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Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure, visual disturbances, papilledema, and headache. There is an association between sex hormone excess and IIH, particularly in patients undergoing hormonal therapy for gender transition.
The increased androgen concentration during testosterone therapy for gender reassignment presents an opportunity for studying different treatment modalities. First-line treatment of IIH involves dietary changes and a carbonic anhydrase inhibitor such as acetazolamide. Surgical treatment options include shunts (ventriculoperitoneal [VP] or lumboperitoneal), optic nerve sheath fenestration (ONSF), and endovascular stenting.
Though both ONSF and VP shunting remain standards of care when medical therapy fails, there are little data on this procedural combination. We present the first patient to undergo a successful simultaneous ONSF and VP shunt treatment for IIH.
A 23-year-old morbidly obese (body mass index = 54 km/m2) female-to-male transgender patient on testosterone therapy with a history of migraine and asthma but no ocular history presented to the emergency department with worsening left-sided headache and blurry vision. Vital signs in the emergency department revealed temperature of 36.4°C, blood pressure of 146/102 mm Hg, pulse of 62, and respiratory rate of 14. The patient's headache, nausea, and photophobia began 3 weeks before presentation. The blurred vision OS started 2 weeks before presentation, whereas vision began to decline OD 2 days prior.
Ophthalmology was consulted to evaluate for optic disc edema. Uncorrected visual acuity was 20/400 OD and 20/300 OS with no improvement with pinhole in either eye. Pupils were equally round and reactive, with no evidence of relative afferent pupillary defect (RAPD). Intraocular pressures via Tono-Pen (Reichert Technologies, Depew, NY) were 23 mm Hg OD and 24 mm Hg OS. Motility examination revealed full motility OD and –4 abduction deficit OS. Visual fields in both eyes were constricted 360 degrees on confrontation testing. The patient could not identify the test plate during Ishihara color testing. Anterior segment examination was unremarkable bilaterally. Ophthalmoscopic examination revealed 360-degree edema of both optic nerves with obscuration of vessels, diffuse pallor, multiple peripapillary hemorrhages, and cotton wool spots; retinal vessels were tortuous with venous engorgement. Multiple flame hemorrhages were noted in the macula and periphery OU.
Neurology noted a normal neurologic examination aside from a left abduction deficit. Magnetic resonance imaging of the brain and orbits without contrast medium was only remarkable for flattening of the posterior globes and slight prominence of the distal aspect of both optic nerves (Fig. 1). Magnetic resonance angiography and venography of the head were without venous sinus thrombosis. Lumbar puncture yielded an opening pressure of 51 cm H2O, negative cerebrospinal fluid (CSF) cultures and Gram stain, and normal CSF protein, glucose, and cell count. Based on these findings, the patient was diagnosed with IIH, started on 500 mg acetazolamide, and given the extent of vision loss, underwent bilateral ONSF and VP shunt placement.
One week postoperatively, the patient's cranial nerve VI palsy improved, but horizontal diplopia at distance was noted. His headaches improved. Uncorrected visual acuity had improved to 20/60 OD and 20/100 OS, with pinhole to 20/70 OS, without RAPD. Motility examination was full OD with an abduction deficit OS. One month later, the patient's visual acuity improved to 20/30 OD and 20/40 OS with 1+ RAPD OD, residual –1 abduction deficit OS, continued 360-degree field constriction, and colors 8/14 OD and 10/14 OS. The patient was lost to follow-up.
Although the pathogenesis of IIH remains unknown, disruption of CSF circulation and metabolic or hormonal effects have been proposed.
Our patient presented with headache, visual loss, and left sixth cranial nerve palsy 5 months following initiation of testosterone treatment. Because of the imminent permanent visual loss, surgical treatment was pursued. Visual acuity, abduction, and field deficits improved at 1-month follow-up. Treatment for IIH is specific to patient presentation and depends on the severity and time course of visual loss and structural considerations. However, combined ONSF and VP shunting procedures present a promising treatment option for persistent vision-threatening IIH.
Footnotes and Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
What do transgender patients teach us about idiopathic intracranial hypertension?.