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DRAFT - Pending Review
This plan requires physician review before clinical use.

Low Pressure Headache / Spontaneous Intracranial Hypotension

VERSION: 1.1 CREATED: January 30, 2026 REVISED: January 30, 2026 STATUS: Draft - Pending Review


DIAGNOSIS: Low Pressure Headache / Spontaneous Intracranial Hypotension (SIH)

ICD-10: G96.00 (Cerebrospinal fluid leak, unspecified), G96.01 (Cranial cerebrospinal fluid leak, spontaneous), G96.02 (Spinal cerebrospinal fluid leak, spontaneous), G97.0 (Cerebrospinal fluid leak from spinal puncture), G96.08 (Other cranial cerebrospinal fluid leak), G96.09 (Other spinal cerebrospinal fluid leak), G96.810 (Intracranial hypotension, unspecified), G96.811 (Intracranial hypotension, spontaneous), G96.819 (Other intracranial hypotension), G44.51 (Headache attributed to intracranial hypotension)

CPT CODES: 62270 (Lumbar puncture, diagnostic), 62272 (Therapeutic spinal puncture), 62273 (Epidural blood patch), 72141 (MRI cervical spine without contrast), 72146 (MRI thoracic spine without contrast), 72148 (MRI lumbar spine without contrast), 72156 (MRI cervical spine with and without contrast), 72157 (MRI thoracic spine with and without contrast), 70553 (MRI brain with and without contrast), 72240 (CT myelography, cervical), 72255 (CT myelography, thoracic), 72265 (CT myelography, lumbar), 78630 (Cisternography, radionuclide), 62320 (Epidural injection, cervical/thoracic), 62322 (Epidural injection, lumbar/sacral)

SYNONYMS: Spontaneous intracranial hypotension, SIH, low CSF pressure headache, CSF leak headache, CSF hypovolemia, intracranial hypotension, orthostatic headache, postural headache, post-dural puncture headache, PDPH, post-LP headache, post-lumbar puncture headache, spinal headache, CSF fistula, dural tear headache, spontaneous CSF leak, meningeal diverticulum leak, spinal CSF leak, low-pressure syndrome, CSF hypotension syndrome, spontaneous spinal CSF leak, pachymeningeal enhancement headache, brain sagging, cerebral ptosis, sinking brain syndrome

SCOPE: Evaluation and management of headache due to low cerebrospinal fluid pressure in adults. Covers spontaneous intracranial hypotension (SIH), post-lumbar puncture headache (PDPH), and post-surgical/post-traumatic CSF leaks. Includes diagnostic workup (MRI brain, MRI spine, CT myelography, cisternography), conservative management, epidural blood patch, and surgical repair for refractory cases. Excludes cranial CSF rhinorrhea/otorrhea from skull base defects (managed by otolaryngology/neurosurgery), and CSF leaks secondary to VP shunt overdrainage.


DEFINITIONS:

  • Spontaneous Intracranial Hypotension (SIH): Low CSF pressure (<6 cm H2O) or CSF hypovolemia from spontaneous spinal CSF leak, causing orthostatic headache and characteristic MRI findings
  • Post-Dural Puncture Headache (PDPH): Headache occurring within 5 days of lumbar puncture or spinal anesthesia, typically orthostatic, due to persistent dural rent and CSF leak
  • Orthostatic Headache: Headache that worsens significantly within 15 minutes of assuming upright posture and improves within 15-30 minutes of recumbency
  • Epidural Blood Patch (EBP): Injection of autologous blood into the epidural space to seal a dural defect and restore CSF volume
  • Pachymeningeal Enhancement: Diffuse, smooth, non-nodular dural enhancement on gadolinium-enhanced MRI, characteristic of intracranial hypotension
  • CSF Hypovolemia: Reduction in total CSF volume causing brain sagging and traction on pain-sensitive structures, even when opening pressure may be normal
  • Dural Ectasia: Weakness and ballooning of the dural sac, often associated with connective tissue disorders, predisposing to CSF leaks
  • CSF-Venous Fistula: Abnormal connection between a spinal nerve root sleeve and an adjacent epidural vein, causing CSF drainage into the venous system

PRIORITY KEY: STAT = Immediate | URGENT = Within hours | ROUTINE = Standard | EXT = Extended/atypical cases | - = Not applicable to this setting

═══════════════════════════════════════════════════════════════ SECTION A: ACTION ITEMS ═══════════════════════════════════════════════════════════════

1. LABORATORY WORKUP

1A. Essential/Core Labs

Test Rationale Target Finding ED HOSP OPD ICU
CBC with differential (CPT 85025) Baseline; rule out infection or anemia contributing to symptoms Normal URGENT ROUTINE ROUTINE URGENT
CMP (CPT 80053) Electrolytes, renal function; baseline before IV fluids and medications Normal URGENT ROUTINE ROUTINE URGENT
Coagulation studies (PT/INR, aPTT) (CPT 85610, 85730) Required before epidural blood patch or lumbar puncture; assess bleeding risk Normal coagulation URGENT ROUTINE ROUTINE URGENT
ESR (CPT 85652) / CRP (CPT 86140) Rule out inflammatory or infectious cause of meningeal enhancement Normal URGENT ROUTINE ROUTINE URGENT
Pregnancy test (beta-hCG) (CPT 84703) Affects imaging decisions (gadolinium, CT myelography) and treatment Document status STAT STAT ROUTINE STAT

