Case Presentation: A 34-year-old morbidly obese man with a history of recurrent headaches was admitted to the hospital with two months of rapidly progressive bilateral vision loss, weight gain, and lower extremities (LE) and scrotal swelling. An eye exam revealed severe papilledema in both eyes. Labs revealed brain natriuretic peptide 588 pg/ml and chest X-ray showed vascular congestion. The patient was admitted to the ICU with acute hypoxic and hypercapnic respiratory failure from HF requiring intravenous diuresis and non-invasive ventilation. An echocardiogram revealed biventricular (BiV) dysfunction with mild pulmonary hypertension. CT head showed small ventricles and a small pituitary gland for age raising suspicion for IIH. The diagnosis was confirmed by a lumbar puncture with opening pressure > 60 cm. Acetazolamide was started, a ventriculoperitoneal shunt (VP) was placed and the patient was discharged with treatment for new BiV failure. Later, cardiac catheterization showed normal coronaries, mildly elevated wedge pressure, and compensated HF.
Discussion: Fulminant IIH is defined as intracranial hypertension with no secondary cause, severe vision loss within 4 weeks of symptom onset, and progressive vision loss over days, which can lead to blindness (1). The patient presented with two life-threatening conditions: fulminant IIH and respiratory failure due to new HF. The treatment of IIH can only be initiated after successful management of symptoms of decompensated HF. Vision loss, LE edema, and scrotal edema in our patient developed over several weeks, during which the leading outpatient focus had been primarily vision loss and weight gain. Two weeks of diuresis were required to optimize the patient’s volume status prior to the consideration of VP shunt placement in our case. Adderley et al., in a 2019 cohort study in the United Kingdom found that IIH in women, independent of obesity, is associated with a 2-fold increase in cardiovascular disease (CVD) (2). The elevated CVD risk in patients with IIH is beyond that expected from obesity suggesting additional underlying systemic metabolic factors unique to IIH, including that of centripetal fat distribution as a cause of elevated intracranial pressures (3) as well as the unique profile of androgen excess affecting cerebrospinal fluid dynamics (4). We believe that IIH can predispose HF in both women and men.
Conclusions: The incidence of IIH is rising annually. An increased CVD risk has been demonstrated in patients with IIH. We think that for every patient with suspected IIH, it will be advantageous to evaluate CVD risk and initiate management if required. For those with rapidly progressive visual loss, surgical intervention is essential to save visual sight and can only be done once the patient’s CVD risk factors are optimized.