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Recurrent episodes of night blindness in a patient with short bowel syndrome

  • Clinical Case Report
  • Published:
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Abstract

Purpose

To describe clinical characteristics in a patient with recurrent episodes of night blindness due to vitamin A deficiency caused by short bowel syndrome in Crohn disease.

Methods

Retrospective analysis of best-corrected visual acuity (BCVA), kinetic perimetry, slit-lamp biomicroscopy, ophthalmoscopy, fundus photography, fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT), dark adaptometry (DA) and electroretinography (ERG). Serum vitamin A level was measured.

Results

A 44-year-old man with a 3-year history of night blindness suffered from a short bowel syndrome with chronic malabsorption due to ileocecal resection in Crohn disease. Both eyes had a BCVA of 0.9, Bitot’s spots of the conjunctiva and no significant fundus abnormalities. SD-OCT showed no remarkable changes, whereas FAF was brighter than normal in the center of the fovea. DA showed normal cone and a lack of rod function. The dark-adapted 0.01 ERG was non-detectable, the dark-adapted 3.0 ERG severely diminished, but the light-adapted 3.0 and 30 Hz flicker ERGs were within normal limits. Serum vitamin A level was 0.11 µg/ml (normal 0.30–0.65 µg/ml). Treatment with intravenous vitamin A caused a rapid recovery of night vision and ERG. However, during the following 3 years, he had three further episodes of night blindness with loss of rod function. During each period, parenteral vitamin A substitution brought complete recovery of night vision and rod function. BCVA, fundus, FAF findings and SD-OCT remained unchanged during the course.

Conclusions

In patients with known chronic malabsorption serum vitamin A level should be regularly checked to avoid recurrent night blindness episodes. ERG might be more sensitive than serum vitamin A level and is recommended in case of night blindness but still normal vitamin A level.

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References

  1. Bitot P (1863) Sur une lesion conjonctivale non encore decrite coincidant avec l’hemeralopie. Gaz hebd méd 10:284–288

    Google Scholar 

  2. Darby WJ, McGanity WJ, McLaren DS, Paton D, Alemu Z, Gebre-Medhen M (1960) Bitot’s spots and vitamin A deficiency. Public Health Rep 75:738–743

    PubMed Central  CAS  PubMed  Google Scholar 

  3. McLaren DS, Oomen HAPC, Escapini H (1966) Ocular manifestations of Vitamin-A deficiency in man. Bull World Health Organ 34:357–361

    PubMed Central  CAS  PubMed  Google Scholar 

  4. Sommer A (1998) Xerophthalmia and vitamin A status. Prog Retin Eye Res 17:9–31

    Article  CAS  PubMed  Google Scholar 

  5. Smith J, Steinemann TL (2000) Vitamin A deficiency and the eye. Int Ophthalmol Clin 40:83–91

    Article  CAS  PubMed  Google Scholar 

  6. Wald G (1955) The photoreceptor process in vision. Am J Ophthalmol 40:18–41

    Article  CAS  PubMed  Google Scholar 

  7. Matthews RG, Hubbard R, Brown PK, Wald G (1963) Tautomeric forms of metarhodopsin. J Gen Physiol 47:215–240

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  8. Dowling JE, Wald G (1960) The biological formation of vitamin A acid. Pro Natl Acad Sci USA 46:587–608

    Article  CAS  Google Scholar 

  9. World Health Organization: global prevalence of vitamin A deficiency in populations at risk 1995–2005. WHO global database on vitamin A deficiency. Geneva, World Health Organisation, 2009. http://www.who.int/nutrition/publications/micronutrients/vitamin_a_deficiency/9789241598019/en/. Cited July 2015

  10. McLaughlin S, Welch J, MacDonald E, Mantry S, Ramaesh K (2014) Xerophthalmia—a potential epidemic on our doorstep? Eye 28:621–623

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  11. Zobor D, Kohl S, Wissinger B, Zrenner E, Jägle H (2012) Rod and cone function in patients with KCNV2 retinopathy. PLoS One 7:e46762

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Marmor MF, Fulton AB, Holder GE, Miyake Y, Brigell M, Bach M (2009) ISCEV Standard for full-field clinical electroretinography (2008 update). Doc Ophthalmol 118:69–77

    Article  CAS  PubMed  Google Scholar 

  13. Renner AB, Walter A, Fiebig BS, Jägle H (2012) Gyrate atrophy: clinical and genetic findings in a female without arginine-restricted diet during her first 39 years of life and report of a new OAT gene mutation. Doc Ophthalmol 125:81–89

    Article  PubMed  Google Scholar 

  14. Weleber RG, Watzke RC, Shults WT, Trzupek KM, Heckenlively JR, Egan RA, Adamus G (2005) Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies. Am J Ophthalmol 139:780–794

    Article  CAS  PubMed  Google Scholar 

  15. Adamus G (2009) Autoantibody targets and their cancer relationship in the pathogenicity of paraneoplastic retinopathy. Autoimmun Rev 8:410–414

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  16. Miyake Y, Yagasaki K, Horiguchi M, Kawase Y, Kanda T (1986) Congenital stationary night blindness with negative electroretinogram: A new classification. Arch Ophthalmol 104:1013–1020

    Article  CAS  PubMed  Google Scholar 

  17. Becker-Cohen R, Rinat C, Ben-Shalom E, Feinstein S, Ivgi H, Frishberg Y (2012) Vitamin A deficiency associated with urinary retinol binding protein wasting in Dent’s disease. Pediatr Nephrol 27:1097–1102