1B. Extended Workup (Second-line)

Test Rationale Target Finding ED HOSP OPD ICU
TSH (CPT 84443) Hypothyroidism can cause headache; rule out secondary cause Normal - ROUTINE ROUTINE -
Magnesium (CPT 83735) Low magnesium can worsen headache; contributes to muscle cramps >1.8 mg/dL URGENT ROUTINE ROUTINE URGENT
Blood cultures (CPT 87040) If febrile or concern for meningitis/epidural abscess mimicking SIH No growth URGENT ROUTINE - URGENT
Connective tissue markers (if recurrent SIH) Marfan, Ehlers-Danlos screening; associated with dural weakness Document - - EXT -

1C. Rare/Specialized (Refractory or Atypical)

Test Rationale Target Finding ED HOSP OPD ICU
Genetic testing for connective tissue disorders Recurrent SIH in young patients; EDS, Marfan syndrome evaluation Document - - EXT -
Beta-2 transferrin (nasal or ear fluid) Confirm CSF vs. other fluid if rhinorrhea or otorrhea present Positive = CSF leak URGENT ROUTINE ROUTINE URGENT
CT cisternography with intrathecal contrast Localize CSF leak site when MRI and CT myelography are inconclusive Identify leak site - EXT EXT -

2. DIAGNOSTIC IMAGING & STUDIES

2A. Essential/First-line

Study Timing Target Finding Contraindications ED HOSP OPD ICU
MRI brain with and without gadolinium (CPT 70553) First-line diagnostic study; perform urgently if SIH suspected Diffuse pachymeningeal enhancement, brain sagging, subdural collections, pituitary engorgement, venous distension sign Pacemaker, metal implants; gadolinium contraindicated in severe renal impairment (eGFR <30) URGENT URGENT ROUTINE URGENT
CT head without contrast (CPT 70450) If MRI unavailable; rule out subdural hematoma, hemorrhage Rule out SDH, hemorrhage; CT often normal in SIH None in emergency STAT URGENT - STAT

2B. Extended (Leak Localization)

Study Timing Target Finding Contraindications ED HOSP OPD ICU
MRI total spine with and without contrast (CPT 72156, 72157, 72158) After brain MRI confirms SIH; localize leak level Epidural fluid collections, meningeal diverticula, dural ectasia, nerve root sleeve irregularity, extradural CSF Pacemaker, metal implants - URGENT ROUTINE -
CT myelography (intrathecal contrast) (CPT 72240, 72255, 72265) Gold standard for leak localization; after failed blind EBP or before targeted treatment Contrast extravasation at leak site; epidural contrast pooling Contrast allergy, coagulopathy, infection at injection site - URGENT ROUTINE -
Dynamic CT myelography Fast CSF leaks not captured on conventional CT myelo; rapid-sequence imaging after intrathecal injection Active contrast extravasation during imaging Same as CT myelography - EXT EXT -
Digital subtraction myelography (DSM) CSF-venous fistula detection; gold standard for CVF when CT myelography negative CSF-venous fistula (contrast filling epidural vein) Same as CT myelography; requires interventional suite - EXT EXT -

2C. Rare/Specialized

Study Timing Target Finding Contraindications ED HOSP OPD ICU
Radionuclide cisternography (CPT 78630) Confirm CSF leak and assess CSF dynamics; less precise for localization Early bladder activity (<4 hours), absent activity over convexities at 24-48 hours, parathecal activity Intrathecal injection contraindications - EXT EXT -
MR myelography (heavily T2-weighted) Non-invasive leak localization; useful for epidural collections and meningeal diverticula Extrathecal fluid signal along spine Pacemaker, metal implants - ROUTINE ROUTINE -
Intrathecal gadolinium MRI (off-label) Slow leaks not detected by CT myelography; high sensitivity Gadolinium extravasation at leak site Off-label use; requires informed consent; use low dose (0.5 mL gadoteridol) - EXT EXT -

MRI Brain Findings in SIH (Diagnostic Reference)

Finding Significance Sensitivity
Diffuse pachymeningeal (dural) enhancement Most characteristic finding; smooth, non-nodular, bilateral ~83%
Brain sagging / downward displacement Cerebellar tonsillar descent, flattening of pons, effacement of prepontine cistern ~50-75%
Subdural collections (hygromas or hematomas) Traction on bridging veins; may require evacuation if large ~40-50%
Pituitary engorgement Hyperemia of pituitary gland; convex superior margin ~50-60%
Venous distension sign Engorgement of dural venous sinuses and cerebral veins ~75%
Decrease in ventricular size Small ventricles compared to baseline; "slit ventricles" Variable
Optic chiasm descent Sagging of optic chiasm below plane of anterior clinoids Variable

LUMBAR PUNCTURE (CPT 62270)

Indication: Confirm low CSF pressure (opening pressure <6 cm H2O diagnostic; may be normal in 20-30% of SIH); obtain CSF to rule out meningitis or other causes of meningeal enhancement; therapeutic in IIH (NOT therapeutic in SIH - do not drain CSF)

