Anesthetic Anesthetic Considerations for a Considerations for a

Parturient with Arnold-Parturient with Arnold-Chiari Type I Chiari Type I

Malformation and Malformation and SyringomyeliaSyringomyelia

Presenter: Jason Jacobs, CA-2Presenter: Jason Jacobs, CA-2

December 21, 2007December 21, 2007

Chiari Chiari Malformation & Malformation & SyringomyeliaSyringomyelia

Chiari MalformationChiari Malformation

Cleland – 1883 Cleland – 1883 hindbrain herniation and prolapse hindbrain herniation and prolapse

through the foramenthrough the foramen

Four ClassesFour Classes Type I: most common (CMI) and typically Type I: most common (CMI) and typically

presents in adulthoodpresents in adulthood Cerebellar tonsil displacementCerebellar tonsil displacement 25% have associated Syringomyelia25% have associated Syringomyelia

Type II: typically presents in infancyType II: typically presents in infancy Cerebellar vermis, brainstem, & 4th ventricle Cerebellar vermis, brainstem, & 4th ventricle

displacementdisplacement 95% associated with hydrocephalus and 95% associated with hydrocephalus and

myelomeningocelemyelomeningocele Type III: Type III:

Herniation into a high cervical myelomeningocele Herniation into a high cervical myelomeningocele with 4th ventricle hydrocephaluswith 4th ventricle hydrocephalus

Type IV: Type IV: Cerebellar hypoplasia without herniationCerebellar hypoplasia without herniation

Primary Presenting Primary Presenting SymptomsSymptoms

Severe post occipital HA Severe post occipital HA (worse with (worse with ↑↑ ICP ICP valsalva, valsalva,

exertion, coughing, laughing)exertion, coughing, laughing) Neck and UE pain, numbness, Neck and UE pain, numbness,

or tinglingor tingling Loss of pain and temp Loss of pain and temp

sensationsensation Musculoskeletal painMusculoskeletal pain WeaknessWeakness

(Less common: bulbar sx & resp (Less common: bulbar sx & resp impairment)impairment)

SyncopeSyncope DizzinessDizziness VertigoVertigo TinnitusTinnitus Nystagmus Nystagmus FatigueFatigue

DiagnosisDiagnosis

MRI is the diagnostic test of choice MRI is the diagnostic test of choice

TreatmentTreatment

ICP managementICP management Posterior fossa decompression and Posterior fossa decompression and

duraplasty duraplasty

Posterior Fossa Posterior Fossa DecompressionDecompression

SyringomyeliaSyringomyelia Greek (syrinx = tube) (myelos=marrow)Greek (syrinx = tube) (myelos=marrow)

Syringomyelia = “Tube in the Marrow”Syringomyelia = “Tube in the Marrow” Ollivier d’Angers – 1827 Ollivier d’Angers – 1827 Syrinx: an abnormal cavitation within the Syrinx: an abnormal cavitation within the

spinal cordspinal cord

SyringomyeliaSyringomyelia

Prevalence: ~8 per 100,000 peoplePrevalence: ~8 per 100,000 people Most common cause is CMI Most common cause is CMI

Others: neoplasm, arachnoiditis, traumaOthers: neoplasm, arachnoiditis, trauma ~84% associated with craniocervical ~84% associated with craniocervical

junction malformations junction malformations

Clinical PresentationClinical Presentation

Lower cervical/upper thoracic Lower cervical/upper thoracic UE UE motor/sensory abnormalitiesmotor/sensory abnormalities WeaknessWeakness Pain/temp deficits (Pain/temp deficits (touch and touch and

proprioception spared)proprioception spared) Burning neck and/or back pain Burning neck and/or back pain Referred chest painReferred chest pain

Syringobulbia: extension into the Syringobulbia: extension into the brainstembrainstem

Respiratory FunctionRespiratory Function

Paraspinal weakness Paraspinal weakness kyphoscoliosis kyphoscoliosis restrictive resp. restrictive resp. defectsdefects

