Spontaneous hemorrhage after external ventricular drain placement in the setting of low factor VII secondary to liver cirrhosis
- Department of Neurosurgery, Brigham and Women’s Hospital, Boston, Massachusetts, United States.
- Department of Neurology, Brigham and Women’s Hospital, Boston, Massachusetts, United States.
- Department of Medicine, Brigham and Women’s Hospital, Boston, Massachusetts, United States.
Melissa Ming Jie Chua
Department of Neurology, Brigham and Women’s Hospital, Boston, Massachusetts, United States.
DOI:10.25259/SNI_446_2020Copyright: © 2020 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Melissa Ming Jie Chua1, Alvin S. Das2, Julie Aurore Losman3, Nirav J. Patel1, Saef Izzy2. Spontaneous hemorrhage after external ventricular drain placement in the setting of low factor VII secondary to liver cirrhosis. 25-Nov-2020;11:403
How to cite this URL: Melissa Ming Jie Chua1, Alvin S. Das2, Julie Aurore Losman3, Nirav J. Patel1, Saef Izzy2. Spontaneous hemorrhage after external ventricular drain placement in the setting of low factor VII secondary to liver cirrhosis. 25-Nov-2020;11:403. Available from: https://surgicalneurologyint.com/surgicalint-articles/10408/
Background: Alterations in normal coagulation and hemostasis are critical issues that require special attention in the neurosurgical patient. These disorders pose unique challenges in the management of these patients who often have concurrent acute ischemic and hemorrhagic injuries. Although neurosurgical intervention in such cases may be unavoidable and potentially life-saving, these patients should be closely observed after instrumentation.
Case Description: A 57-year-old male with liver cirrhosis secondary to amyloid light-chain amyloidosis was admitted to the intensive care unit for the management of delayed hydrocephalus. An external ventricular drain (EVD) was placed for the treatment and monitoring of hydrocephalus. Five days after EVD placement, a head computed tomography scan revealed a tract hemorrhage. However, on repeated imaging, the size of the hemorrhage continued to increase despite aggressive blood pressure control and several doses of phytonadione. Extensive coagulopathy workup was remarkable for low factor VII levels. In that setting, recombinant activated factor VII was administered to normalize factor VII levels, and the tract hemorrhage stabilized.
Conclusion: To the best of our knowledge, this is the first case of spontaneous hemorrhage after EVD placement in the setting of liver cirrhosis-associated factor VII deficiency. Our case highlights the importance of identifying coagulation disorders in neurosurgical patients at high risk for coagulopathy and closely monitoring them postoperatively.
Keywords: Coagulopathy, Factor VII, Liver cirrhosis
Hemostasis is particularly important in neurosurgical patients as minor abnormalities can pose significant bleeding risks leading to worsened morbidity and mortality. As such, coagulopathy disorders should be diagnosed and managed appropriately, especially after instrumentation. Common causes of acquired coagulopathies in neurosurgical patients include antithrombotic use, thrombocytopenia, sepsis, disseminated intravascular coagulation, uremia, and liver disease.[
A 57-year-old Caucasian male with atrial fibrillation (not on anticoagulation), hypertension, monoclonal gammopathy of undetermined significance, and AL amyloidosis with known liver involvement leading to cirrhosis initially presented to his local emergency department with right-sided weakness and headache. His blood pressure on presentation was 164/78 mmHg, and he was found to have a left parietal intraparenchymal hemorrhage and an incidental right parietal arteriovenous malformation [
He was readmitted 1 month later with severe headaches and worsening right-sided weakness (3-/5 strength) with a blood pressure of 148/73 mmHg. A head computed tomography (CT) revealed an increase in size of the left parietal hemorrhage. At that time, he also experienced epistaxis as well as catheter site bleeding that resolved spontaneously. Laboratory workup showed a stable platelet count of 127 × 109/L, normal factor X activity (338%), normal von Willebrand factor activity (736%), and a mildly elevated international normalized ratio (INR) of 1.4 for which he was given intravenous (IV) Vitamin K (phytonadione). Interval imaging demonstrated stability of the intraparenchymal hemorrhage; however, due to concern for seizures and altered mental status, he had a prolonged hospitalization on the neurology service. He was subsequently discharged back to rehabilitation with an antiepileptic drug regimen of levetiracetam (2000 mg twice daily), clobazam (20 mg twice daily), and lacosamide (50 mg daily).
The patient was readmitted 2 months later to our institution for worsening encephalopathy. A head CT showed a mild increase in ventricular size in addition to the resolving left parietal intraparenchymal hemorrhage. An electroencephalogram and extensive metabolic and infectious workup were unremarkable. Given the possibility of delayed hydrocephalus as explanation for his worsening encephalopathy, the patient was trialed on cerebrospinal fluid diversion with an EVD [
Noncontrast head computed tomography scans, axial views. (a) Immediately post-external ventricular drain (EVD) placement. (b) Spontaneous tract hemorrhage 5 days post-EVD placement. Interval enlargement of the EVD tract hemorrhage with 6 days (c) and 8 days. (d) Post-EVD placement shown here. (e) Recombinant factor VII was given on post-EVD placement day 8 with stabilization of the tract hemorrhage on day 9. (f) Acute development of the right-sided subdural hemorrhage 10-day post-EVD placement.
We report a rare case of worsening hemorrhage almost 1 week after EVD placement in the setting of an acquired VII deficiency from liver cirrhosis secondary to AL amyloidosis. Liver cirrhosis is a known cause of coagulopathy which can complicate the postoperative management of a neurosurgical patient. Studies have shown up to 2.4% incidence of major bleeding after invasive procedures in cirrhotic patients.[
Liver disease can often lead to low platelet counts and platelet dysfunction.[
rFVIIa is only approved for replacement therapy in congenital factor VII deficiency where the recommended dose is 15–30 mcg/kg every 4–6 h until hemostasis is attained.[
Randomized clinical trials using rFVIIa in patients presenting with intracranial hemorrhage have yielded mixed results. In one study of anticoagulant-related intracranial hemorrhage, rFVIIa was effective in normalizing INR without producing any thromboembolic complications.[
Our case highlights the importance of identifying coagulation disorders in neurosurgical patients at high risk for coagulopathy and closely monitoring them postoperatively. Particularly in cases where coagulopathies are unable to be reversed, there should be a low threshold to check coagulation factor levels including factor VII. In cases where correction of low factor VII levels do not achieve adequate hemostasis, aminocaproic acid should be considered as it was shown to be effective in our patient. To the best of our knowledge, this is the first case of spontaneous hemorrhage after EVD placement in the setting of liver cirrhosis-associated factor VII deficiency.
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