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A Severe Case of Vision Threatening Papilledema due to Idiopathic Intracranial Hypertension

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Title: A Severe Case of Vision Threatening Papilledema due to Idiopathic Intracranial Hypertension

Author: Robert Henseler, 4th Year Medical Student, Rutgers University – New Jersey Medical School

CC: Headache and Blurry Vision

HPI: A 20 y/o obese woman presents with a 2-week history of blurry vision and headaches. She was originally diagnosed with a urinary tract infection and prescribed cefuroxime. After seeing her regular physician, she was referred to an ophthalmologist for her visual symptoms where optic nerve swelling on exam and visual field defects on Humphrey Visual Field (HVF) testing were detected. She also complained of neck pain and photophobia so she was sent to the emergency room to rule out the possibility of meningitis. Ophthalmology was consulted and grade 4 disc edema was noted, showing hemorrhages off the discs, tortuous vasculature, and few macular and peripheral hemorrhages. She also had visual field defects on confrontation testing, a left RAPD, and acuity of 20/100 OD and 20/80 OS. A CT and MRI were performed which suggested increased intracranial pressure. A lumbar puncture (LP) was then performed with opening pressure of 56cm H20. At the time of her first LP, 32ml of CSF were removed and closing pressure was 7cm H20. Patient had some resolution of headache and reported slightly improved vision. Her symptoms returned the next day and a repeat LP done the with 30ml of fluid removed.

Testing During Admission:

LP in Lateral Decubitus Position:

MRI Orbits:

MRI Brain:

CTV:

The patient was then seen in the neuro-ophthalmology clinic for evaluation.

Neuro-Ophthalmologic History, Exam, and Testing Obtained Day After Admission:

Headache History:

Weight History:

IIH Associations:

Exam:

Hospital Course:

Patient continued to stay in the Neuro Critical Care Unit. A lumbar drain was placed by neurosurgery after her visit to the ophthalmology clinic. Neuro-ophthalmology continued to follow the patient and it was decided that if she did not have resolution of increased ICP and vision threatening papilledema then a bilateral nerve sheath fenestration would be performed by oculoplastic surgery. Due to lack of improvement the patient underwent surgery on hospital day 3. Her surgery was performed successfully with no complications. Neuro-ophthalmology continued to follow the patient during her stay at the hospital and then manage her care following discharge on hospital day 4. She was discharged on acetazolamide 1000 mg BID to lower CSF production, gabapentin for acetazolamide induced peripheral neuropathy, and hydrocodone/acetaminophen 10/325 for pain.

4 Days after Discharge:

Interval History:

Exam:

Plan:

12 Days after Discharge:

Interval History:

Exam:

Plan:

18 Days after Discharge:

Interval History: 

Exam:

Plan:

Discussion of Case:

This was a sudden onset severe case of IIH where many specialties were involved in trying to prevent papilledema induced vision loss. The important aspects of care after admission to the hospital were quick imaging (MRI of the Orbits, Brain and CT Venogram), LP in lateral decubitus, and ophthalmology consultation, examination, and HVF testing. The initially differential diagnosis for increased ICP could include a multitude of pathologic processes.

Differential Diagnosis of Papilledema and Increased ICP:

The MRI of the orbits, brain, and spine are used to assess for many of these causes as well as the ability to show signs of increased ICP. It is also important to rule out brain herniation which would be a contraindication to LP. The CTV was performed to rule out venous sinus thrombosis. The LP in lateral decubitus position is then used to assess the opening pressure. It is also important to test for infectious causes and meningitis. Quick ophthalmologic consultation is also vital to look at the severity of papilledema and guide management. HVF testing was used to track visual field deficits resulting from papilledema and OCT was used to measure and follow the amount of optic nerve swelling. After all the testing, as well as obtaining a thorough history, the diagnosis of IIH was made with weight gain and obesity as the most likely etiology.

The rapid progression and severity of symptoms in the patient’s clinical course led to more aggressive treatment of her vision threatening papilledema. During her first 2 days in the hospital she had two LPs performed and a drain placed to remove fluid and control pressure. The decision for bilateral optic nerve sheath fenestration—while controversial due to risk of damage to the optic nerve—can be safely performed by a well-trained oculoplastic surgeon and provide significant relief to the optic nerves. Performing optic nerve sheath fenestrations unilaterally is more common. During the case, discussions were made about how best to perform optic nerve sheath fenestration while a patient has a drain. Having fluid surrounding the optic nerve allows for easier and safer surgery but increases the pressure and can cause more damage to the optic nerve. It was decided that moving forward it is best to place the drain at a height so as to maintain a pressure of 20 cmH20 a few hours prior to surgery thus allowing safe surgical approach while minimizing the risk of progressive visual damage.

Since the patient continued to have severe symptoms of increased ICP as well as minimal improvement of OCT and HVF the dose of her acetazolamide was increased to 2000mg BID at day 12 post D/C. This is a large dose and the patient was unable to tolerate the adverse effects of the medication and still continued to have symptoms of increased ICP. While not usually necessary to control optic nerve swelling and IIH symptoms, the decision was made to place a CSF shunt. This was a severe and rapid case of IIH. Aggressive medical and surgical treatment was used in order to minimize optic nerve swelling and symptoms of increased ICP. In summary, severe visual symptoms should always be addressed rapidly by an ophthalmologist as delay can lead to permanent visual deficits. Management should always be individually tailored as not all cases require the same treatment course.

Fundus Photo Right – 4 days post D/C

Fundus Photo Left – 4 days post D/C

Grade 3 papilledema OU with loss of major vessels bilaterally as they exit the disc. The left disc is more edematous than the right. Vessels are tortuous and areas of hemorrhage are seen.

OCT Right – during admission

OCT Right – 4 days post D/C

OCT Right – 12 days post D/C

OCT Right – 18 days post D/C

The edema of the right optic nerve progresses to week day 12 and then begins to resolve slightly by day 18 even though measurements are still above normal limits.

 OCT Left – during admission

OCT Left – 4 days post D/C

OCT Left – 12 days post D/C

OCT Left – 18 days post D/C

Optic nerve swelling is more significant in the left eye compared to the right which corresponds to the left RAPD as well as exam findings. It is slightly improved from baseline after 2 LPs, a drain, and optic nerve sheath fenestration, but then progresses again on day 12. By day 18 after 1 week of 2000mg BID acetazolamide it is resolving slightly.

HVF Right – during admission

HVF Right – 4 days post D/C

HVF Right – 12 days post D/C

HVF Right – 18 days post D/C

The HVF on the right shows enlarge blind spots temporally and arcuate defects nasally. There is progression of visual field losses until day 12 post D/C with some improvement by day 18 post D/C. These findings correspond with patient symptoms as well as with OCT measurements.

HVF Left – during admission

HVF Left – 4 days post D/C

HVF Left – 12 days post D/C

HVF Left – 18 days post D/C

The HVF on the left shows enlarge blind spots temporally and arcuate defects nasally that expand past midline temporally. There is progression of visual field losses until day 12 post D/C with some improvement by day 18 post D/C. The visual field defects are more significant in the left eye than the right eye. These findings correspond with exam findings, fundus photographs, and OCT measurements.

Identifier: Moran_CORE_25503