A Case of Idiopathic Intracranial Hypertension with Bilateral Transverse Sinus Stenosis Treated with Balloon Angioplasty
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.280636 PMID: 32189697
Source of Support: None, Conflict of Interest: None
A 20-year-old-female presented with headache and blurred vision for 15 days, following 2-3 days of fever. Magnetic resonance imaging (MRI) of the brain showed tortuous optic nerves with a flattened optic cup and partial empty sella suggestive of idiopathic intracranial hypertension (IIH) [Figure 1]a and [Figure 1]b. No other lesion or meningeal enhancement was seen. MR venogram (MRV) suggested bilateral transverse sinus stenosis [Figure 1]c. On examination, she had gross visual impairment. She could only appreciate hand movements and had grade 4 papilledema of both eyes [Figure 1]d and [Figure 1]e. After lumbar puncture, CSF opening pressure was high (21 mmHg) with normal composition (protein 32 mg/dl, glucose 92 mg/dl, 2 cells/cmm- all lymphocytes, ADA 3.2 u/L).
She was started on six 250 mg tablets of Acetazolamide hourly without significant improvement of symptoms. Digital subtraction angiography (DSA) was done, venous phase was suggestive of stenosis of bilateral transverse-sigmoid junction [Figure 2]a and [Figure 2]b with very high pressure gradient around the stenotic segment (Right side 49 mmHg, Left 46 mm Hg).
Balloon angioplasty was done serially with 5 mm and 7 mm balloons, respectively [Figure 2]c, [Figure 2]d, [Figure 2]e. Then approximately 30 ml cerebrospinal fluid (CSF) was drained consecutively over the following three days. Within seven days, her vision improved (Right eye 6/9, Left 6/24) with partial improvement of papilledema (Grade 2). She was discharged with Tab acetazolamide and antiplatelet drugs. On follow-up visit after three weeks, her headache fully improved with vision 6/9 in her right eye and 6/24 in her left eye. Fundoscopy suggested mild secondary optic atrophy (right<left) with normal CSF pressure. Antiplatelets were stopped. MRI with MRV was repeated which showed improvement of imaging findings and transverse sinus stenosis as compared to the previous scans [Figure 3]a, [Figure 3]b, [Figure 3]c. Subsequently, DSA also showed improvement of Transverse sinus stenosis. On follow-up visit after 8 months, further improvement of vision was observed (bilaterally - 6/9) and with complete reduction of papilledema [Figure 3]d and [Figure 3]e.
Impaired venous sinus drainage due to transverse or upper sigmoid stenosis has become the focus of attention for the possible cause and target for management of medically refractory cases of IIH. A pressure gradient of >10 mm Hg has been used in several studies to decide the indication for sinus stenting. This case illustrates the use of balloon angioplasty only in IIH – to elucidate the possible role of less invasive procedures, to minimize the need of intravascular metallc device placements and decrease oral/anticoagulant/antiplatelet use in such cases. Though angioplasty has resulted in dramatic improvement of symptoms in several studies, to our knowledge, angioplasty only in absence of stenting has only been used in a few cases ,, as described in brief in [Table 1]. In one such case, angioplasty was only done as a result of stent failure.
Our patient was a young female who presented with fulminant IIH which is defined as (i) the acute onset (<4 weeks) of symptoms and signs of intracranial hypertension and (ii) rapid worsening of visual loss over few days. After confirming venous sinus stenosis on imaging and demonstrating a high-pressure gradient of >50 mm Hg across the stenotic segments, balloon dilatation of the stenosed segments were done. This dramatically improved the patient's symptoms immediately and the pressure gradient was abolished. Though stenting was planned, and she was loaded with oral antiplatelet agents, the patient was reluctant for stenting, and hence it was decided to give her trial of angioplasty only.
On follow-up, her symptoms improved significantly and no re-stenosis of the transverse sinuses was observed. After angioplasty she was observed for any worsening of headache/vision or any recurrence of venous stenosis so that stenting can be planned, if required. As she was stable, clinically and radiologically, stenting was not done.
To conclude, balloon Angioplasty can be tried in IIH with venous sinus stenosis, as it is less invasive than shunting, optic nerve fenestration or even venous sinus stenting, and avoids the need of prolonged anticoagulation/antiplatelet use. However, long-term follow up and larger number of cases are required to prove its efficacy.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3]