Timing: URGENT if diagnosis uncertain and need to differentiate from meningitis; ROUTINE for elective diagnosis; CAUTION: LP can worsen symptoms in SIH patients and is often deferred if MRI findings are classic

Volume Required: 1-3 mL only (minimal volume; do NOT perform large-volume LP in suspected SIH)

Study Rationale Target Finding ED HOSP OPD ICU
Opening pressure Diagnostic criterion for intracranial hypotension; <6 cm H2O diagnostic but may be normal (0-6 cm H2O supports diagnosis) <6 cm H2O (may be 0 or negative; normal in 20-30%) URGENT URGENT ROUTINE URGENT
Cell count (tubes 1 and 4) (CPT 89051) Rule out meningitis or SAH; SIH may show mild pleocytosis (<5-50 WBC) WBC <5 typical; mild elevation possible in SIH URGENT URGENT ROUTINE URGENT
Protein (CPT 84157) Often mildly elevated in SIH (45-100 mg/dL) due to meningeal irritation; rule out infection Normal 15-45 mg/dL; mildly elevated in SIH is expected URGENT URGENT ROUTINE URGENT
Glucose with serum glucose (CPT 82945) Rule out infectious meningitis Normal (>60% of serum glucose) URGENT URGENT ROUTINE URGENT
Gram stain and culture (CPT 87205, 87070) Rule out bacterial meningitis if fever or meningismus present No organisms URGENT URGENT - URGENT
RBC count (CPT 89051) Rule out traumatic tap vs. xanthochromia from chronic SIH-related subdural collections Document; xanthochromia may occur in SIH URGENT URGENT ROUTINE URGENT

Special Handling: Measure opening pressure with patient in lateral decubitus, legs extended, relaxed; use smallest gauge needle possible (22G or smaller) to minimize additional leak risk Contraindications: LP may worsen SIH symptoms; defer if MRI brain findings are classic for SIH; mass lesion; coagulopathy (INR >1.5, platelets <50,000); local infection at puncture site


3. TREATMENT

3A. Acute/Conservative Management

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
IV normal saline IV Volume resuscitation and symptom relief in acute SIH/PDPH 75-150 mL/hr :: IV :: continuous :: NS at 75-150 mL/hr; bolus 1L if dehydrated; maintain euvolemia Heart failure, volume overload, renal failure I/O, daily weights, electrolytes STAT STAT - STAT
Caffeine sodium benzoate IV IV Acute treatment of PDPH; cerebral vasoconstriction to counteract CSF hypotension 500 mg once :: IV :: once :: 500 mg IV infused over 1 hour; may repeat once in 4-6 hours; max 1000 mg/day Cardiac arrhythmia, uncontrolled tachycardia, seizure disorder Heart rate, blood pressure, arrhythmia URGENT URGENT - URGENT
Caffeine oral PO Mild SIH/PDPH; cerebral vasoconstriction 200 mg q6h :: PO :: q6h PRN :: 200-300 mg PO q6h (coffee ~100 mg/cup); max 900 mg/day; adjunct to other measures Cardiac arrhythmia, severe anxiety, insomnia Heart rate, sleep quality, anxiety URGENT ROUTINE ROUTINE -
Strict bed rest (Trendelenburg) - Reduce hydrostatic pressure across dural defect; facilitate seal Flat or Trendelenburg position :: - :: continuous :: Strict bed rest in supine or slight Trendelenburg (10-15 degrees) for 24-48 hours initially; allow bathroom privileges only DVT risk with prolonged immobility DVT prophylaxis, skin integrity STAT STAT - STAT
Abdominal binder - Increase intra-abdominal and epidural venous pressure to tamponade leak Apply snugly :: - :: continuous :: Apply abdominal binder continuously while upright; may improve symptoms by increasing epidural pressure Abdominal surgery, respiratory compromise Respiratory comfort, skin integrity URGENT ROUTINE ROUTINE -
Acetaminophen PO Headache symptom relief 1000 mg q6h :: PO :: q6h PRN :: 1000 mg PO q6h PRN headache; max 3000 mg/day Hepatic disease, chronic alcohol use >3 drinks/day LFTs if prolonged use URGENT ROUTINE ROUTINE -
Ibuprofen PO Headache and meningeal inflammation relief 400 mg q6h :: PO :: q6h PRN :: 400-600 mg PO q6h PRN; max 2400 mg/day; take with food Renal disease, GI bleeding, anticoagulation, aspirin allergy Renal function, GI symptoms URGENT ROUTINE ROUTINE -
Ondansetron IV/PO Nausea associated with orthostatic symptoms and SIH 4 mg q8h PRN :: IV :: q8h PRN :: 4-8 mg IV or PO q8h PRN nausea; max 24 mg/day QT prolongation, severe hepatic impairment QTc if repeated dosing URGENT ROUTINE ROUTINE -
Docusate sodium PO Prevent straining during bed rest which can worsen CSF leak 100 mg BID :: PO :: BID :: 100 mg PO BID; continue throughout bed rest period and until regular activity resumes Intestinal obstruction Bowel frequency - ROUTINE ROUTINE -
Enoxaparin SC DVT prophylaxis during prolonged bed rest in hospitalized SIH patients 40 mg daily :: SC :: daily :: 40 mg SC daily; begin within 24 hours of admission if on bed rest >48 hours; hold 12 hours before and after epidural blood patch Active bleeding, HIT, severe renal impairment (CrCl <30: reduce to 30 mg daily), coagulopathy, within 12 hours of EBP Platelet count q2-3 days; signs of bleeding; renal function - ROUTINE - ROUTINE