    Article  PubMed  Google Scholar 

  18. Apushkin MA, Fishman GA (2005) Improvement in visual function and fundus findings for a patient with vitamin A-deficient retinopathy. Retina 25:650–652

    Article  PubMed  Google Scholar 

  19. Chae T, Foroozan R (2006) Vitamin A deficiency in patients with a remote history of intestinal surgery. Br J Ophthalmol 90:955–956

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  20. McBain VA, Egan CA, Pieris SJ, Supramaniam G, Webster AR, Bird AC, Holder GE (2007) Functional observations in vitamin A deficiency: diagnosis and time course of recovery. Eye 21:367–376

    Article  CAS  PubMed  Google Scholar 

  21. Anastasakis A, Plainis S, Giannakopoulou T, Papadimitraki E, Fanouriakis C, Tsilimbaris MK (2013) Xerophthalmia and acquired night blindness in a patient with a history of gastrointestinal neoplasia and normal serum vitamin A levels. Doc Ophthalmol 126:159–162

    Article  PubMed  Google Scholar 

  22. Da Rocha Lima B, Pichi F, Lowder CY (2014) Night blindness and Crohn’s disease. Int Ophthalmol 34:1141–1144

    Article  Google Scholar 

  23. Saker S, Morales M, Jhittay H, Wen Y, Amoaku W (2015) Electrophysiological and microperimetry changes in vitamin A deficiency retinopathy. Doc Ophthalmol 130:231–240

    Article  PubMed  Google Scholar 

  24. Perlman I, Barzilai D, Haim T, Schramek A (1983) Night vision in a case of vitamin A deficiency due to malabsorption. Br J Ophthalmol 67:37–42

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  25. Slater GH, Ren CJ, Siegel N, Williams T, Barr D, Wolfe B, Dolan K, Fielding GA (2004) Serum fat-soluble vitamin deficiency and abnormal calcium metabolism after malabsorptive bariatric surgery. J Gastrointest Surg 8:48–55

    Article  PubMed  Google Scholar 

  26. Spits Y, De Laey JJ, Leroy BP (2004) Rapid recovery of night blindness due to obesity surgery after vitamin A repletion therapy. Br J Ophthalmol 88:583–585

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  27. Genead MA, Fishman GA, Lindeman M (2009) Fundus white spots and acquired night blindness due to vitamin A deficiency. Doc Ophthalmol 119:229–233

    Article  PubMed  Google Scholar 

  28. Fok JS, Li JY, Yong TY (2012) Visual deterioration caused by vitamin A deficiency in patients after bariatric surgery. Eat Weight Disord 17:e144–e146

    Article  CAS  PubMed  Google Scholar 

  29. Hayashi T, Gekka T, Nakano T, Tsuneoka H (2011) Improvement in S-cone- mediated visual fields and rod function after correction of vitamin A deficiency. Eur J Ophthalmol 21:657–660

    Article  PubMed  Google Scholar 

  30. Aleman TS, Garrity ST, Brucker AJ (2013) Retinal structure in vitamin A deficiency as explored with multimodal imaging. Doc Ophthalmol 127:239–243

    Article  PubMed  Google Scholar 

  31. Blomhoff R, Green MH, Berg T, Norum KR (1990) Transport and storage of vitamin A. Science 250:399–404

    Article  CAS  PubMed  Google Scholar 

  32. Ahmed F (1999) Vitamin A deficiency in Bangladesh: a review and recommendations for improvement. Public Health Nutr 2:1–14

    Article  CAS  PubMed  Google Scholar 

  33. Blomhoff R, Blomhoff HK (2006) Overview of retinoid metabolism and function. J Neurobiol 66:606–630

    Article  CAS  PubMed  Google Scholar 

  34. McLaren DS, Kraemer K (2012) Xerophthalmia. World Rev Nutr Diet 103:65–75

    Article  CAS  PubMed  Google Scholar 

  35. Elison JR, Friedman AH, Brodie SE (2004) Acquired subretinal flecks secondary to hypovitaminosis A in a patient with hepatitis C. Doc Ophthalmol 109:279–281

    Article  PubMed  Google Scholar 

  36. Bone RA, Landrum JT, Cains A (1992) Optical density spectra of the macular pigment in vivo and in vitro. Vision Res 32:105–110

    Article  CAS  PubMed  Google Scholar 

  37. Bone RA, Landrum JT (1992) Distribution of macular pigment components, zeaxanthin and lutein, in human retina. Methods Enzymol 213:360–366

    Article  CAS  PubMed  Google Scholar 

  38. Snodderly DM, Brown PK, Delori FC, Auran JD (1984) The macular pigment. I. Absorbance spectra, localization, and discrimination from other yellow pigments in primate retinas. Invest Ophthalmol Vis Sci 25:660–673

    CAS  PubMed  Google Scholar 

  39. Rumi G Jr, Szabó I, Vincze A, Matus Z, Tóth G, Mózsik G (2000) Decrease of serum carotenoids in Crohn’s disease. J Physiol Paris 94:159–161

    Article  PubMed  Google Scholar 

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Correspondence to Agnes B. Renner.

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Renner, A.B., Dietrich-Ntoukas, T. & Jägle, H. Recurrent episodes of night blindness in a patient with short bowel syndrome. Doc Ophthalmol 131, 221–230 (2015). https://doi.org/10.1007/s10633-015-9516-8

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  • DOI: https://doi.org/10.1007/s10633-015-9516-8

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