gms | German Medical Science

Symposium Idiopathic Intracranial Hypertension (Pseudotumor cerebri)

07.10.2017, Düsseldorf

Coagulation and venous stent, thrombosis and bleeding risk

Meeting Abstract

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  • Rainer Zotz - Institut für Laboratoriumsmedizin, Blutgerinnungsstörungen und Transfusionsmedizin, Düsseldorf

Symposium Idiopathic Intracranial Hypertension (Pseudotumor cerebri). Düsseldorf, 07.-07.10.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. Doc17siih10

doi: 10.3205/17siih10, urn:nbn:de:0183-17siih101

This is the English version of the article.
The German version can be found at: http://www.egms.de/de/meetings/siih2017/17siih10.shtml

Published: November 30, 2017

© 2017 Zotz.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.


Outline

Text

Idiopathic intracerebral hypertension (IIH) and cerebral venous sinus thrombosis can lead to impaired vision, which is why the initial suspected diagnosis is often made by an ophthalmologist. The risk constellations of these two disorders overlap with respect to certain risk factors: 1. premenopausal women; 2. female hormone intake; 3. thrombophilia. Based on these similarities, one can suspect a common pathophysiological pathway.

Risk factors for IIH are obesity (gynecoid form/lower body) or weight gain without obesity, female sex before menopause, polycystic ovary syndrome, drugs (estrogens, levonorgestrel, tamoxifen, tetracyclines, etc.), sleep apnea and thrombophilia. This risk constellation suggests a pathophysiology which works via hormonal changes in obesity and polycystic ovary syndrome, as well as potential venous microthromboses in thrombophilic patients. Significant thrombophilic risk factors which also increase the risk of IIH include lupusanticoagulant, increased factor VIII activity, PAI-1, increased lipoprotein(a), deficiencies of proteins C or S, antithrombin deficiency, and the MTHFR mutation.

In management of IIH, a stepwise procedure is recommended. In the acute setting, the focus should lie on a quick decrease of intracranial pressure or a reduction of papilledema via lumbar puncture in order to prevent vision loss. Optic nerve sheath fenestration leads to a permanent stabilization in almost 90% of surgically treated optic nerves; this procedure is recommended in patients with papilledema and strong visual reduction and with no or weak headaches. Internal cerebrospinal fluid diversion via shunts can be used when trying to achieve a more permanent normalization of intracranial pressure; lumboperitoneal shunts should be preferred, possibly with position-dependent gravitational valves in order to prevent overdrainage. This procedure is recommended in patients with papilledema, visual reduction and headaches. If fibrous (postthrombotic) changes or arachnoid granulations hinder venous drainage and a pressure gradient is measurable near the stenosis, endovascular stent angioplasty may represent an advisable alternative to cerebrospinal fluid diversion; however, this procedure may be subject to severe, if rare, complications. Success rates differ. In most studies, patients were administered dual antiplatelet therapy with ASS 325 mg and clopidogrel 75 mg over 3-6 months. Afterwards, patients were switched to ASS monotherapy (we recommend ASS 100 mg/die). In the periinterventional setting, unfractionated heparin (ca. 70 E/kg body weight) was used in addition. Due to venous flow conditions, we recommend continuing anticoagulation with low-molecular-weight heparin (e.g., LMWH 1x100 E/kg body weight/die) over 2-3 months until endothelization of the stents is complete. As of now, there are no randomized studies investigating this issue. Both the dual antiplatelet therapy and heparin increase the risk of bleeding.

Taking into account shared risk factors of idiopathic intracerebral hypertension (IIH) and cerebral sinus venous thrombosis, it may be suspected that both disorders also share pathophysiological mechanisms; this may include the presence of small, undiscovered cerebral thromboses or microthromboses in IIH. This could explain the constellation of thrombophilic risk factors typical of IIH analogous to that of cerebral sinus venous thrombosis, including the well-established interaction between estrogens and thrombophilic risk factors.


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