There are ocular changes associated with a wide spectrum of congenital, familial and acquired liver diseases and disorders. The early identification of ocular changes may aid diagnosis of the underlying liver condition. This is particularly important in conditions where there are effective treatments which can arrest hepatic damage. It is also of considerable value in patients who have untreat-able disorders because identification of the correct diagnosis may offer prognostic information and spare the patient unnecessary invasive investigation. This article discusses the ocular findings in selected liver diseases and reviews the current literature on the subject. The principles of investigation and diagnosis and treatment are described. Data on the incidence and prevalence of ocular involvement in liver conditions are included where such figures are available. The potential transmission of viral hepatitis following corneal grafting is discussed.
Triger DR : Primary biliary cirrhosis. Medicine International 1990, 84: 3470–72.
Berman SJ : Sporadic anicteric leptospirosis in South Vietnam: a study of 150 patients. Ann Intern Med 1973, 79: 167–173.
McLain JB, Ballou WR, Harrison SM, Steinweg DL : Doxy-cycline therapy for leptospirosis. Ann Intern Med 1984, 100: 696–8.
Edwards CN, Nicholson GD, Hassell TA, Everard CO, Callender J : Penecillin therapy in icteric leptospirosis. Am J Trop Med Hyg 1988, 39(4): 388–90.
Gilks CF, Lambert HP, Broughton ES, Baker CC : Failure of penecillin prophylaxis in laboratory acquired leptospirosis. Postgrad Med J 1988, 64: 236–8.
Golding PL, Smith M, Williams R : Multisystem involvement in chronic liver disease. Am J Med 1973, 55: 772–82.
Tsianos EV, Hoofnagle JH, Fox PC, Alspaugh M, Jones EA, Schafer DF, Moutsopolous HM : Sjogren's syndrome in patients with primary biliary cirrhosis. Hepatology 1990, 11: 730–4.
East T and Savin LH : Acase of Gaucher' s disease with biopsy of the typical pingueculae. Br J Ophthalmol 1940, 24: 611–3.
Sussman W and Scheinberg IH : Disappearance of Kayser-Fleischer Rings. Arch Ophthalmol 1969, 82: 738–41.
Fleming CR, Dickson ER, Wahner HW, Hollenhorst RW, McCall Jt : Pigmented Corneal Rings in Non-Wilsonian Liver Disease. Ann Int Med 1977, 86: 285–8.
Lipman RM and Deutsch TA : A yellow-green posterior limbal ring in a patient who does not have Wilson's disease. Arch Ophthalmol 1990, 108: 1385.
Phinney RB, Mondino BJ, Abrahim A : Corneal Icterus Resulting from Stromal Bilirubin Deposition. Ophthalmology 1989, 96: 1212–14.
Alagille D, Estrada A, Hadchouel M, Gautier M, Odievre M, Dommergues JP : Syndromic paucity of interlobular bile ducts (Alagille's syndrome or arteriohepatic dysplasia): Review of 80 cases. J Pediatr 1987, 110: 195–200.
Johnson B : Ocular Pathologic features of Arteriohepatic Dysplasia (Alagille's Syndrome). Am J Ophthalmol 1990, 110: 504–12.
Traboulsi EI, Lustbader JM, Lemp MA : Keratoconus in Alagille's syndrome: Am J Ophthalmol 1989, 108: 332–3.
Puklin JE, Riely CA, Simon RM, Cotlier E : Anterior Segment and Retinal Pigmentary Abnormalities in Arteriohepatic Dysplasia. Ophthalmology 1981, 88: 337–47.
Whitley CB, Ramsay NCK, Kersey JH, Krivit W : Bone Marrow Transplantation for Hurler syndrome. Assessment of metabolic correstion. Birth Defects 1986, 22: 7–24.
Goldberg MF, Maumenee AE, McKusick VA : Corneal Dystrophies Associated With Abnormalities of Mucopolysac-charide Metabolism. Arch Ophthalmol 1965, 74: 516–20.
Kenyon KR, Topping TM, Green WR, Maumenee AE : Ocular pathology of the Maroteaux-Lamy syndrome (systemic mucopolysaccharidoses type VI). Am J Ophthal-mol 1972, 73: 718–41.
Goldberg MF : A review of selected inherited corneal dystrophies associated with systemic diseases. Birth Defects 1971, 7: 13–25.
von Noorden GK, Zellwegger H, Ponsetti IV : Ocular findings in Morquio-Ullrich's disease. Arch Ophthalmol 1960, 64: 585–91.
Kenyon KR : Ocular Manifestations and Pathology of Systemic Mucopolysaccharidoses. Birth Defects 1976, 12: 133–53.
Merin S, Livni N, Berman ER, Yatziv S : Mucolipidoses IV: Ocular, systemic andultrastructural findings. Invest Ophthalmol 1975, 14: 437–48.
Kelly TE, Thomas GH, Taylor HA et al.: Mucolipidosis III (Pseudo-Hurler Polydystrophy). Clinical and Laboratory Studies in a series of 12 patients. John Hopkins Med J 1975, 137: 156–75.
Huang SS and Huang PC : Biochemical diagnosis of Genetic and Metabolic Eye Disease in Renie WA. Goldberg's Genetic and Metabolic Eye Disease, 2nd ed. Little Brown, Boston 1986: p27–80.
