2Case Presentation Chief Complaint Pt is a 37 y/o female whopresented to the ED on 2/17/07 with a complaint of a constant pressure like headache which had lasted for the last 2 weeks. The headaches awoke her from sleep.
3History of Present Illness Onset was 2/4/2007.At first got better with OTC medication, now is constant.Pt describes headaches as a tight feeling bifrontal, extending posterior, left side hurts more than right.Exacerbated by light and in the sitting, standing position.Relieved somewhat by lying flat.
4HPI cont. Complains of new onset nausea and vomiting. Patient felt lightheaded for last few days, “nearly walking into walls”.Unable to perform daily activities or go to work.Pt was seen in OSH – SWGH twice during last 2 weeks for same condition.
5Allergies: LatexPMHx: deniesPSHx: C/S twice under spinal.Social Hx: Non-smoker, social drinker, denies drug useTakes no medications.
6In the ED Physical exam: BP 144/81, HR 77, T 96.8, RR 14, 99% RA Gen: Pt lying on cot, cloth covering her face. Slow to move but is A & O x 3. Winced to light being turned on, but no photophobia to fundoscopic exam.Neuro: CN’s normal and intact. 5/5 strength and motor in UE/LE. Movement is slightly slow but not clumsy.Rest of physical exam normal.
7Hospital CourseIV started. 1 liter NS bolus given, second liter running.CBC, BMP and urine Hcg ordered.CT Head without contrast and CT angio of head ordered.Pt given pain meds with slight improvement.
8Pt admitted to 9CDiagnosis of Headache, Pain control and dehydration.Neurology consult requested.
9Differential diagnosis of Acute Headache. MigraineTension HACluster HAStrokeSubarachnoid HemorrhageIntracerebral HemorrhageCerebral InfarctionArterial Dissection – Carotid or VertebralAcute HydrocephalusMeningitisEncephalitisGiant Cell ArteritisTumorTrauma
10Neurology consult Pt seen by neurologist next day. History consistent with that given in ED.No visual disturbancesNo tinnitus or diplopia.No history of trauma.
11Neurology Exam ROS negative Physical Exam WNL Neurologic Exam: A & O x 3 , CN intactMotor, sensation, coordination – WNLReflexes 2+ & symmetricGait normal, no romberg.
12ImagingCT of head – slit like ventricles and low lying cerebellar tonsils. Findings could be either due to pseudotumor cerebri with coexistent Chiari I malformation or CSF hypotension.
13ImagingCT Angio of brain – Negative except for somewhat prominent venous structures supplying the transverse sinuses bilaterally. Findings could be manifestation of CSF hypotension syndrome. Although given the plain CT results pseudotumor cerbri also warrants consideration. Most significantly, no evidence of dural sinus thrombosis.
14Pseudotumor cerebri AKA Benign Intracranial hypertension Defined as a syndrome of elevated ICP without evidence of hydrocephalus, focal lesions or frank brain edema.Usually occurs in obese women between agesSymptoms include HA, visual disturbances, or diplopia secondary to abducens nerve palsies.Fundoscopic exam shows papilledema.
15Pseudotumor cerebri Usually a benign and self limiting disorder. Condition associated with drugs such as Vit A intoxication, Danazol, Accutane, Tetracyclines, oral contraceptives and corticosteroid withdrawal.Systemic disorders include hypoparathyroidism, lupus.
16CT of head can show small ventricles Treatment includes carbonic anhydrase inhibitors – reduce CSF production. Corticosteroids, Lasix.Weight loss.Correct underlying systemic disorder.In the most severe cases Ventriculoperitoneal shunt can be done.
17Radiology Impression: Orthostatic HA suspect spontaneous low CSF pressure HA.Neurology Recommendation: Trial of caffeine 500mg IV. If no relief in 1 hour give 2nd dose. If no relief in AM will do MRI of head.
18MRI of head done – Results show inferior displacement of the brainstem and cerebellum towards the foramen magnum with diffuse dural venous enhancement. Findings most consistant with intracranial hypotension.Neurology recommends epidural blood patch and Anesthesia was consulted.
19Acute pain service saw the patient and recommended conservative treatment at this time. Abdominal binder, caffeine, fluids and percocet for pain.Patient was referred to Chronic pain for followup.
