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IIH, 7 weeks pregnant and Arnold-Chiari

  • 1.  IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago

    Hello,

    I have a 34-year-old non-obese patient with IIH that used to be well-controlled.

    However, there is a recurrence of papilledema and it turns out she is 7 weeks into her pregnancy for which she stopped Diamox.

    The optic disc edema is more than 300 µm in thickness (Frisen grade 3-4).

    Since she has an Arnold-Chiari malformation, lumbar puncture is not possible.

    The neurosurgeon prefers to avoid surgery and she is in her first trimester of her pregnancy which makes Diamox less straightforward.

    What would you do?

    Kind regards,

    Michel



  • 2.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago
    I think she can wait till 12 weeks and then take diamox 
    With frequent Visual Field follow up 
    Because topiramate is not also permitted in the First trimester 





  • 3.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago
    It depends on what the field looks like:
    If it is normal you can do close follow-up

    Our neurologists like serial LP in pregnancy. Is it a true chiari? How much herniatian? Many IIH patients have low lying tonsils from the IIH without having a true chiari. Cervical puncture can be done in Chiari but that seems high risk for serial procedure 

    Loop diuretic is probably lowest risk, torsemide has the lowest risk according to US FDA pregnancy classification at category B but I think the efficacy of loop diuretics is less and carries the risk of hypokalemia, renal hypoperfusion and renal impairment, and oligohydramnios.

    Diamox is actually low risk in pregnancy and many patients take it during the first trimester because they do not know they are pregnant, retrospective studies have not identified a significant birth defect risk.

    Other than shunt, ONSF would be an option although presumably this would expose the patient to two anesthetic episodes instead of one for shunting, but if you think the patient has progressive vision loss and you can't convince NSGY to operate it remains an option.

    I'm not a fan of venous sinus stenting in pregnancy due to the need for dual anti platelet and risk of hemorrhaging.

    I generally prefer diamox for patients with vision threatening papilledema in pregnancy and have a more tolerant goal of trying to reduce papilledema to grade 2 rather than 0.

    These situations necessitate team approach and shared decision making with the patient ultimately making the decision that fits best with their personal risk tolerance and careful monitoring.

    Best of luck,

    Drew



    Original Message


  • 4.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago
    Consider careful follow up and reduced weight gain. Some literature implies reduced risk of weight gain in pregnancy. 




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  • 5.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago

    Dear all, 

    Many thanks for your advice. Also a good reminder that a partial reduction in the optic disc edema may be good enough.

    I will try to see if I can get through the first trimester with watchful waiting and then start acetazolamide.

    Visual field was fine until recently, but I will monitor her closely to see if that remains the case.

    Our neurologist also suggested the use of acetazolamide based on a publication in Continuum written by Dr. Friedman!

    Michel

    Micbhel


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  • 6.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago
    Again, there is no evidence that Diamox is threatening to fetal malformation even in the first trimester.
    Scott Forman, MD
    I have personally managed over half a dozen patients who took Diamox through there entire pregnancy with no problems. 





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  • 7.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago
    I will say my high risk OB service who followed such patients with me were not concerned about the teratogenic warnings. They monitored amniotic fluid more carefully but I never had a patient need to be induced for oligohydramnios



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  • 8.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 6 days ago
    This is what I tell the moms with IIH who have Grade 3 or worse, or field loss, or a lot of symptoms:
    Benefit should outweigh risk

    AU TGA pregnancy category: B3
    US FDA pregnancy category: C
    Animal studies have shown teratogenicity at doses in excess of 10 times the recommended dose in humans. There is no evidence of a direct association between this drug and birth defects during human pregnancy. In the Collaborative Perinatal Project (CCP), a retrospective study that evaluated 50,282 mother-child pairs, 12 had first trimester exposure to this drug with no anomalies observed. A total of 1024 anytime exposures were reported and the number of infants with malformations was less than expected (18 vs 18.06). There are no adequate and well-controlled studies in pregnancy women.




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  • 9.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 4 days ago
    Really helpful, thank you

    Daniel Schwartz MD

    Attending Neurologist/Neuro-Ophthalmologist
    Westchester Medical Center

    Assistant Professor of Neurology and Ophthalmology
    New York Medical College

    100 Woods Road, Taylor Pavilion, E104
    Valhalla NY 10595




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  • 10.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 2 days ago
    If VFs normal? then follow conservatively.  If not, then restart the Diamox.  It is safe in pregnancy.

    Robert




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  • 11.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 2 days ago
    Just one notice after the first trimester, if you are going to give a good dose of acetazolamide it is advised that you do frequent foetal ultrasound because it was reported , not frequently that a loading dose of acetazolamide may decreased fluid around the foetus and at least in two cases that I was following up it caused some foetal distress that required only lowering the dose not stopping the drug 



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  • 12.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted yesterday

    Thank you very much for this warning! I will aim to reduce the edema to reduce the worst of it, but not necessarily try to have it resolve completely. Grade 2 should be safe.

    Michel


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  • 13.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted yesterday

    Yes, this is what I hope to do.

    If anything happens to the baby, then the parents might blame me. Even if it would be unrelated to the Diamox, there is obviously no study on humans.

    If anything happens to her eyesight (probably the greater risk at this point), then I am to blame without question. I have a low threshold for starting acetazolamide in this case.

    Michel


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  • 14.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted yesterday

    This is very useful, many thanks

    Michel


    Original Message


  • 15.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 8 days ago
    Giver her Diamox. There is no evidence of fetal malformation and Neuro-ophthalmologists have always supported its use throughout all trimesters, 





  • 16.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 7 days ago
    How are her visual fields as they will guide the management along with symptoms of elevated ICP.

    Matt



    Sent from my Galaxy






  • 17.  RE: IIH, 7 weeks pregnant and Arnold-Chiari

    Posted 4 days ago

    Many thanks everyone for your clear answers. Today, her visual fields are still fine and the ganglion cell layer shows no thinning. I will follow her closely, but the patient agrees to start the Diamox in case of further doubt or deterioration.

    Michel


    Original Message


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