3B. Epidural Blood Patch (Primary Definitive Treatment)

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Epidural blood patch - blind/non-targeted (CPT 62273) Epidural First-line definitive treatment for PDPH and SIH; seals dural defect with autologous blood clot 15-30 mL autologous blood :: Epidural :: once :: Inject 15-30 mL autologous blood into lumbar or thoracolumbar epidural space under fluoroscopy; patient lies flat 1-2 hours post-procedure; ~70-90% PDPH success rate; ~30-50% SIH success rate on first attempt Active systemic infection/bacteremia, local skin infection, coagulopathy (INR >1.5, platelets <50K), patient refusal Vital signs during and 1h post-procedure; neurologic exam; pain at injection site; radicular symptoms (transient acceptable) URGENT URGENT ROUTINE -
Epidural blood patch - targeted (CPT 62273) Epidural SIH after leak localization by CT myelography or MRI spine; targeted to level of leak 15-30 mL autologous blood :: Epidural :: once :: Inject 15-30 mL autologous blood at or near identified leak level under fluoroscopic or CT guidance; higher success rate for SIH when targeted; may require multi-level injection Active systemic infection/bacteremia, local skin infection, coagulopathy (INR >1.5, platelets <50K), patient refusal Vital signs during and 1h post-procedure; neurologic exam; radicular symptoms; headache improvement within 24-72 hours - URGENT ROUTINE -
Repeat epidural blood patch Epidural Persistent symptoms after initial EBP; may attempt 2-3 times before escalating 20-30 mL autologous blood :: Epidural :: once :: Repeat EBP 5-7 days after first attempt if symptoms persist; may use larger volume (up to 30 mL); consider targeted approach if not done initially Active systemic infection/bacteremia, local skin infection, coagulopathy (INR >1.5, platelets <50K), patient refusal Vital signs during and 1h post-procedure; neurologic exam; consider leak localization if second EBP fails - URGENT ROUTINE -

3C. Pharmacologic Adjuncts

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Theophylline PO Adjunctive treatment for SIH; increases CSF production via adenosine receptor antagonism 200 mg BID :: PO :: BID :: Start 200 mg PO BID; titrate to 300 mg BID based on serum levels; target serum level 10-15 mcg/mL; may take 1-2 weeks for effect Seizure disorder, cardiac arrhythmia, active peptic ulcer, hepatic impairment Theophylline level q2-4 weeks initially; heart rate; GI symptoms; drug interactions (CYP1A2) - ROUTINE ROUTINE -
Hydrocortisone IV Refractory SIH not responding to EBP; anti-inflammatory effect on meninges; may increase CSF production 200 mg daily :: IV :: daily :: 200 mg IV on day 1 then taper: 200 mg IV daily x 2d, 150 mg x 2d, 100 mg x 2d, then convert to oral; total course 7-14 days Active untreated infection, uncontrolled diabetes Blood glucose q6h, blood pressure, electrolytes - URGENT - URGENT
Prednisone (oral taper) PO Outpatient management of refractory SIH; meningeal inflammation 60 mg daily :: PO :: daily :: 60 mg PO daily x 5 days, then taper by 10 mg every 3-5 days over 3-4 weeks Active untreated infection, uncontrolled diabetes, GI bleeding Blood glucose, blood pressure, mood, GI symptoms - ROUTINE ROUTINE -
Gabapentin PO Neuropathic headache component; adjunctive pain management in SIH 300 mg qHS :: PO :: qHS then TID :: Start 300 mg PO qHS; titrate by 300 mg q3-5d; target 900-1800 mg/day divided TID; max 3600 mg/day Severe renal impairment (adjust dose) Sedation, dizziness, peripheral edema - ROUTINE ROUTINE -
Pregabalin PO Alternative to gabapentin for neuropathic pain component 75 mg BID :: PO :: BID :: Start 75 mg PO BID; increase to 150 mg BID after 1 week; max 300 mg BID Severe renal impairment (adjust dose), angioedema history Sedation, weight gain, peripheral edema - ROUTINE ROUTINE -

