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• Further considerations in retinopathy with renal failure
  Moin D. Mohamed
  Published on: 18 October 2002

• Published on: 18 October 2002

  Further considerations in retinopathy with renal failure

  • Moin D. Mohamed, Wellcome Trust Clinical Research Fellow
  • Other Contributors:
    • Martin A. McKibbin

  Dear Editor

  We were interested to read the letter by Patel et al. reporting the unusual and striking fundus appearance of retinal arteriolar calcification in association with chronic renal failure.[1] However, we feel that two important conditions have been omitted from the comment, which merit further discussion.

  First, Monckeberg’s sclerosis, which in its classic form is characterised by ‘pipe-stem’...

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  Dear Editor

  We were interested to read the letter by Patel et al. reporting the unusual and striking fundus appearance of retinal arteriolar calcification in association with chronic renal failure.[1] However, we feel that two important conditions have been omitted from the comment, which merit further discussion.

  First, Monckeberg’s sclerosis, which in its classic form is characterised by ‘pipe-stem’ calcific deposition in the medial coat of muscular arteries in middle-aged and elderly individuals,[2] and is described with widespread systemic distribution.[3,4] Interestingly though, a juvenile form is seen, particularly in association with chronic renal failure and diabetes.[5] The pathological mechanism of Monckeberg’s sclerosis is uncertain, but is thought to encompass elements both of dystrophic and metastatic calcification.[6] In the reported case, it would be of interest to know if there was any evidence of vascular calcification elsewhere in this individual, or whether it was localised to the retinal arterioles only.

  Secondly, Senior-Loken syndrome (renal-retinal syndrome, nephronophthisis associated with retinitis pigmentosa or retinal aplasia) is also germane to this discussion.[7] Nephronophthisis is a major cause of progressive medullary cystic renal disease leading to chronic renal failure in adolescents.[8] It is associated with a variable retinal phenotype, a reflection of both clinical and genetic heterogeneity. When associated with retinitis pigmentosa, there is an absence of the classic bone spicule pigmentation, but optic nerve head pallor and attenuation of the blood vessels is seen. Also, electroretinographic alterations have been reported in nephronophthisis despite normal a normal fundus examination.[9] In view of the phenotypic overlap seen with the reported case, results of electroretinography would be pertinent to the discussion. We would also advocate a molecular genetic analysis at the known loci associated with Senior-Loken syndrome, which could potentially reveal an interesting new allelic variant.

  Finally, whilst the fundus photograph elegantly demonstrates the stark changes seen in the retinal vasculature, this evidence could be further augmented with the addition of an ultrasound B-scan image demonstrating the pathognomonic echogenic features of intra-ocular calcification.

  Moin Mohamed
  Vision Research Group
  Molecular Medicine Unit
  University of Leeds

  Martin McKibbin
  Eye Clinic
  St James’s University Hospital
  Beckett Street
  Leeds

  References

  (1) Patel DV, Snead MP, and Satchi K. Retinal arteriolar calcification in a patient with chronic renal failure. Br J Ophthalmol 2002;86:1063

  (2) Juergens JL et al. Peripheral vascular diseases. 5th edition. Philadelphia: W Saunders. 1980. pp.238-240.

  (3) Lachman AS, Spray TL, Kerwin DM, Shugoll GI, Roberts WC. Medial calcinosis of Monckeberg. A review of the problem and a description of a patient with involvement of peripheral, visceral and coronary arteries. Am J Med 1977 Oct;63(4):615-22.

  (3) Castillo BV Jr, Torczynski E, Edward DP. Monckeberg's sclerosis in temporal artery biopsy specimens. Br J Ophthalmol 1999 Sep;83(9):1091-2.

  (4) Monckeberg's sclerosis: an unusual presentation. Top C, Cankir Z, Silit E, Yildirim S, Danaci M. Angiology 2002 Jul-Aug;53(4):483-6.

  (5) Byts' IuV, Holdobina OV, Dosenko VIe, Dudko MO, Larionova NA. The current concepts of the pathogenesis of Monckeberg-type arteriosclerosis. Fiziol Zh 2000;46(2):64-72.

  (6) Online Mendelian Inheritance in Man, OMIM (TM). Johns Hopkins University, Baltimore, MD. MIM Number: #266900: 6/10/2002. http://www.ncbi.nlm.nih.gov/omim/

  (7) Gusmano R, Ghiggeri GM, Caridi G. Nephronophthisis-medullary cystic disease: clinical and genetic aspects. J Nephrol 1998 Sep-Oct;11(5):224-8.

  (8) Orssaud C, Kleinknecht C, Habib R, Broyer M. Hereditary chorioretinal degeneration and nephronophthisis. The role of Senior-Loken syndrome. Ophtalmologie 1989 Sep-Dec;3(4):270-2.

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  Conflict of Interest:
  None declared.

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