Intracranial Pressure Monitoring in Intracranial Hypotension

Dr. Abhay Moghekar, M.B.,B.S. Johns Hopkins University – School of Medicine • Continuous ICP Monitoring Setup • cICP in skull based CSF leaks • cICP normative data in upright position • cICP in spontaneous spinal CSF leaks • cICP guided percutaneous intervention

• Units: 1 mm Hg = 1.3 cm of Water Limitations of measuring opening pressure during lumbar puncture • Improper positioning, especially when performed under fluoroscopic guidance

• Diurnal variation, increased pressure with sleep-disordered breathing (apnea or hypopnea)

• Hyperventilation due to anxiety and/or pain lower ICP

• Pressures often normal in lateral decubitus position – not surprising since the majority of leak patients have abnormal pressures only during upright position when they are most symptomatic •Direct measurement and graphic recording of intracranial pressure through a trephined opening was described by LEYDEN in 1866.

•KEY & RETZIUS (1875) were the first to measure CSF pressure in animals, and KNOLL (1886) produced the first graphic records CSF pressure

•Lumbar puncture as a clinical method introduced by QUINCKE in 1891 Bedside continuous CSF pressure monitoring

Then… and Heart rate, EKG, respirations, and oxygen saturation are recorded in now… addition to CSFP referenced to the external auditory meatus. Signals are amplified and conditioned using a standard physiology monitoring system (Solar 8000M, GE Healthcare, Waukesha, WI). Analog voltages are sampled at 100 Hz and digital data are logged on a bedside computer (PowerLab data acquisition system and LabChart Pro software; ADInstruments, Colorado Springs, CO). CSF Pressure values in mm H2O measured @ LP*

Acute IIH Chronic IIH Normal Obese Normal Lean Average 343.9 253 167 SD 36.46 136 SD37.6 Range 200-550(100%) >250 (90%) >250 (28%) 200-250(44%) 200-250(25%) <200 (28%) *no sedation, local, 22-gauge needle, <1 ml of CSF loss, left lateral decubitus,head and legs extended, pressure recorded for 1' Revised Criteria: Adults: 5-25 cm, Children: 5-28 cm Normal continuous Intracranial Pressure

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PPG PPG (V) 0 2:56:40 AM 2:58:20 AM 3:00:00 AM 3:01:40 AM 3:03:20 AM 3:05:00 AM 3:06:40 AM 3:08:20 AM Continuous ICP Monitoring – IIH (LP opening pressure: 19 cm H2O)

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• CSF otorrhea and rhinorrhea can be congenital, post blunt head trauma, cranial or endoscopic surgery, or spontaneous

• Spontaneous idiopathic CSF leaks are 25–87% more likely to recur after surgical closure •Hubbard et al, Spontaneous cerebrospinal fluid rhinorrhea: Evolving concepts in diagnosis and surgical management based on the Mayo Clinic experience from 1970 through 1981. Neurosurgery 16:314–321, 1985 •Ommaya et al, Nontraumatic cerebrospinal fluid rhinorrhea. J Neurol Neurosurg Psychiatr 31:214– 215, 1968 • Pseudotumor cerebri and spontaneous CSF leak share common risk factors CSF Otorrhea & Rhinorrhea

• Non-traumatic cases have higher incidence of surgical failure • Some have findings of empty sella - ?forme fruste of PTC CSF Otorrhea & Rhinorrhea - preop

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100 95 90 85 80 75 SaO2 SaO2 (%) 70 7:07:30 AM 7:08:20 AM 7:09:10 AM 7:10:00 AM 7:10:50 AM 7:11:40 AM Perioperative continuous cerebrospinal fluid pressure monitoring in patients with spontaneous cerebrospinal fluid leaks: Reh DD, Gallia GL, Ramanathan M, Solomon D, Moghekar A, Ishii M, Lane AP. Am J Rhinol Allergy. 2010 May-Jun;24(3):238-43. Perioperative ICP management of spontaneous skull based CSF Leaks

• Neuro-ophthalmology assessment • Lumbar puncture • Sleep study – mandibular advancement device for OSA, NOT CPAP • Lumbar continuous ICP Monitoring pre- op and post-op

Perioperative continuous cerebrospinal fluid pressure monitoring in patients with spontaneous cerebrospinal fluid leaks. Xie YJ, Shargorodsky J, Lane AP, Ishii M, Solomon D, Moghekar A, Gallia GL, Reh DD. Int Forum Allergy Rhinol. 2015 Jan;5(1):71-7 What are normal intracranial (not lumbar) pressures when upright ? 11 patients with hydrocephalus, posterior fossa tumors and aqueductal stenosis etc Location matters

3-4 cm What is normative CSF pressure in the sitting/standing position

Author N Subjects Reference Range

Chapman- 8 patients Foramen of Monro -5 to +5 cm H2O Cossman

Loman 13 normals Cisterna magna -8.5 to +4.5 cm of H2O

Bradley 1 patient Convexity -16.5 cm H2O

Fox 18 patients Foramen of Monro -14 to +7 cm

Andresen 4 normals Convexity -10 to 0 mm Hg

Juhler 4 normals Convexity -9 to 0 mm Hg What is low pressure?

Less than -10 mm Hg when monitored by a parenchymal transducer in the cortex.