3D. Interventional/Refractory Treatment

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Epidural fibrin glue patch Epidural Refractory SIH after failed EBP (2-3 attempts); seals dural defect with fibrin sealant Fibrin sealant at leak site :: Epidural :: once :: Inject fibrin glue (e.g., Tisseel) at identified leak site under fluoroscopic or CT guidance; may combine with blood patch; performed by anesthesia or interventional radiology Active infection, known allergy to fibrin/bovine components, coagulopathy Post-procedure neuro exam, headache assessment, vital signs; observe 2-4 hours post - URGENT EXT -
CT-guided percutaneous fibrin glue injection Percutaneous Precisely localized leak refractory to EBP; allows direct placement at dural defect Fibrin sealant via CT-guided needle :: Percutaneous :: once :: CT-guided needle placement at leak site followed by fibrin glue injection; requires interventional radiology expertise Active infection, coagulopathy, inability to identify leak site Post-procedure neuro exam; imaging follow-up - EXT EXT -
Surgical dural repair Surgical Definitive treatment for identified dural tear or meningeal diverticulum refractory to all conservative and percutaneous therapies Surgical exploration and primary dural repair :: - :: once :: Open or minimally invasive surgical repair of dural defect; may include muscle/fascia graft; requires neurosurgical expertise; hospitalization 3-7 days Poor surgical candidate, unable to localize leak Post-op neuro exam, wound care, headache assessment, CSF leak recurrence monitoring - ROUTINE EXT ROUTINE
CSF-venous fistula ligation/embolization Surgical/Endovascular Confirmed CSF-venous fistula on DSM; increasingly recognized cause of refractory SIH Surgical ligation or transvenous embolization :: - :: once :: Surgical ligation of fistulous nerve root sleeve; or transvenous embolization of draining vein; requires specialized center Unable to confirm fistula, coagulopathy for surgery Post-procedure neuro exam; repeat imaging to confirm resolution - EXT EXT -
Continuous epidural saline infusion Epidural Bridge therapy while awaiting definitive treatment; refractory symptoms NS at 20-50 mL/hr :: Epidural :: continuous :: Continuous epidural normal saline infusion at 20-50 mL/hr via epidural catheter; temporary measure for severe refractory symptoms Epidural infection, coagulopathy Catheter site infection, neurologic exam, headache response - EXT - EXT
Intrathecal saline infusion Intrathecal Severe refractory SIH as bridge to definitive repair; restores CSF volume temporarily NS at 15-30 mL/hr :: Intrathecal :: continuous :: Intrathecal normal saline via lumbar drain at 15-30 mL/hr; temporary measure only; risk of infection increases with duration Active infection, coagulopathy Infection surveillance, neurologic exam, ICP monitoring - EXT - EXT

3E. Subdural Hematoma Management (Complication of SIH)

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Observation with serial imaging - Small, asymptomatic subdural collections; most resolve after CSF leak treatment Serial CT head q1-2 weeks :: - :: per protocol :: Most subdural hygromas/hematomas resolve after successful treatment of underlying CSF leak; avoid surgical evacuation if possible None Neuro checks, serial CT, symptom progression URGENT ROUTINE ROUTINE URGENT
Epidural blood patch (treat underlying cause) Epidural Subdural collections secondary to SIH; treating the leak often resolves the subdural 15-30 mL autologous blood :: Epidural :: once :: EBP should be performed BEFORE considering surgical evacuation; inject 15-30 mL autologous blood into epidural space under fluoroscopy; SDH from SIH often recurs after drainage if leak not addressed Active systemic infection/bacteremia, local skin infection, coagulopathy (INR >1.5, platelets <50K), patient refusal Vital signs during and 1h post-procedure; neurologic exam; serial CT for SDH URGENT URGENT ROUTINE -
Neurosurgical evacuation Surgical Large symptomatic SDH with mass effect or neurologic deterioration not responding to CSF leak treatment Burr hole or craniotomy :: - :: once :: Surgical evacuation only if mass effect, midline shift >5 mm, or declining mental status AND CSF leak has been addressed; high recurrence if leak untreated Poor surgical candidate Post-op neuro checks, repeat CT, ICP monitoring STAT STAT - STAT

4. OTHER RECOMMENDATIONS

4A. Referrals & Consults

Recommendation ED HOSP OPD ICU
Neurology consult for diagnosis confirmation and management of intracranial hypotension URGENT ROUTINE ROUTINE URGENT
Anesthesiology or pain medicine consult for epidural blood patch placement URGENT URGENT ROUTINE URGENT
Neuroradiology consult for CT myelography interpretation and leak localization - URGENT ROUTINE -
Interventional radiology consult for CT-guided fibrin glue injection or targeted epidural blood patch - ROUTINE ROUTINE -
Neurosurgery consult for surgical dural repair if refractory to blood patch and fibrin glue treatments - ROUTINE ROUTINE -
Neurosurgery consult for subdural hematoma evaluation if symptomatic mass effect develops STAT STAT - STAT
Headache specialist referral for chronic post-SIH headache or persistent symptoms despite leak resolution - - ROUTINE -
Genetics/rheumatology referral for connective tissue disorder evaluation if recurrent spontaneous CSF leaks - - EXT -
Physical therapy for gradual return to activity and deconditioning after prolonged bed rest - ROUTINE ROUTINE -