Vocal cord abductor paresisVocal cord abductor paresis

Autonomic involvementAutonomic involvement

Arrhythmias Arrhythmias hemodynamic hemodynamic compromisecompromise Weak CV reflexes?Weak CV reflexes? Documented cases of sudden cardiac or Documented cases of sudden cardiac or

resp arrest aresp arrest a Delayed gastric emptying, urinary Delayed gastric emptying, urinary

bladder dysfunction, and impaired bladder dysfunction, and impaired thermoregulationthermoregulation

DiagnosisDiagnosis

MRI is the diagnostic test of choiceMRI is the diagnostic test of choice

TreatmentTreatment

Goal: correct ASAPGoal: correct ASAP posterior fossa decompression and posterior fossa decompression and

duraplastyduraplasty without surgical treatment without surgical treatment

1/3 – symptoms stability1/3 – symptoms stability 2/3 – progressive deterioration…severe 2/3 – progressive deterioration…severe

neuro deficitsneuro deficits

Before and AfterBefore and After

PregnancyPregnancy

CMI/Syringomyelia CMI/Syringomyelia potential risk potential risk for increased ICP during pregnancy for increased ICP during pregnancy +/or delivery+/or delivery

planned pregnancy…planned pregnancy…safe?...symptoms?safe?...symptoms?

PregnancyPregnancy

Mueller: (7 parturients w/ Mueller: (7 parturients w/ CMI+Syringomyelia)CMI+Syringomyelia) Effect of pregnancyEffect of pregnancy

Some Some ↑ ↑ symptoms at timessymptoms at times NO significant NO significant ↑↑ symptoms during or after symptoms during or after

deliverydelivery Effect of epiduralEffect of epidural

NO complications NO complications

PregnancyPregnancy

Parker: Parker: CMI + Syringomyelia CMI + Syringomyelia operative vaginal operative vaginal

delivery under epidural delivery under epidural NO complications NO complications Syringomyelia Syringomyelia c-section under epidural c-section under epidural

NO complicationsNO complications No No ↑↑ ICP ICP vaginal delivery is ok ( vaginal delivery is ok (take take

precautions!)precautions!) Uncorrected CMI +/or Uncorrected CMI +/or ↑↑ ICP ICP c- c-

sectionsection

Anesthetic Anesthetic Considerations Considerations

No firm recommendations to dateNo firm recommendations to date Detailed pre-anesthetic assessment Detailed pre-anesthetic assessment

Neuro H&PNeuro H&P Note altered respiratory fxNote altered respiratory fx Note autonomic neuropathyNote autonomic neuropathy

Avoid Avoid ↑↑ ICP ICP

General AnesthesiaGeneral Anesthesia

Challenges: airway manipulationChallenges: airway manipulation Avoid sudden Avoid sudden ↑↑ ICP ICP close watch on close watch on

depth/bp during depth/bp during induction/intubation/extubationinduction/intubation/extubation

autonomic dysfunction autonomic dysfunction Cardio Cardio A-line A-line Close temp monitorsClose temp monitors

Paralysis:Paralysis: Prior neuro denervation Prior neuro denervation avoid sux avoid sux Prior weakness Prior weakness sensitivity to non- sensitivity to non-

depolarizing agents depolarizing agents

General AnesthesiaGeneral Anesthesia

Sicuranza: corrected CMI Sicuranza: corrected CMI GA for GA for c-sectionc-section Reasoning:Reasoning:

vaginal delivery vaginal delivery compromise compromise neurosurgical repair? neurosurgical repair?

neuraxial avoided neuraxial avoided dural scar tissue dural scar tissue interference with epidural cath?interference with epidural cath?

Uncomplicated deliveryUncomplicated delivery mother and infant doing well at 6 weeks mother and infant doing well at 6 weeks

postoppostop

General AnesthesiaGeneral Anesthesia

Agusti: corrected CMI and Agusti: corrected CMI and Syringomyelia Syringomyelia GA for c-section GA for c-section NO complications or worsened NO complications or worsened

symptomssymptoms Neonatal outcome excellent Neonatal outcome excellent

(Prolonged neuromuscular blockade (Prolonged neuromuscular blockade noted)noted)

Neuraxial AnesthesiaNeuraxial Anesthesia Strong Contraindications:Strong Contraindications:

