Name___________________		Date__________________

IIH/PTC Chart Supplement

History:

Ocular history/symptoms? (see new patient form)		

Onset of symptoms:_______days _____weeks_____months		

Pt height_____ft_____inches	Pt weight_________lbs		BMI =_________

Hx of acute visual loss?   			N		Y	OD	OS	OU   Time frame? ___________days

Symptoms and Past Medical History/Medications:

Any visual disturbance or vision loss? 		N		Y 	Diplopia?		N		Y 

Transient vision loss?				N		Y	Pulsatile tinnitus?	N		Y

Permanent vision loss ?				N		Y 	Recomb. growth hormone?	N		Y

Visual Field loss?				N		Y	Lithium use?		N		Y

Obesity?  					N		Y 	Tetracycline class abx?	N		Y

Sleep apnea?					N		Y 	Vit. A derivatives? 	N		Y

Headache?					N		Y 	Other drugs*?		N		Y

*There is moderate evidence that corticosteroids may be associated with IIH. Drugs that were weakly associated with IIH include cyclosporine, progestin-only contraceptives, combined oral contraceptives, second- and third-generation fluoroquinolones, sulfenazone, gonadotropin-releasing hormones and luteinizing hormone-releasing hormone agonist, nalidixic acid, amiodarone, stanozolol, danazol, divalproic acid, sulfasalazine, ketoconazole, and ustekinumab. (Clin Dermatol. 2020 Apr;21(2):163-172). see also (AJO. 2019 Jan; 197:74-79).  Association with oral contraceptives is controversial.

Exam:

Visual acuity (w or w/o)		OD - 20/		OS – 20/

APD?			 	N		Y	OD	OS

Visual field loss?			N		Y	OD	OS	(get HVF)

Ocular motility			normal		abnormal

Diplopia?			N		Y 	(CN6?)		

Optic Nerve Edema? 		N		Y	OD	OS	(Frisen scale 1-5 – see below) _____ (get photos)

Optic Nerve Pallor?		N		Y	OD	OS

MRI/MRV normal?		N		Y

LP Opening Pressure (lateral or prone position) _______ (elevated/consistent with IIH if greater than 25 cm of water). 
(usually have normal or low protein level, normal glucose levels and a normal cell count); Note that normal OP measured in the sitting position may be higher than in the lateral or prone position.  

Differential Diagnosis:  1.  Intracranial mass lesions (tumor, abscess).  2.  Increased cerebrospinal fluid (CSF) production, eg, choroid plexus papilloma.  3.  Decreased CSF absorption, eg, subarachnoid hemorrhage.  4.  Obstructive hydrocephalus.  
5.  Obstruction of venous outflow, eg, venous sinus thrombosis, jugular vein compression, neck surgery

Papilledema Grading System (Frisen Scale) 
Note: Permanent visual loss is the major morbidity of IIH/PTC and is mostly related to the severity of papilledema
	
Stage 0 - Normal Optic Disc - Blurring of nasal, superior and inferior poles in inverse proportion to disc diameter. Radial nerve fiber layer (NFL) without NFL tortuosity.  Rare obscuration of a major blood vessel, usually on the upper pole. 	
Stage 1 -Very Early Papilledema - Obscuration of the nasal border of the disc.  No elevation of the disc borders. Disruption of the normal radial NFL arrangement with
grayish opacity accentuating nerve fiber layer bundles. Normal temporal disc margin. Subtle grayish halo with temporal gap (best seen with indirect ophthalmoscopy).   Concentric or radial retrochoroidal folds.	
Stage 2 - Early Papilledema - Obscuration of all borders. Elevation of the nasal border. Complete peripapillary halo.	
Stage 3 - Moderate Papilledema - Obscurations of all borders. Increased diameter of optic nerve head. Obscuration of one or more segments of major blood vessels
leaving the disc. Peripapillary halo-irregular outer fringe with finger-like extensions. 	
Stage 4 - Marked Papilledema - Elevation of the entire nerve head. Obscuration of all borders. Peripapillary halo. Total obscuration on the disc of a segment of a major
blood vessel. 	
Stage 5 - Severe Papilledema - Dome-shaped protrusions representing anterior expansion of the optic nerve head. Peripapillary halo is narrow and smoothly demarcated.  Total obscuration of a segment of a major blood vessel may or may not be present. Obliteration of the optic cup. 	

Diagnostic Criteria - modified Dandy criteria: IIH is a diagnosis of exclusion. 

1.	Signs and symptoms of increased ICP (headaches, nausea, vomiting, transient visual obscurations, papilledema)
2.	No localizing neurologic signs, except for unilateral or bilateral sixth cranial nerve palsy
3.	CSF opening pressure >25 cm H2O with normal CSF composition
4.	No evidence of hydrocephalus, mass, structural, or vascular lesion (including venous sinus thrombosis) on imaging
5.	No other cause of increased ICP identified

•	Factors that may be independently associated with a worse visual outcome:  1. Gender (male).  2. Race (Black).  
3. Morbid obesity 4. Anemia.  5. Obstructive sleep apnea. 6. Acute onset of symptoms and signs of raised ICP (fulminant IIH)

Assessment:

 Exam, history and scans are inconsistent with IIH/PTC.	 Exam, history and scans are consistent with IIH/PTC.

Plan:
  Follow closely (q ________).				  Order MRI/MRV.

  Suggest weight loss					 Start po acetazolamide (500 mg BID) 
	(e.g. 6% of body weight in IIHTT)			(advance as indicated/tolerated to 4 g per day)

  Order LP w/ opening pressure				  Stop offending drugs					

  Refer for optic nerve sheath fenestration (ONSF)		  Neurosurgery consult for shunt procedure

  Letter/Call to PCP ____________________		 Refer to _____________________ for stat consult.	

Note:	You need urgent surgical consultation (shunt, fenestration, etc.) for fulminant presentation (severe vision loss, severe papilledema, severe visual field loss, extremely elevated OP).
Discussed:
  Risks/side effects of acetazolamide discussed		  Risks/benefits of surgery discussed

  Alternatives/Options 					  Alternative/second opinions