4B. Patient/Family Instructions

Recommendation ED HOSP OPD ICU
Remain flat or in Trendelenburg position as much as possible to reduce headache while awaiting definitive treatment (upright posture worsens symptoms) ROUTINE ROUTINE ROUTINE -
Increase oral fluid intake to at least 2-3 liters daily to support CSF production and hydration ROUTINE ROUTINE ROUTINE -
Increase caffeine intake (coffee, tea) to 300-500 mg/day as this constricts cerebral blood vessels and may reduce headache ROUTINE ROUTINE ROUTINE -
Avoid Valsalva maneuvers (straining, heavy lifting, coughing) which can worsen or re-open CSF leaks ROUTINE ROUTINE ROUTINE -
After epidural blood patch: lie flat for 1-2 hours post-procedure, then gradually increase activity over 24-48 hours; avoid heavy lifting for 2-4 weeks - ROUTINE ROUTINE -
Return to ED immediately if headache suddenly becomes the worst headache of life, new neurologic symptoms develop (weakness, numbness, vision changes, confusion), or fever occurs (may indicate subdural hematoma, meningitis, or abscess) ROUTINE ROUTINE ROUTINE -
Some patients have persistent headache after successful leak treatment; this typically improves over weeks to months - ROUTINE ROUTINE -
Follow up with neurology in 1-2 weeks after epidural blood patch to assess response and plan further workup if needed - ROUTINE ROUTINE -

4C. Lifestyle & Prevention

Recommendation ED HOSP OPD ICU
Stay well-hydrated (2-3 L daily) to support CSF production and overall recovery ROUTINE ROUTINE ROUTINE -
Gradual return to physical activity after symptom resolution; avoid strenuous exercise for 4-6 weeks after blood patch - ROUTINE ROUTINE -
Avoid excessive caffeine withdrawal if caffeine has been used therapeutically (taper gradually to prevent rebound headache) - ROUTINE ROUTINE -
Compression stockings during prolonged bed rest for DVT prevention; ambulate when tolerated - ROUTINE - -
If connective tissue disorder identified: avoid contact sports and activities with high risk of spinal trauma - - ROUTINE -
Inform future anesthesiologists of SIH history; use smallest gauge spinal needle if LP/spinal anesthesia required in future - ROUTINE ROUTINE -

═══════════════════════════════════════════════════════════════ SECTION B: REFERENCE (Expand as Needed) ═══════════════════════════════════════════════════════════════

5. DIFFERENTIAL DIAGNOSIS

Alternative Diagnosis Key Distinguishing Features Tests to Differentiate
Migraine Pulsating, unilateral; nausea/photophobia; NOT positional (no clear orthostatic pattern); prior history Clinical history; no pachymeningeal enhancement on MRI; no orthostatic pattern
Post-lumbar puncture headache (iatrogenic) Clear history of recent LP or spinal anesthesia within days; typically resolves spontaneously or with EBP History of procedure; timing correlation; response to blood patch
Idiopathic intracranial hypertension (IIH) Headache WORSE when lying down; papilledema; elevated opening pressure (>25 cm H2O); obesity LP with elevated OP; MRI shows empty sella, optic nerve sheath distension (no pachymeningeal enhancement)
Cerebral venous sinus thrombosis (CVST) Progressive headache; seizures; focal deficits; hypercoagulable state; papilledema MRV or CT venography showing venous thrombosis
Meningitis (infectious) Fever, neck stiffness, photophobia; meningeal enhancement may mimic SIH CSF pleocytosis, low glucose, positive cultures; fever; systemic illness
Leptomeningeal carcinomatosis Nodular (not smooth) meningeal enhancement; cranial neuropathies; known malignancy MRI shows nodular enhancement (vs. smooth in SIH); CSF cytology positive
Chiari I malformation Positional headache may overlap; but usually Valsalva-provoked; not clearly orthostatic MRI sagittal showing tonsillar descent >5 mm below foramen magnum; no pachymeningeal enhancement
Colloid cyst of third ventricle Positional headache (worse with certain head positions); intermittent hydrocephalus MRI/CT showing colloid cyst in third ventricle; acute hydrocephalus
Subdural hematoma (primary) Headache, confusion, focal deficits; history of trauma or anticoagulation CT/MRI showing subdural collection without brain sagging or pachymeningeal enhancement
Medication overuse headache Chronic daily headache; overuse of analgesics >15 days/month Medication diary; no orthostatic pattern; no MRI enhancement
Postural orthostatic tachycardia syndrome (POTS) Orthostatic symptoms (lightheadedness, palpitations); headache may be positional Tilt table test; HR increase >30 bpm without significant BP drop; normal MRI brain
Tension-type headache Bilateral, pressing, mild-moderate; no positional component Clinical diagnosis; no MRI abnormalities

