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Endovascular Stenting in a Transgender Patient With Idiopathic Intracranial Hypertension.

I diopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure (ICP) of unknown etiology. Common presenting symptoms include headache, transient obscuration of vision (TOV), diplopia, and pulsatile tinnitus (1). Transverse venous sinus stenosis may be observed on magnetic resonance venography (MRV) in 30%–93% of IIH cases (2). In cases where focal venous sinus stenosis is observed with a cerebral venous pressure gradient .8 mm Hg, endovascular stenting is often considered a viable treatment option (2). Most IIH cases occur in overweight women between 20 and 40 years of age (1). With the advent of testosterone therapy in gender reassignment, there have been cases of IIH occurring in karyotypically female patients during female-to-male gender reassignment. Most of these patients were successfully treated with medical management, but none have been treated with endovascular stenting (3). In this report, we present a patient diagnosed with IIH while undergoing female-to-male gender reassignment who was successfully managed with endovascular stenting. A 36-year-old patient (karyotypically 46XX) undergoing female-to-male reassignment was referred to neuroophthalmology for a 6-month history of constricting visual fields, TOVs lasting seconds, headache, dizziness, and “ear popping.” Three months before his neuroophthalmology visit, he was found to have papilledema and the opening pressure was 21 cm H2O on lumbar puncture. At our initial evaluation, he was taking acetazolamide 1 g/day. His hormone therapy consisted of testosterone cypionate 100 mg injected intramuscularly every week for 50 months before symptom onset. His body mass index was 25 kgm2. Visual acuities were 20/20 in each eye, with normal pupillary testing and full extraocular movements. There was bilateral papilledema, more marked in the left eye. Whereas the right visual field was full, there was a nasal field loss in the left eye. MRV indicated mild narrowing of the left transverse sinus and a partially occluded right transverse sinus (Fig. 1A). Optical coherence tomography (OCT) indicated retinal nerve fiber layer (RNFL) measurements of 91 mm in the right eye and 140 mm in the left eye. His IIH medications were changed to daily acetazolamide 1.5 g, furosemide 20 mg, and topiramate 25 mg. This led to slight decrease in headaches, some improvement in papilledema but no improvement in visual field testing. The patient was referred to neuroradiology, and central venous pressure gradients (CVPG) across transverse sinuses were 13.5 mm Hg bilaterally. Due to lack of improvement after 5 months of medical management and bilaterally elevated CVPG, he underwent endovascular stenting. The right transverse sinus appeared slightly dominant compared to the left with narrowing at the distal aspects of both transverse sinuses (Fig. 1B). Before stent placement, average pressure gradient across the torcula and right sigmoid sinus was 12.7 mm Hg based on 3 separate measurements. After stent placement, average pressure gradient was 2.7 mm Hg. The internal carotid artery angiogram indicated proper positioning of a widely patent stent at the distal aspect of the right transverse sinus without residual stenosis (Fig. 1C). One month later, the patient reported resolution of headaches and TOVs. OCT revealed some residual RNFL thinning (84 mm in the right eye and 77 mm in the left eye). At 6-month follow-up, the patient was doing well without headaches, his papilledema had resolved, and automated visual fields were full. MRV indicated that the stent remained patent. Our transgender patient undergoing female-to-male reassignment with testosterone therapy developed IIH. Previously reported IIH cases in transgender patients were all female-to-male reassignment and, like ours, all patients received testosterone therapy (3). In 3 cases, patients were successfully managed with medical treatment by altering testosterone therapy and adding acetazolamide. One patient achieved remission with optic nerve sheath fenestration, and another had an emergent lumboperitoneal shunt (3). Unlike previous reports, our patient had transverse sinus stenosis Doheny Eye Institute (KK, RK, AAS), Los Angeles, California; Huntington Memorial Hospital (AK), Pasadena, California; Department of Ophthalmology (RK, AAS), Doheny Eye Center, David Geffen School of Medicine at UCLA, Los Angeles, California; The Ottawa Eye Institute (RK), University of Ottawa, Ottawa, Canada; and Ottawa Hospital Research Institute (RK), Ottawa, Canada.