Patient refusalPatient refusal Patient inability to maintain still during the needle Patient inability to maintain still during the needle

puncture exposing the neural structures to unacceptable puncture exposing the neural structures to unacceptable risk of injury risk of injury

Raised ICP which theoretically may predispose to Raised ICP which theoretically may predispose to brainstem herniationbrainstem herniation

Relative contraindications: Relative contraindications: Intrinsic and idiopathic Coagulopathy such as that Intrinsic and idiopathic Coagulopathy such as that

occurring with administration of Coumadin or heparinoccurring with administration of Coumadin or heparin Skin or soft tissue infection at the proposed site of needle Skin or soft tissue infection at the proposed site of needle

insertioninsertion Severe hypovolemiaSevere hypovolemia Lack of anesthesiologist experience Lack of anesthesiologist experience [often-cited relative contraindication of preexisting [often-cited relative contraindication of preexisting

neurologic disease is not usually based on medical criteria neurologic disease is not usually based on medical criteria but rather on legal considerations]but rather on legal considerations]

Neuraxial AnesthesiaNeuraxial Anesthesia

Advantage: avoid airway Advantage: avoid airway manipulationmanipulation

Acceptable option! Acceptable option! especially with especially with a tough airwaya tough airway

Cranio-spinal pressure dissociation: Cranio-spinal pressure dissociation: Dural puncture Dural puncture relative negative relative negative

spinal pressure? spinal pressure? further tentorial further tentorial herniation?herniation?

Post-dural Puncture?Post-dural Puncture?

Barton: case of CMI diagnosed with Barton: case of CMI diagnosed with symptoms after “wet tap”symptoms after “wet tap”

Hullander: case of CMI presenting Hullander: case of CMI presenting as a recurrent spinal headacheas a recurrent spinal headache

Chicken or the Egg?Chicken or the Egg? Either way Either way now part of the now part of the

differential of PDPHdifferential of PDPH

Epidural AnesthesiaEpidural Anesthesia

Epidural space distention Epidural space distention subarachnoid compression? subarachnoid compression? ↑↑ ICP ICP Hilt: 2 cases of Hilt: 2 cases of ↑ ↑ ICP after epidural ICP after epidural

bolusbolus Rapid blockade Rapid blockade large large ↓bp ↓bp with with

autonomic neuropathyautonomic neuropathy

Slowly establish blockade!Slowly establish blockade!

Epidural AnesthesiaEpidural Anesthesia Semple: uncorrected CMI Semple: uncorrected CMI epidural epidural

for c-sectionfor c-section Reasoning:Reasoning:

Pt had pre-eclampsia Pt had pre-eclampsia c-section c-section Hx of difficult intubation Hx of difficult intubation epidural epidural

Healthy female Healthy female APGAR: 8/9 APGAR: 8/9 Mother and infant with uncomplicated Mother and infant with uncomplicated

hospital stayhospital stay

Epidural AnesthesiaEpidural Anesthesia

Nel: CMI and Syringomyelia Nel: CMI and Syringomyelia epidural for c-section epidural for c-section Reasoning:Reasoning:

Risk of aggravation of syrinx Risk of aggravation of syrinx c-section c-section Slow blockade Slow blockade hemodynamic stability hemodynamic stability Healthy childHealthy child NO maternal neuro deterioration 6 NO maternal neuro deterioration 6

weeks laterweeks later

Spinal AnesthesiaSpinal Anesthesia Safe when CMI was correctedSafe when CMI was corrected Herniation: a real threat?Herniation: a real threat?

few reportsfew reports

Spinal AnesthesiaSpinal Anesthesia

Landau: corrected CMI Landau: corrected CMI spinal for spinal for c-sectionc-section Reasoning:Reasoning:

Hx of c-section Hx of c-section c-section c-section Avoid local toxicity and large bore “wet tap” Avoid local toxicity and large bore “wet tap”

spinal spinal Neurosurgical consult: dural puncture Neurosurgical consult: dural puncture

shouldn’t cause herniation or affect ICPshouldn’t cause herniation or affect ICP NO complications NO complications Maternal neurological stability 1 month Maternal neurological stability 1 month

later later

Spinal AnesthesiaSpinal Anesthesia

Krzystof: CMI Krzystof: CMI spinal for c-section spinal for c-section CMI was newly diagnosed during her CMI was newly diagnosed during her

1st trimester when she was mildly 1st trimester when she was mildly symptomaticsymptomatic

NO complications with spinal or NO complications with spinal or delivery delivery

NO maternal postpartum symptom NO maternal postpartum symptom exacerbations exacerbations

Safest Anesthetic Safest Anesthetic Management? Management?