6. MONITORING PARAMETERS

Parameter Frequency Target/Threshold Action if Abnormal ED HOSP OPD ICU
Headache severity (0-10 VAS) with positional assessment Per assessment; daily inpatient; each visit outpatient Improving trend; resolution of orthostatic component Escalate to EBP if conservative measures fail; repeat EBP or advance to imaging/intervention STAT ROUTINE ROUTINE STAT
Neurologic examination (mental status, cranial nerves, motor, sensory) Per assessment; daily inpatient Normal; no new focal deficits Urgent imaging if new deficits (concern for SDH, brain herniation) STAT ROUTINE ROUTINE STAT
Orthostatic vital signs Each assessment in ED; daily inpatient No significant orthostatic changes Increase IV fluids; assess for concurrent volume depletion STAT ROUTINE ROUTINE STAT
CT head (for subdural collections) After diagnosis; repeat if symptoms worsen or fail to improve No new or enlarging subdural collections Neurosurgical consultation for large or symptomatic SDH URGENT ROUTINE ROUTINE URGENT
MRI brain with gadolinium (follow-up) 4-8 weeks after treatment; earlier if symptoms recur Resolution of pachymeningeal enhancement and brain sagging Consider repeat EBP or advanced leak localization - ROUTINE ROUTINE -
Theophylline level (if on theophylline) q2-4 weeks initially; then q3 months when stable 10-15 mcg/mL Dose adjust; hold if toxic (>20 mcg/mL) - ROUTINE ROUTINE -
Blood glucose (if on steroids) q6h while on IV steroids; daily on oral taper <180 mg/dL Insulin sliding scale or hypoglycemic agent adjustment - ROUTINE ROUTINE ROUTINE
Rebound intracranial hypertension monitoring Daily during first week after successful CSF leak treatment; each outpatient visit for 3 months No new headache worse when lying down; no papilledema Fundoscopic exam; consider LP to measure OP; acetazolamide if confirmed rebound IH - ROUTINE ROUTINE -

7. DISPOSITION CRITERIA

Disposition Criteria
Discharge from ED Mild orthostatic headache responsive to oral analgesics and caffeine; able to tolerate PO; no red flags (new neurologic deficits, fever, worst headache of life); outpatient MRI and neurology follow-up within 1 week arranged
Admit to hospital (floor) Severe orthostatic headache preventing ambulation or oral intake; need for IV hydration and caffeine; scheduled for epidural blood patch; new diagnosis of SIH requiring urgent MRI and workup; concern for subdural hematoma
Admit to hospital (observation) Post-dural puncture headache not responding to conservative measures after 24-48 hours; awaiting epidural blood patch
Neurosurgery/ICU transfer Large subdural hematoma with mass effect or neurologic deterioration; posterior fossa crowding from brain sagging with signs of brainstem compression
Discharge from hospital Headache significantly improved (>50% reduction) after EBP or conservative treatment; tolerating oral medications; able to ambulate; close outpatient follow-up arranged within 1-2 weeks
Outpatient management Mild symptoms manageable with oral analgesics and caffeine; able to maintain hydration; no red flags; MRI brain already obtained or scheduled; neurology follow-up within 1-2 weeks
Outpatient follow-up schedule Post-EBP: 1-2 weeks; Refractory SIH: weekly until improved; After resolution: 3 months then annually for 1-2 years to monitor for recurrence

8. EVIDENCE & REFERENCES

Recommendation Evidence Level Source
Orthostatic headache is hallmark of SIH Expert consensus; diagnostic criterion Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006 PubMed: 16705110
MRI brain with gadolinium showing pachymeningeal enhancement is most sensitive diagnostic finding Class II, Level B Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache 2013 PubMed: 23808630
Epidural blood patch is first-line definitive treatment for PDPH Class I, Level A Boonmak P, Boonmak S. Epidural blood patching for preventing and treating post-dural puncture headache. Cochrane Database Syst Rev 2010 PubMed: 20091522
Epidural blood patch effective for SIH Class II, Level B Sencakova D et al. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology 2001 PubMed: 11723293
CT myelography is gold standard for CSF leak localization Class II, Level B Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia. Mayo Clin Proc 1999 PubMed: 10560599
Caffeine provides short-term benefit for PDPH Class II, Level B Camann WR et al. Effects of oral caffeine on postdural puncture headache. Anesth Analg 1990 PubMed: 2405733
Conservative treatment (bed rest, hydration, caffeine) as initial approach Expert consensus Schievink WI. Spontaneous spinal cerebrospinal fluid leaks. Cephalalgia 2008 PubMed: 19037970
CSF opening pressure may be normal in 20-30% of SIH patients Class III, Level B Schievink WI et al. Diagnostic criteria for headache due to spontaneous intracranial hypotension. Headache 2011 PubMed: 21658029
Targeted EBP has higher success rate than blind EBP for SIH Class III, Level B Kranz PG et al. CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol 2011 PubMed: 21349964
Fibrin glue sealant effective for refractory CSF leaks Class III, Level C Schievink WI et al. Treatment of spontaneous intracranial hypotension with percutaneous placement of fibrin sealant. J Neurosurg 2004 PubMed: 15200130
Surgical repair for refractory dural defects Class III, Level C Schievink WI et al. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998 PubMed: 9452231
Brain sagging on MRI correlates with clinical severity Class III, Level B Savoiardo M et al. Spontaneous intracranial hypotension with deep brain swelling. Brain 2007 PubMed: 17535837
Connective tissue disorders (EDS, Marfan) predispose to SIH Class III, Level B Schievink WI et al. Connective tissue disorders with spontaneous spinal CSF leaks and intracranial hypotension. Neurosurgery 2004 PubMed: 14683542
Subdural hematomas complicate SIH and often resolve after leak treatment Class III, Level B De Noronha RJ et al. Subdural haematoma: a potentially serious consequence of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry 2003 PubMed: 12754345
CSF-venous fistulas are an underrecognized cause of SIH Class III, Level C Schievink WI et al. A classification system of spontaneous spinal CSF leaks. Neurology 2016 PubMed: 27440149
Digital subtraction myelography is gold standard for CSF-venous fistula detection Class III, Level C Farb RI et al. Spontaneous intracranial hypotension: a systematic imaging approach for CSF leak localization and management. AJNR Am J Neuroradiol 2019 PubMed: 30923083
Theophylline may increase CSF production via adenosine receptor antagonism Class III, Level C Mokri B. Headaches caused by decreased intracranial pressure: diagnosis and management. Curr Opin Neurol 2003 PubMed: 12858068
Hydrocortisone/corticosteroids as adjunctive therapy for refractory SIH Class III, Level C Grimaldi D et al. Spontaneous low cerebrospinal pressure: a mini review. Neurol Sci 2004 PubMed: 15549523
ICHD-3 diagnostic criteria for headache attributed to low CSF pressure Expert consensus Headache Classification Committee of the International Headache Society. ICHD-3. Cephalalgia 2018 PubMed: 29368949
Intrathecal gadolinium MRI for slow leak detection Class III, Level C Akbar JJ et al. The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol 2012 PubMed: 22173753
MR myelography for non-invasive leak screening Class II, Level B Wang SJ. Spontaneous intracranial hypotension. Continuum (Minneap Minn) 2021 PubMed: 34048402
Dynamic CT myelography for fast CSF leaks Class III, Level C Kranz PG et al. Imaging signs in spontaneous intracranial hypotension: prevalence and relationship to CSF pressure. AJNR Am J Neuroradiol 2016 PubMed: 26869465
Continuous epidural saline infusion as bridge therapy Case series Bai J et al. Continuous epidural saline infusion for treatment of refractory spontaneous intracranial hypotension. Pain Physician 2017

