neuroparalytic keratitis. In: Fraunfelder F, Roy FH, eds. Current Ocular Therapy. 5th ed. Philadelphia: WB Saunders; 2000:369-371.

  12. Goins KM. New insights into the diagnosis and treatment of neurotrophic keratopathy. Ocul Surf. Apr 2005;3(2):96-110. [Medline].

  13. Bonini S, Lambiase A, Rama P, Caprioglio G, Aloe L. Topical treatment with nerve growth factor for neurotrophic keratitis. Ophthalmology. Jul 2000;107(7):1347-51; discussion 1351-2. [Medline].

  14. Lambiase A, Rama P, Bonini S, Caprioglio G, Aloe L. Topical treatment with nerve growth factor for corneal neurotrophic ulcers. N Engl J Med. Apr 23 1998;338(17):1174-80. [Medline].

  15. Hosotani H, Ohashi Y, Yamada M, Tsubota K. Reversal of abnormal corneal epithelial cell morphologic characteristics and reduced corneal sensitivity in diabetic patients by aldose reductase inhibitor, CT-112. Am J Ophthalmol. Mar 1995;119(3):288-94. [Medline].

  16. Nishida T, Chikama T, Morishige N, Yanai R, Yamada N, Saito J. Persistent epithelial defects due to neurotrophic keratopathy treated with a substance p-derived peptide and insulin-like growth factor 1. Jpn J Ophthalmol. Nov-Dec 2007;51(6):442-7. [Medline].

  17. Lugo M, Arentsen JJ. Treatment of neurotrophic ulcers with conjunctival flaps. Am J Ophthalmol. May 15 1987;103(5):711-2. [Medline].

  18. Prabhasawat P, Tesavibul N, Komolsuradej W. Single and multilayer amniotic membrane transplantation for persistent corneal epithelial defect with and without stromal thinning and perforation. Br J Ophthalmol. Dec 2001;85(12):1455-63. [Medline].

  19. Kruse FE, Rohrschneider K, Volcker HE. Multilayer amniotic membrane transplantation for reconstruction of deep corneal ulcers. Ophthalmology. Aug 1999;106(8):1504-10; discussion 1511. [Medline].

  20. Solomon A, Meller D, Prabhasawat P, et al. Amniotic membrane grafts for nontraumatic corneal perforations, descemetoceles, and deep ulcers. Ophthalmology. Apr 2002;109(4):694-703. [Medline].

  21. Reynolds SA, Kabat AG. Therapeutic options for the management of early neurotrophic keratopathy: a case report and review. Optometry. Oct 2006;77(10):503-7. [Medline].

  22. Cavanagh HD, Colley AM. The molecular basis of neurotrophic keratitis. Acta Ophthalmol Suppl. 1989;192:115-34. [Medline].

  23. Hauck MJ, Harold Lee H, Timoney PJ, Shoshani Y, Nunery WR. Neurotrophic Corneal Ulcer After Retrobulbar Injection of Chlorpromazine. Ophthal Plast Reconstr Surg. Nov 11 2011;[Medline].

  24. Matsumoto Y, Dogru M, Goto E, et al. Autologous serum application in the treatment of neurotrophic keratopathy. Ophthalmology. Jun 2004;111(6):1115-20. [Medline].

  25. Parmar DN, Alizadeh H, Awwad ST, et al. Ocular surface restoration using non-surgical transplantation of tissue-cultured human amniotic epithelial cells. Am J Ophthalmol. Feb 2006;141(2):299-307. [Medline].

  26. Schrader S, Wedel T, Moll R, Geerling G. Combination of serum eye drops with hydrogel bandage contact lenses in the treatment of persistent epithelial defects. Graefes Arch Clin Exp Ophthalmol. Oct 2006;244(10):1345-9. [Medline].

  27. Yamada N, Matsuda R, Morishige N, et al. Open clinical study of eye-drops containing tetrapeptides derived from substance P and insulin-like growth factor-1 for treatment of persistent corneal epithelial defects associated with neurotrophic keratopathy. Br J Ophthalmol. Jul 2008;92(7):896-900. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.