Libert J, Van Hoof F, Farriaux JP, Toussaint D : Ocular findings in I-cell disease (mucolipidoses type II). Am J Ophthalmol 1977, 83: 617–28.
Quigley HA, Goldberg M : Conjunctival ultrastructure in mucolipidosis III (pseudo-Hurler polydystrophy). Invest Ophthalmol 1971, 10: 568–80.
Emery JM, Green WR, Wyllie RG, Howell RR : Gml-Gangliosidosis. Ocular and pathologic manifestations. Arch Ophthalmol 1971, 85: 177–87.
Cantor LB, Disseler JA, Wilson FM : Glaucoma in the Mar-oteaux Lamy syndrome. Am J Ophthalmol 1989, 108: 426–30.
Quigley HA, Maumenee AE, Stark WJ : Acute Glaucoma in Systemic Mucopolysaccharidoses. Am J Ophthalmol 1975, 80: 70–2.
Spellacy E, Bankes J, Crow J, Dourmashkin R, Shah D, Watts R : Glaucoma in acase of Hurler's disease. Br J Ophthalmol 1980, 64: 773–8.
Christiansen SP, Smith TJ, Henslee-Downey PJ : Normal Intraocular Pressure After A Bone Marrow Transplant in Glaucoma Associated With Mucopolysaccharidoses Type I-H. Am J Ophthalmol 1990, 109: 230–1.
Burke JP, O'Keefe M, Bowell R, Naughten ER : Ophthalmic Findings In Classical Galactosemia-A Screened Population. J Pediatr Ophthalmol Strabismus 1989, 26(4): 165–8.
Torvik A, Torp S, Kase BF, Ek J, Skjeldal O, Stokke O : Infantile Refsum's disease: a generalised peroxisomal disorder. Case report with postmortem examination. J Neurol Sci 1988, 85(1): 39–53.
Stanesu-Segal B, Evrard P : Zellweger syndrome, retinal involvement. Metab Pediatr Syst Ophthalmol 1989, 12: 96–9.
Mowat AP : Liver disorders in childhood. 2nd ed. Butter-worths, London, 1987: 201–2.
Shimozawa N, Suzuki Y, Orii T, Yokota S, Hashimoto T . Biochemical and morphologic aspects of peroxisomes in the human rectal mucosa: diagnosis of Zellweger syndrome simplified by rectal biopsy. Pediatr Res Dec 1988, 24(6): 723–7.
Aubourg P, Scotto J, Rocchiccioli F, Feldmann-Pautrat D, Robain O : Neonatal adrenoleukodystrophy. J Neurol Neu-rosurg Psychiatry 1986, 49: 77–86.
Duke-Elder S : System of Ophthalmology. Kimpton, London 1964: Vol III part 2: 1127–8.
Berginer VM, Salen G, Shefer S . Long term replacement of cerebrotendernous xanthomatosis with chenodeoxycholic acid. New Engl J Med 1984, 311: 1649–52.
Gills JP, Hobson R, Hanley WB, McKusick VA : Electroreti-nography and fundus oculi findings in Hurler's disease and allied mucopolysaccharidoses Arch Ophthalmol 1965, 74: 596–603.
Crocker AC, Farber S : Niemann-Pick disease: a review of eighteen patients. Medicine 1958, 37: 1–95.
Videback A : Niemann-Pick's disease: Acute and chronic type? Acta Paediatr 1949, 37: 95–116.
Landing BH, Silverman FN, Craig MM, Jacoby MD, Lahey ME, Chadwick DL : Familial neurovisceral lipidosis. Am J Dis Child 1964, 108: 503–22.
Fine RN, Wilson WA, Donneil GA : Retinal changes in Glycogen storage type I. Am J Dis Child 1968, 115: 328–31.
Walt RP, Kemp CM . Lyness L, Bird AC . Sherlock S : Vitamin A treatment for night blindness in primary biliary cirrhosis. Br Med J 1984, 288: 1030–1.
Grey RHB : Visual field changes following hepatic transplantation in a patient with primary biliary cirrhosis. Br J Ophthalmol 1991, 75: 377–80.
Harper CC, Giles M, Finlay-Jones R : Clinical signs in the Wernicke-Korsakoff complex: a retrospective analysis of 131 cases diagnosed at necropsy. J Neurol Neurosurg Psychiat 1986, 49: 341–5.
Grover W, Naiman J : Progressive paresis of vertical gaze in lipid storage disease. Neurology 1971, 21: 896–9.
Topical diagnosis of neuropathic ocular motility disorders in: Miller NR (ed) Walsh and Hoyt's Clinical Neuro-Ophthal-mology (4th ed). Williams & Wilkins, Baltimore, 1985: Vol 2: 652–784.
O'Day DM : Diseases Potentially Transmitted Through Corneal Transplantation. Ophthalmology 1989, 96: 1133–8.
Holland EJ, Bennett SR, Brannian R et al.: The risk of cyto-megalovirus transmission by penetrating keratoplasty. Am J Ophthalmol 1988, 105: 357–60.
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O'Neill, D. The eye and liver disorders. Eye 6, 366–370 (1992). https://doi.org/10.1038/eye.1992.75