Chantigian: (small series) CMI Chantigian: (small series) CMI GA and GA and RA for both vaginal and c-sectionsRA for both vaginal and c-sections 30 deliveries (vaginal x 24 & c-section x 5)30 deliveries (vaginal x 24 & c-section x 5) 6 Epidural, 2 Single Shot Spinal, 1 Continuous 6 Epidural, 2 Single Shot Spinal, 1 Continuous

SpinalSpinal 3 General Anesthesia3 General Anesthesia

(rest of vaginal (rest of vaginal local + inhalational…all pre- local + inhalational…all pre-1970)1970)

NO exacerbationsNO exacerbations NO new neuro sxNO new neuro sx

ConclusionsConclusions

CMI and Syringomyelia: varying CMI and Syringomyelia: varying degrees of craniospinal pressure degrees of craniospinal pressure gradients gradients take caution with ICP take caution with ICP

Lack of literature Lack of literature no uniform no uniform recommendationsrecommendations vaginal vs cesarean sectionvaginal vs cesarean section regional vs generalregional vs general

Decisions should be Decisions should be interdisciplinary: interdisciplinary: Anesthesiology, Anesthesiology, Neurology, Neurosurgery, ObstetricsNeurology, Neurosurgery, Obstetrics

Core CompetenciesCore Competencies Patient CarePatient Care: provided the anesthetic management of a : provided the anesthetic management of a

parturient with Chiari Malformation and Syringomyeliaparturient with Chiari Malformation and Syringomyelia Medical KnowledgeMedical Knowledge: in depth review of Chiari : in depth review of Chiari

Malformation and Syringomyelia specifically with Malformation and Syringomyelia specifically with respect to pathophysiology, presentation, and treatment respect to pathophysiology, presentation, and treatment as well as the anesthetic management of a parturient as well as the anesthetic management of a parturient with the abovewith the above

Practice-Based Learning and ImprovementPractice-Based Learning and Improvement: used : used invasive arterial blood pressure monitoring and ensured invasive arterial blood pressure monitoring and ensured adequate depth of anesthesia to avoid increasing ICP adequate depth of anesthesia to avoid increasing ICP

Interpersonal and Communication SkillsInterpersonal and Communication Skills: explained : explained the risks and benefits of both General Anesthesia as well the risks and benefits of both General Anesthesia as well as Regional Anesthesia as Regional Anesthesia

ProfessionalismProfessionalism: respect given to the patient and her : respect given to the patient and her informed decision making as well as OB support staffinformed decision making as well as OB support staff

Systems-Based PracticeSystems-Based Practice: communcation between : communcation between Obstetrics and Anesthesiology as well as Obstetrics and Anesthesiology as well as Neurology/NeurosurgeryNeurology/Neurosurgery

Reflective PracticeReflective Practice Adequate attention was given to prevention Adequate attention was given to prevention

in altered hemodynamics and ICPin altered hemodynamics and ICP Earlier communication between Obstetrics Earlier communication between Obstetrics

and Anesthesia to allow for satisfactory pre-and Anesthesia to allow for satisfactory pre-operative communication with Neurologyoperative communication with Neurology

Consideration could be given to the Consideration could be given to the possibility of an unanticipated difficult airway possibility of an unanticipated difficult airway usage or close proximity of alternative usage or close proximity of alternative approach to airway management (eg. approach to airway management (eg. fiberoptic, glidescope, etc.) fiberoptic, glidescope, etc.)