NOTES

  • SIH is often underdiagnosed; maintain high suspicion in any patient with new orthostatic headache
  • Classic triad: orthostatic headache, diffuse pachymeningeal enhancement on MRI, low CSF opening pressure (though OP may be normal in 20-30%)
  • MRI brain with gadolinium is the most important diagnostic test; do NOT rely on LP alone
  • PDPH typically self-resolves within 1-2 weeks; EBP indicated if symptoms persist beyond 24-48 hours or are severe
  • For SIH, blind EBP success rate is only 30-50% (vs. 70-90% for PDPH); targeted EBP after leak localization is preferred
  • CSF-venous fistulas are an increasingly recognized cause of SIH; require specialized imaging (digital subtraction myelography) for diagnosis
  • Subdural hematomas in SIH patients should be treated by addressing the underlying CSF leak first; surgical evacuation alone leads to high recurrence
  • LP in suspected SIH may worsen symptoms and should be deferred if MRI brain findings are classic; when performed, use smallest gauge needle and remove minimal volume
  • Connective tissue disorders (Ehlers-Danlos, Marfan) are risk factors; screen if recurrent or young patient
  • Rebound intracranial hypertension can occur after successful CSF leak treatment; monitor for new headache pattern (worse lying down)
  • Chronic SIH can lead to superficial siderosis from repeated microbleeding along the neuroaxis

CHANGE LOG

v1.1 (January 30, 2026) - Added ICU coverage across all applicable sections (labs, LP, imaging, acute treatment, monitoring, referrals) per S1 - Fixed cross-references in Section 3E: replaced "Per EBP protocol above", "Per EBP contraindications", and "Per EBP monitoring" with self-contained content per C1/R1 - Added PubMed citation links to all references in Section 8; corrected journal names for Savoiardo (Brain 2007), Schievink connective tissue (Neurosurgery 2004), Mokri theophylline (Curr Opin Neurol 2003), Grimaldi mini review (Neurol Sci 2004), and Kranz targeted EBP (AJNR 2011) per C3/R2 - Moved docusate sodium from Section 4C to Section 3A as proper medication row with structured dosing per R4/M5 - Added enoxaparin (DVT prophylaxis) to Section 3A with complete dosing and EBP timing precautions per R5/M6 - Fixed gabapentin dosing: single start dose (300 mg qHS) in structured field with titration in full instructions per R6/M2 - Fixed pregabalin dosing: single start dose (75 mg BID) in structured field with titration in full instructions per R7/M3 - Added rebound intracranial hypertension monitoring to Section 6 per R8 - Removed duplicate DVT prophylaxis entry from Section 4C (now covered by enoxaparin in 3A and compression stockings in 4C) per M6 - Updated version to 1.1 in frontmatter, VERSION line, and change log

v1.0 (January 30, 2026) - Initial template creation - Comprehensive diagnostic workup including MRI brain, MRI spine, CT myelography, cisternography, and digital subtraction myelography - Conservative management with IV fluids, caffeine, bed rest, and abdominal binder - Epidural blood patch protocol (blind and targeted) - Pharmacologic adjuncts: theophylline, hydrocortisone, gabapentin, pregabalin - Interventional options: fibrin glue, surgical dural repair, CSF-venous fistula treatment - Subdural hematoma management in SIH context - Lumbar puncture section with low-volume caution - CSF-venous fistula recognition and treatment - Connective tissue disorder screening guidance