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Monckeberg

  • 1.  Monckeberg

    Posted 05-03-2025 15:29
    Edited by Michel Van Lint 05-03-2025 15:35

    Just wanted to share this case as I have never seen a Monckeberg before

    Male 69 years-old

    Presents with acute loss of vision in his right eye.

    ESR 81 mm/h

    CRP 6.5

    Platelet count 398x10^3

    Fluorescein angiography appeared to show a delayed choroidal filling (bit unfortunate that the watershed zone is at the border of the image)

    Eye fundus revealed an optic disc edema of the right eye that occurred to be pale of color

    We thought of GCA, but it turned out to be Monckeberg medial calcific sclerosis according to the TAB

    In one of the previous emails here, it was mentioned that it could simulate GCA, but I had no idea it could also present with delayed choroidal filling

    Unfortunate that the watershed area is right at the border of the photo, but the delayed filling can be recognised



  • 2.  RE: Monckeberg

    Posted 05-03-2025 16:12





  • 3.  RE: Monckeberg

    Posted 05-03-2025 22:07
    I have seen Mönckeberg with GCA, and found that previously it has been reported in 6% of TAB samples. How does one rule out GCA completely if one finds only Mönckeberg , after all we have TAB negative GCA too.
    Thanks for sharing very interesting situations Dr Lint

    Best

    Dr Shikha Bassi
    Sankara Nethralaya 
    Chennai
    India





  • 4.  RE: Monckeberg

    Posted 05-03-2025 22:54
    Michel, I wouldn't assume that Monckeberg is responsible for the visual loss or the inflammatory markers. It is likely a coincidental finding that is usually asymptomatic and not inflammatory.

    Mark





  • 5.  RE: Monckeberg

    Posted 05-04-2025 00:31
    If the elastin stain shows disruption of the internal elastic membrane this is consistent with GCA even if no giant cells are seen within that segment of the biopsy. 






  • 6.  RE: Monckeberg

    Posted 05-04-2025 06:50
      |   view attached
    Attached find a photo where Monckeberg
    Simulated GCA and causes disruption of the inner elastic laminate; a published case







  • 7.  RE: Monckeberg

    Posted 05-04-2025 08:28
    Michael. Thanks for the reference. I used to work with a pathologist who would always call monckeberg in cases where there was any calcium noted in the section. I would then ask him to please report on the elastin stain as well. 

    Mitch Strominger. 






  • 8.  RE: Monckeberg

    Posted 05-04-2025 08:49
    Thank you, I am going to contact the histologist tomorrow.
    This was my first time a TAB report came back with Monckeberg...

    Michel





  • 9.  RE: Monckeberg

    Posted 05-04-2025 08:51
    Thank you. Yes, I understand better now....

    Michel





  • 10.  RE: Monckeberg

    Posted 05-04-2025 08:52
    Thanks, I am contacting the histologist tomorrow and if possible create new slides as well to be sure.

    Michel





  • 11.  RE: Monckeberg

    Posted 05-04-2025 22:06

    Hello Michael, et al.-

     

    Monkeberg's Medial Calcific Sclerosis is simply dystrophic calcification at the internal elastic lamina.  It is an isolated finding and has nothing to do with histologic identification of arteritis.  Severe calcification encircling the majority of the artery can be an indication of calciphylaxis.  Th bulkiness of the calcification can narrow the lumen and reduce blood flow.  It is for this reason that I always include medial calcific sclerosis in the path report if I see it, and comment on the degree of calcification if extensive.

     

    Breaks in the internal elastic lamina occur for many reasons, including hypertension, and are extremely common in elderly patients.  Intermittent breaks in the lamina does not signify arteritis.

     

    In order to histologically diagnose healed arteritis, criteria include a discontinuity of the internal elastic lamina for at least 180 degrees, along with disruption of the architecture of the intimal/medial interface in that region, suggesting damage due to a possible prior inflammatory event.

     

    I tell our residents that if the biopsy shows active or healed arteritis, you can, of course, accept a diagnosis of temporal arteritis.  A negative biopsy, however, does not exclude arteritis elsewhere. The biopsy is just a sample of a larger system.  This is true even if you take the accepted steps in analyzing the biopsy as described by Dan Albert years ago  (artery at least 2 cm long, cross-sectioned into 1 mm "barrels", at least 100 sections examined microscopically).  Dan showed this protocol statistically should include "skip areas"  of active inflammation separated by non-inflamed regions, thus making the histologic evaluation more valid.  The primary key to diagnosis is the clinical symptomatology, and perhaps the response to steroids.  Lab tests likewise, are supportive, but not indicative either for or against GCA.  And of course, the pathologist must be handling and examining the specimen appropriately (never on longitudinal sections of the artery).

     

    In the thousands of temporal artery biopsies I have examined, I have occasionally found cases with a giant cell or 2 attached to the calcification in Monkeberg's.  However this is just a foreign body reaction to the calcium, is not accompanied by any additional inflammation, and is not "giant cell arteritis".

