Grand Rounds

From Headache to IIH: A Pediatric Case with an Unexpected Twist

Sneha Rani¹, Akanksha Sancheti¹, Hannah Becker¹, Mayra Matar², Neha Bhagi^2
1Rush Medical College, Chicago, United States, ²John H. Stroger, Jr. Hospital of Cook County, Chicago, United States

Address for Correspondence: 2050 W Ogden Ave Apt 304, Chicago, 60612, United States. Email: sneha_anand@rush.edu

Keywords: Pediatric Neurology, Increased Intracranial Hypertension, Visual Disturbances, Migraine.

Clinical Problem:
A previously healthy 11-year-old male from Venezuela presented to the emergency department with a two-week history of increasing headaches, nausea, vomiting and new-onset visual disturbances, mimicking migraine headaches. Initially had a good response to ibuprofen but later worsened, accompanied by photophobia, blurry vision and perception of "darkness" and "shadows."
Diagnostic Workup Given worsening symptoms, the clinical team pursued imaging and laboratory workup to rule out intracranial pathology, including:
CT Head without Contrast: Short segment of focal asymmetric expansion and hyperdensity of the middle portion of the superior sagittal sinus. Findings may represent intrinsic hyperdensity of the sinus secondary to elevated hematocrit, although dural venous sinus thrombosis would appear similar in the correct clinical setting. No areas of altered brain parenchymal density.

CT Head with Contrast:
1. No CT venogram evidence of dural venous sinus thrombosis.
2. Incidentally noted, prominent bilateral optic nerve sheath complexes and optic discs. Correlate clinically for possible idiopathic intracranial hypertension with CSF opening pressure.
3. Partially visualized, moderate enlargement and striated enhancement of the nasopharyngeal soft tissues, likely secondary to an infectious/inflammatory process.

MRI Brain with and without Contrast, MRI MRV Brain, MRI Orbit with and without Contrast:
1. Left frontal cerebral deep white matter small T2/FLAIR hyperintense focus. Bilateral frontal and occipital periventricular cerebral white matter confluent subtle T2/FLAIR hyperintensities reflect gliosis, nonspecific. No focus on abnormal restricted diffusion or contrast enhancement within the brain to suggest an active demyelinating process.
2. Unremarkable brain MRV. No evidence of dural venous sinus thrombosis.
3. Redemonstrated prominent bilateral optic nerve sheath complexes with prominent subarachnoid spaces surrounding the optic nerves associated with mild enlargement and contrast enhancement of the optic discs, greater on the left. There is also partial empty sella. The constellation of imaging findings is concerning for idiopathic intracranial hypertension associated with bilateral papilledema vs bilateral optic papillitis.

Lumbar puncture: A lumbar puncture with manometry was done to directly measure the intracranial pressure. It revealed a significantly elevated opening pressure of 36+ cm H2O. The CSF analysis: cytology, cell count and culture revealed no anomalies. There were no signs of infection or inflammation. A repeat LP showed improvement to 25+ cm H2O.

What is the differential diagnosis for pediatric patients presenting with headache and visual disturbances and how can serious intracranial pathology be distinguished from benign conditions?