20Patient was discharged on 2/20/07. Instructed to follow with chronic pain and neurology as outpatient.Told to come to ED if symptoms do not improve.
21What is Intracranial Hypotension? Escape of fluid that normally surrounds the brain and spinal cord.Usually characterized by an orthostatic headache – one that worsens with sitting or standing.Headache can be chronic or patient can present without headache.Location of headache varies between patients.
22Headaches in intracranial hypotension OrthostaticCervical or interscapular pain that preceds orthostatic HALingering non orthostatic HAExertional HA wihout orthostatic featuresAcute thunderclap onset of orthostatic HASecond half of day HAParadoxic orthostatic HANo HA
23Cause of Headache CSF supports the brain. Brain weight of 1500gr in air weighs only 48gr in the cranium.Remaining weight supported by pain sensitive structures. Meninges, cerebral and cerebellar veins, CN V, VII, IX and X, upper 3 cervical nerves.CSF decreases – decrease in buoyancy causing traction on these structures.In the upright position the downward displacement is exagerated.
24Evidence of this theory is supported first by study data documenting downward displacement of cranial structures.Evidence in support of this theory comes from data collected by Kunkle, et al who induced postural HA in healthy volunteers by draining CSF. One of his subject had undergone a section of the roots of CN V and IX and upper 4 cervical roots on L side with analgesia in the regions to which these nerves project. This pt experienced HA only on right side.
25Another theory proposes the dilitation of the intracranial vascular structures causes the headaches. This is based on the Monroe-Kellie hypothesis which states that the sum of the volumes of intracranial blood, CSF and brain tissue must remain constant in an intact cranium. According to this hypothesis a loss of CSF will cause an increase in intracranial blood volume.
26Most compensation occurs via venous dilitation due to greater compliance and capacitance. Venous sinus engorgement, pachymeningeal enhancement, subdural effusions and enlargement of pituitary gland may represent compensatory changes to maintain intracranial volume.
27Pain is exacerbated by: LaughingCoughingSneezingJugular Venous CompressionValsalva maneuverAnalgesics have minimal effect and pain is usually relieved with lying flat.
28Other Symptoms: Nausea Vomiting Anorexia Neck pain Dizzyness Diplopia PhotophobiaChanges in hearingUnsteadiness or staggering gaitFacial numbness or weaknessTransient visual obscurationUpper limb radicular symptoms
29Rare symptoms Galactorrhea Stupor Ataxia Parkinsonism Coma Result of compression of pituitary stalk, diencephalon, posterior fossa, and midline structures.
30Etiology of CSF volume depletion True Hypovolemic state – reduced total body waterCSF shunt overdrainageCSF leaks:Traumatic:After definite traumaSpinal tap or epiduralSpinal or cranial surgerySpontaneous:Unknown cause – most commonweakness of the dural sac – ex. Meningeal diverticula or connective tissue abnormalities
31This patient was diagnosed with spontaneous CSF hypotension. Syndrome recognized for more than 55 yearsFirst proposed by Schaltenbrand in 1938 and described as a headache syndrome virtually identical to one following an LP.
32Proposed the following mechanisms: 1. Decreased CSF production2. Increased CSF absorption3. CSF Leakage through small tearsToday the accepted etiology is of CSF leakage.Most occur at the cervicothoracic junction and thoracic levels.
33Can be attributed to minor trauma such as fall, sneezing, sudden twist or stretch. Can cause rupture of preexisting spinal epidural cysts or tarlov cysts or tear in dural nerve sheath.
34Diagnosis CSF analysis – opening pressure can be low – around 60mmhg. Dry taps are encounteredRare instances the was negative pressure – sucking noise as stylet is removed.CSF is typically clear. Protein concentration normal or high. WBC count can be normal or high.Cytology and microbiology are always normalGlucose concentration is never low.
35CT of head is of limited value CT of head is of limited value. It might show subdural fluid collections. Used more to rule out other causes.MRI will show diffuse pachymeningeal enhancement following the administration of gondolinium. This is the most characteristic feature of this syndrome.