ReferencesReferences Mueller et al. Chiari I Malformation with or without Mueller et al. Chiari I Malformation with or without

Syringomyelia and Pregnancy: Case Studies and Review of the Syringomyelia and Pregnancy: Case Studies and Review of the Literature. Literature. American Journal of PerinatologyAmerican Journal of Perinatology. 2005 . 2005 Feb;22(2):67-70. Feb;22(2):67-70.

Parker et al. Maternal Arnold-Chiari Type I Malformation and Parker et al. Maternal Arnold-Chiari Type I Malformation and Syringomyelia: A Labor Management Dilemma. Syringomyelia: A Labor Management Dilemma. American American Journal of PerinatologyJournal of Perinatology. 2002 Nov;19(8):445-50. . 2002 Nov;19(8):445-50.

Cipolla M. Cerbebrovascular Function in Pregnancy and Cipolla M. Cerbebrovascular Function in Pregnancy and Eclampsia. Hypertension. 2007;50:14Eclampsia. Hypertension. 2007;50:14

Miller; Miller; Miller’s Anesthesia 6th edMiller’s Anesthesia 6th ed; Elsevier Inc; ©2005; pp. ; Elsevier Inc; ©2005; pp. 1654, 21281654, 2128

Sicuranza et al. Arnold-Chiari Malformation in a Pregnant Sicuranza et al. Arnold-Chiari Malformation in a Pregnant Woman. Woman. Obstetrics and GynecologyObstetrics and Gynecology. 2003 Nov;102(5 Pt . 2003 Nov;102(5 Pt 2):1191-4.2):1191-4.

Agusti et al. Anesthesia for cesarean section in a patient with Agusti et al. Anesthesia for cesarean section in a patient with Syringomyelia and Arnold-Chiari type I malformation. Syringomyelia and Arnold-Chiari type I malformation. International Journal of Obstetric AnesthesiaInternational Journal of Obstetric Anesthesia. 2004 . 2004 Apr;13(2):114-6. Apr;13(2):114-6.

Hullander et al. Chiari I Malformation presenting as recurrent Hullander et al. Chiari I Malformation presenting as recurrent spinal headache. spinal headache. Anesthesia and AnalgesiaAnesthesia and Analgesia. 1992 75: 1025-26. 1992 75: 1025-26

ReferencesReferences Barton et al. Oscillopsia and horizontal nystagmus with Barton et al. Oscillopsia and horizontal nystagmus with

accelerating slow phases after lumbar puncture in Arnold-accelerating slow phases after lumbar puncture in Arnold-Chiari malformation. Chiari malformation. Annals of NeurologyAnnals of Neurology. 1993: 33: 418-. 1993: 33: 418-2121

Semple et al. Arnold-Chiari malformation in pregnancy. Semple et al. Arnold-Chiari malformation in pregnancy. AnaesthesiaAnaesthesia. 1996 Jun;51(6):580-2.. 1996 Jun;51(6):580-2.

Nel et al. Extradural anaesthesia for Caesarean section in Nel et al. Extradural anaesthesia for Caesarean section in a patient with Syringomyelia and Chiari type I anomaly. a patient with Syringomyelia and Chiari type I anomaly. British Journal of AnaesthesiaBritish Journal of Anaesthesia. 1998; 80: 512-515. 1998; 80: 512-515

Landau et al. Spinal Anesthesia for Cesarean Delivery in a Landau et al. Spinal Anesthesia for Cesarean Delivery in a Woman with a Surgically Corrected Type I Arnold Chiari Woman with a Surgically Corrected Type I Arnold Chiari Malformation. Malformation. Anesthesia and AnalgesiaAnesthesia and Analgesia. 2003; 97: 253-5. 2003; 97: 253-5

Krzysztof. Spinal anesthesia for Cesarean delivery in a Krzysztof. Spinal anesthesia for Cesarean delivery in a parturient with Arnold-Chiari type I malformation. parturient with Arnold-Chiari type I malformation. Canadian Journal of AnesthesiaCanadian Journal of Anesthesia. 2004; 51:639. 2004; 51:639

Chantigian et al. Chiari I Malformation in Parturients. Chantigian et al. Chiari I Malformation in Parturients. Journal of Clinical AnesthesiaJournal of Clinical Anesthesia. 2002; 14:201-205. 2002; 14:201-205