We read with interest Dr Michael Wall's editorial on idiopathic intracranial hypertension (IIH) (1). We describe a surgically treatable case of IIH associated with obstructive sleep apnea (OSA).
A 9-year-old girl presented to our emergency department with complaints of diplopia, headaches, nausea, and vomiting for 5 days associated with mild neck stiffness. She was taking no medications. Her height was 62 inches, weight 159 lbs for a body mass index of 29, (over the 95th percentile for girls her age and height).
Visual acuity was 20/40, right eye and 20/25, left eye. Pupillary reactions, color vision, and confrontation visual fields were normal. Extra-ocular motility demonstrated limited abduction bilaterally. Funduscopic examination revealed mild bilateral optic disc swelling with dilated retinal vessels without hemorrhage or exudate. Neurological examination was normal. Automated visual fields performed 3 days later showed superior arcuate scotomas in each eye and a nasal step in the right eye.
Magnetic resonance imaging of the brain was normal as was magnetic resonance venography. Opening pressure on lumbar puncture was greater than 50 cm H2O, and cerebrospinal fluid analysis was normal. The patient was diagnosed with IIH and prescribed 500 mg of acetazolamide 4 times a day. This was eventually reduced to 500 mg twice a day. Her diplopia resolved, but 6 months later, she complained of intractable headaches and had worsening papilledema. Opening pressure on repeat lumbar puncture was 44 cm H2O.
At this point, the patient's mother reported that the child had OSA, which was diagnosed 2 years previously. Polysomnographic studies had demonstrated severe OSA with a respiratory disturbance index of 21 events per hour. The patient was irregularly using a continuous positive airway pressure machine.
On physical examination, the patient was found to have hypertrophic tonsils and adenoids with complete obstruction of her nasopharynx. She underwent adenotonsillectomy and postoperatively reported immediate relief of her headaches. At her last visit, she had complete resolution of all symptoms referable to IIH and subjective improvement in her sleeping pattern.
Recent studies have suggested a potential relationship between IIH and OSA (2–6). The nocturnal hypoxemia and hypercarbia present during apneic episodes cause cerebral vasodilation and secondary increased ICP (7). OSA may be a risk factor in the worsening of IIH, the importance of which is compounded by the fact that the 2 conditions frequently coexist in patients with obesity. Screening for papilledema is recommended in all patients with OSA who have visual symptoms (8,9). In children who are found to have OSA and IIH, airway obstruction caused by adenotonsillar enlargement should be considered. We are not aware of previously reported cases where the removal of adenotonsillar tissue led to significant improvement in OSA and resolution of intracranial hypertension.
1. Wall M. Idiopathic intracranial hypertension and the idiopathic intracranial Hypertension Treatment Trial. J Neuroophthalmol. 2013;33:1–3.
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8. Waller EA, Bendel RE, Kaplan J. Sleep disorders and the eye. Mayo Clin Proc. 2008;83:1251–1261.
9. Jindal M, Hiam L, Raman A, Rejali D. Idiopathic intracranial hypertension in otolaryngology. Eur Arch Otorhinolaryngol. 2009;266:803–806.