     

    Thank you Claudia for also weighing in on this question.

     

    Best regards,

     

    Nick Hogan

    Depts of Ophthalmology and Pathology

    Director, Ocular Pathology Section

    UT Southwestern Medical Center

    Dallas, TX

     

     



    UT Southwestern

    Medical Center

    The future of medicine, today.






  • 12.  RE: Monckeberg

    Posted 05-05-2025 12:34


    The fundus photograph reveals pallid disc swelling in conjunction with the clear vessel sign.  (Pollock SC, Arnold AC, Miller NR; Acta Ophth 97(7), 2019).

     

    In an older individual, preservation of vascular clarity over the surface of an acutely swollen optic disc appears to be highly specific for GCA.  While the sensitivity of the sign has yet to be determined, it is not pathognomonic for arteritic AION, i.e., there are biopsy-confirmed cases that do not exhibit preservation of vascular clarity.  (An analogy with jaw claudication may be apropos  highly suggestive of GCA when present, but present only in a subgroup of GCA patients).

     

    Given:  a) the patient's age; b) the ESR level; c) the CRP level; d) photographic and angiographic documentation of a sectoral choroidal filling defect from 3:00 to 4:00; e) pallid optic disc swelling; and f) preservation of vascular clarity over the surface of the swollen disc, a diagnosis of arteritic AION appears to be likely.

     

    Careful reexamination of the TA biopsy specimen is indicated, ideally with multiple new sections.  In addition, it would be helpful to know:

     

    1)    The results of the clinical examination of the contralateral superficial temporal artery, esp. with respect to any enlargement, nodularity, tenderness, and/or pulselessness.

     

    2)    Whether or not the patient reported symptoms of GCA other than acute visual loss.

     

     

               Steve

     

    Stephen C. Pollock, MD

    ReplyForward






  • 13.  RE: Monckeberg

    Posted 05-05-2025 14:02
    Dear Stephen,

    Many thanks. With regards to GCA, I have come to grow rather stubborn and I rely more on the clinic than the TAB.
    Indeed, the pale optic disc edema, delayed choroidal filling, and otherwise unexplained ESR are all red flags to me.
    The TAB was negative and the PET-scan as well, which will give me a hard time convincing the rheumatologist, but I don't see my self tapering the steroids too soon and will keep an eye on the ESR/CRP.

    I asked for additional slides/sections based on the existing biopsy, but still wait for an answer.

    In the meantime, this case has been quite educational to me with regards to Monckeberg. Although I had heard of it, I had never seen it reported before, but now I know better....

    Kind regards,
    Michel








  • 14.  RE: Monckeberg

    Posted 05-05-2025 13:55
    Dear Nick,

    Many thanks to you as well. I also saved this e-mail.
    Thank you for this elaborate explanation.

    Kind regards,
    Michel





  • 15.  RE: Monckeberg

    Posted 05-04-2025 21:17
    Michael 

    I practice as an Ocular Pathologist and NeuroOphthalmologist 

    Monckeberg calcification is common in elderly patients especially with HTN.
    -Monckeberg has elastic lamina disruption oNLY on the area of calcification, the histiocytes are generally in the INNER side, meaning form the media -lumen towards the elastic lamina.

    Vasculitis has elastic lamina disruption, duplication and the histiocytic inflammation is form the OUTER to the inner-Elastic lamina
    -additionally, the histiocytes are located in the elastic lamina disruption and NOT in the area of calcification
    -vasculitis can ALSO have lymphocytic inflammation in the vasa vasorum (small vessels surrounding the vessels, in the adventitia and around)
    -EOSINOPHILS are usually also more visible in Vasculitis inflammation, and NOT in monckeberg 

    To make it a bit more challenging in your case... YES: Severe reduced blood flow(ischemia) may be secondary to severe calcification of the main arteries, but that is generally seen in peripheral arteries/limbs
    -I HAVE SEEN ONE CASE, with CKD and severe calcification of external and internal arteries of the head-temporal arteries and scalp, that presented with NAION with severe ischemia of the head/scalp but the Temporal Artery biopsy showed NO SIGNS of GCA
    ---because of CKD she did have very high ESR as well, making dx confusing

    Hope this helps

    Claudia Prospero Ponce








  • 16.  RE: Monckeberg

    Posted 05-05-2025 01:17
    Thank you very much for your input, Claudia. Very informative.

    Michael


    --

    Michael Paul M.D

    Eye diseases and Surgery

    Director Emeritus

    Ophthalmic Plastic and Reconstructive Surgery

       ,Orbital and Lacrimal Surgery Service 

    Neuro-Ophthalmology

    Dept  of Ophthalmology

    Edith Wolfson Medical Center

    Holon, Israel

    972-3-5049554

    Fax: 972-3-5018703


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  • 17.  RE: Monckeberg

    Posted 05-05-2025 13:51
    Dear Claudia,

    Thank you so much for this detailed explanation!
    This is quite informative and will save this email in a special folder for reference.

    Kind regards,
    Michel