Normal ICP LabChartchange Window with position

8/27/2008 2:18:58.016 PM 8/27/2008 2:21:52.216 PM

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2:19:00 PM 2:19:30 PM 2:20:00 PM 2:20:30 PM 2:22:00 PM 2:22:30 PM 2:23:00 PM 2:26:00 PM 2829 30 32 33 ICP changes with sitting in a EDS patient with no classic MRI brain findings over 60 mins LabChart Reader Window Severe orthostatic headaches; negative imaging 100

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8:15:00 PM 8:20:00 PM 8:25:00 PM 8:30:00 PM 8:35:00 PM 8:40:00 PM CSF Leak s/p multilevel epidural blood/fibrin patches with persistent orthostatic symptoms LabChart Reader Window

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PP... 0 12:20:00 PM 12:30:00 PM 12:40:00 PM 12:50:00 PM 1:00:00 PM 1:10:00 PM 1:20:00 PM 1:30:00 PM 123 4 5 6 7 8 9 10 11 12 Multi-level perineural cysts Day 1 baseline monitoring, sitting up Day 2 after 4 multilevel blood/fibrin patches Day 3 after an additional 2 levels were targeted Spinal CSF Leak – baseline Day 1 monitoring - supine Day 2 – post patch rebound hypertension - supine

Mean CSFP was greater or equal to 19 mm Hg (about 25 cm water) for 27 mins (8.7% of the recording) and greater or equal to 15 mm Hg (about 20 cm water) for 64 (20.5%). Mean peak CSFP ranged from 9.3 to 42.5, with average mean peak CSF of 16.1. Mean peak CSFP was greaterLabChart or equal Window to 25 for 25 minutes (8.0%). Mean waveform amplitude was 5.6, and ranged from 2.9 to 18.5. Mean waveform amplitude was greater than or equal to 5 for 142 minutes (45.5%). 100 50

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0 PPG... 2:28:10 AM 2:28:20 AM 2:28:30 AM 2:28:40 AM 2:28:50 AM 2:29:00 AM 2:29:10 AM 2:29:20 AM 2:29:30 AM Skull based CSF leak A 61 year-old male with beta-2 transferrin positive left-sided rhinorrhea BMI was 26.6, no clinical history of obstructive sleep apnea, meningitis, trauma, or sinonasal/cranial surgery.

• High-resolution CT showed left sided planum sphenoidale defect • T2-weighted MR demonstrated left sided meningoencephalocele extending into the left sphenoid sinus

• Prior to endoscopic endonasal repair, a lumbar spinal catheter was inserted • Overnight CSF pressure monitoring revealed CSF pressure greater or equal to 19 mm Hg 11% of the time. • Mean CSF pulse waveform amplitude was greater than 5 mm Hg for 53.4% of the time. • Lumbar drainage was performed for 3 days • Overnight post-operative CSF pressure monitoring showed CSF pressure greater than 19 mm Hg only 5.6% of the time. • Positional CSF pressure testing was done… Supine CSF Pressure Recordings

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7:54:20 PM4 7:54:40 PM 5 7:55:00 PM 7:55:206 PM 7:55:407 PM 7:56:00 PM 7:56:20 PM 8 7:56:40 PM Angiography demonstrates focal transverse venous sinus stenosis

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L IJV One year following surgical leak repair he underwent a diagnostic angiogram with cerebral venous sinus manometry

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2 1 0 PPG (V) PPG -1 5:26:30 PM 5:27:00 PM 5:27:30 PM 5:28:00 PM 5:28:30 PM 5:29:00 PM 5:29:30 PM 5:30:00 PM 31 32 33 34 35 36 37 38 39 Bilateral transverse sinus pressure gradient of A 6 x 40 mm right transverse sinus stent was 6.7 mm Hg across the areas of stenosis. then deployed in the right transverse sinus Pressure gradients decreased to 0.4 mm Hg right and 2.3 mm Hg left Eight months following stenting, the patient was weaned off acetazolamide and he remains asymptomatic at 30 months follow up Venous Sinus Stenting in the Management of Patients with Intracranial Hypertension Manifesting with Skull Base Cerebrospinal Fluid Leaks. Iyer RR, Solomon D, Moghekar A, Goodwin CR, Stewart CM, Ishii M, Gailloud P, Gallia GL. World Neurosurg. 2017 Oct;106:103-112 ICP Monitoring in large Tarlov Cysts with no radiographic signs of leak Age Diagnoses Upright pressure Orthostatic (mm Hg) headache 58 Tarlov cyst. -13 Yes 49 Sacral dural cyst -16 Yes 41 Sacral cyst -14 Yes 49 Marfan syndrome -25 Yes Sacral dorsal ectasias 25 Tarlov cyst -10.7 Yes 36 Tarlov cysts -7 Yes 39 Tethered cord syndrome, Tarlov cyst -20 Yes 31 Sacral cyst -12 Yes 66 Large sacral internal meningocele -5 Yes 42 chronic low back pain, sacral dural cysts, -13 Yes infertility, bicornuate uterus. 34 Occult spina bifida -4 Yes Meningocele cICP Implications post-treatment –

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Sleep Apnea related ICP elevations

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• Sleep Apnea • Body position • Rebound intracranial hypertension • Venous Stenosis • Avoid futile treatment • Guide appropriate treatment Complications

• Scalp infection: <1%

• Seizures: <1% ICP duringLabChart a WindowGTC Seizure –

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PPG (V) PPG -0 3:15:00 AM 3:20:00 AM 3:25:00 AM 3:30:00 AM 3:35:00 AM 3:40:00 AM 22 Acknowledgments

Neurology Radiology • Dr. Aruna Rao • Dr. Ferdinand Hui • Dr. David Solomon • Dr. Majid Khan • Dr. Ari Blitz Neurosurgery • Dr. Mark Luciano Neuroscience Nurses