36MRI findings in SIH Diffuse pachymeningeal enhancement. Descent of brain – Cerebellar tonsils, obliteration of some subarachnoid cisterns, crowding of post. FossaEnlargement of pituitaryFlattening or tenting of optic chiasmSubdural fluid collectionsEngorged cerebral venous sinusesDecreased size of ventricles
37In the spine – extra arachnoid fluid collections Meningeal diverticulaID of level of leakID of actual leak site – rareEngorgement of epidural veinsSpinal pachymeningeal enhancement
38CT Myelogram – used to demonstrate CSF leaks and is the study of choice to find its location. Radioisotope cisternography – Uses indium-111. introduced intrathecally and its movement is followed by sequential scanning at certain time intervals.Normally by 24 hours radioactivity can be detected over the cerebral convexities.If there is a CSF leak there will be minimal activity.Early accumulation will be detected in the kidneys and bladder
39Treatment of CSF leaksHA usually resolve with conservative treatment within 2-12 days.Bed rest – supine position reduces CSF pressure at leak site and promote meningeal healing.Fluids – increases CSF volume by fluid restoration.Caffeine – Thought to produce arterial vasoconstriction through blockade of adenosine receptors. Intracranial blood flow venous engorgement are decreased.Steroids – effort to restore CSF volume. No evidence indicates that steroids have any effect on CSF Production or absorption.Abdominal binder
40Treatment Other treatment options include: Epidural Blood Patch Continuous epidural saline infusionEpidural infusion of dextranEpidural injection of fibrin glueIntrathecal fluid injectionSurgical correction
41Epidural blood patchAccording to Mokri, epidural blood patch is the treatment of choice in individuals who have failed conservative therapy.Technique introduced by Gormley who observed that the incidence of ICH following a lumbar puncture is less in individuals when the LP is traumatic and bloody.
42Study by Mokri, Sencakova, and McClelland identified 54 patients with SIH. 29 patients received EBP4 Patients were eliminated.Followup was obtained via review of records, correspondence and phone calls.Ages ranged for 18 to women 5 men.
43All patients received EBP using 10 -20ml of blood First EBP 9 out of 25 improved.15 who failed first EBP underwent 2nd.5 reponded well.Of the 10 remaining, 2 were treated surgically. Remainder underwent a 3rd EBP 4 responded well. 4 other underwent surgical correction.
44This technique involves injecting blood into the epidural space. Pt experiences immediate relief due to volume replacement.In spontaneous CSF leaks patients usually require more than one blood patch.Procedure is most effective is done at level of leak.If level unknown – blood can be injected into lumbar space and pt head can be lowered to allow the blood to ascend. Data indicates that this might be effective over 9 spinal segments
45ComplicationsMost common complication is back discomfort at injection site – 30%ParesthesiaRadiculopathyChemical meningitis
46Pt arrives back in ED on 2/28/07 with worsening of HA. Pt is actively vomiting. HA is continuous and getting worse.Pt was compliant with abdominal binder, caffeine, fluids. Pain meds give mild relief.Pt admitted to 9C. Neurology and Anesthesia consulted.
47Anesthesia Acute Pain Service went to evaluate pt. on 9C. Risks and Benefits of epidural blood patch discussed at length. All question were asked and answered.Pt wanted to try a trial of steroids prior to epidural blood patch.
48On 3/2/07 pt was brought to OR for epidural blood patch under fluoroscopy. Epidural space was identified and 3cc of blood was injected.Pt states that she has immediate resolution of symptoms.Post op – pt sitting in bed. Denies HA, denies N/V.Pt discharged home. Follow with neurology and chronic pain as needed.
493/9/07 pt calls MHMC complaining of HA. Pain no longer occipital. Only bifrontal L>R.Has nausea and vomiting.Sees neurology and a 2nd epidural blood patch is recommended. Pt started on steroids – 8mg Decadron for 5 days.
50Repeat CT of head shows cerebellar tonsils inferiorly displaced within the foramen magnum. 4th ventricle appears small. Findings consistent with intracranial hypotension.
51Chronic pain was consulted by neurology. Recommendations by chronic pain were to start cafergot TID, abdominal binder, phenergan for nausea.Will do epidural blood patch – 15ml of blood.If no response, CT myelogram to evaluate were CSF leakage is. Rupture of Tarlov cyst.F/U one week after procedure.
52Epidural blood patch done on 3/19/07. 15ml blood injected in epidural space.Resolution of symptoms.Pt followed up on April 3 with chronic pain.Pts symptoms have improved. Still has a mild HA but is functional.No further treatment needed at this time.If HA returns – pt to undergo CT myelogram.