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Anesthetic Considerations for a Parturient with Arnold-Chiari Type I Malformation and Syringomyelia

Presenter: Jason Jacobs, CA-2 December 21, 2007

Chiari Malformation & Syringomyelia

Chiari Malformation

Cleland 1883 hindbrain herniation and prolapse through the foramen

Four Classes

Type I: most common (CMI) and typically presents in adulthood


Cerebellar tonsil displacement 25% have associated Syringomyelia Cerebellar vermis, brainstem, & 4th ventricle displacement 95% associated with hydrocephalus and myelomeningocele

Type II: typically presents in infancy


Type III:

Herniation into a high cervical myelomeningocele with 4th ventricle hydrocephalus


Cerebellar hypoplasia without herniation

Type IV:

Primary Presenting Symptoms

Severe post occipital HA

(worse with ICP valsalva, exertion, coughing, laughing)


Neck and UE pain, numbness, or tingling Loss of pain and temp sensation Musculoskeletal pain Weakness

Syncope Dizziness Vertigo Tinnitus Nystagmus Fatigue

(Less common: bulbar sx & resp impairment)

Diagnosis

MRI is the diagnostic test of choice

Treatment

ICP management Posterior fossa decompression and duraplasty

Posterior Fossa Decompression

Syringomyelia

Greek (syrinx = tube) (myelos=marrow)

Syringomyelia = Tube in the Marrow

Ollivier dAngers 1827 Syrinx: an abnormal cavitation within the spinal cord

Syringomyelia

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

Others: neoplasm, arachnoiditis, trauma

~84% associated with craniocervical junction malformations

Clinical Presentation

Lower cervical/upper thoracic UE motor/sensory abnormalities


Weakness Pain/temp deficits (touch and proprioception spared) Burning neck and/or back pain Referred chest pain

Syringobulbia: extension into the brainstem

Respiratory Function

Paraspinal weakness kyphoscoliosis restrictive resp. defects Vocal cord abductor paresis

Autonomic involvement

Arrhythmias hemodynamic compromise


Weak CV reflexes? Documented cases of sudden cardiac or resp arrest a

Delayed gastric emptying, urinary bladder dysfunction, and impaired thermoregulation

Diagnosis

MRI is the diagnostic test of choice

Treatment

Goal: correct ASAP posterior fossa decompression and duraplasty without surgical treatment
1/3 symptoms stability 2/3 progressive deteriorationsevere neuro deficits

Before and After

Pregnancy

CMI/Syringomyelia potential risk for increased ICP during pregnancy +/or delivery planned pregnancysafe?...symptoms?

Pregnancy

Mueller: (7 parturients w/ CMI+Syringomyelia)

Effect of pregnancy
Some symptoms at times NO significant symptoms during or after delivery

Effect of epidural

NO complications

Pregnancy

Parker:
CMI + Syringomyelia operative vaginal delivery under epidural NO complications Syringomyelia c-section under epidural NO complications

No ICP vaginal delivery is ok (take precautions!) Uncorrected CMI +/or ICP c-section

Anesthetic Considerations

No firm recommendations to date Detailed pre-anesthetic assessment


Neuro H&P Note altered respiratory fx Note autonomic neuropathy

Avoid ICP

General Anesthesia

Challenges: airway manipulation Avoid sudden ICP close watch on depth/bp during induction/intubation/extubation autonomic dysfunction

Cardio A-line Close temp monitors Prior neuro denervation avoid sux Prior weakness sensitivity to non-depolarizing agents

Paralysis:

General Anesthesia

Sicuranza: corrected CMI GA for c-section

Reasoning:
vaginal delivery compromise neurosurgical repair? neuraxial avoided dural scar tissue interference with epidural cath?

Uncomplicated delivery mother and infant doing well at 6 weeks postop

General Anesthesia

Agusti: corrected CMI and Syringomyelia GA for c-section


NO complications or worsened symptoms Neonatal outcome excellent

(Prolonged neuromuscular blockade noted)

Neuraxial Anesthesia

Strong Contraindications:

Patient refusal Patient inability to maintain still during the needle puncture exposing the neural structures to unacceptable risk of injury Raised ICP which theoretically may predispose to brainstem herniation
Intrinsic and idiopathic Coagulopathy such as that occurring with administration of Coumadin or heparin Skin or soft tissue infection at the proposed site of needle insertion Severe hypovolemia Lack of anesthesiologist experience [often-cited relative contraindication of preexisting neurologic disease is not usually based on medical criteria but rather on legal considerations]

Relative contraindications:

Neuraxial Anesthesia

Advantage: avoid airway manipulation Acceptable option! especially with a tough airway Cranio-spinal pressure dissociation:

Dural puncture relative negative spinal pressure? further tentorial herniation?

Post-dural Puncture?

Barton: case of CMI diagnosed with symptoms after wet tap Hullander: case of CMI presenting as a recurrent spinal headache Chicken or the Egg?

Either way now part of the differential of PDPH

Epidural Anesthesia

Epidural space distention subarachnoid compression? ICP

Hilt: 2 cases of ICP after epidural bolus

Rapid blockade large bp with autonomic neuropathy

Slowly establish blockade!

Epidural Anesthesia

Semple: uncorrected CMI epidural for c-section

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

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

Epidural Anesthesia

Nel: CMI and Syringomyelia epidural for csection

Reasoning:

Risk of aggravation of syrinx c-section

Slow blockade hemodynamic stability Healthy child NO maternal neuro deterioration 6 weeks later

Spinal Anesthesia

Safe when CMI was corrected Herniation: a real threat?

few reports

Spinal Anesthesia

Landau: corrected CMI spinal for c-section

Reasoning:
Hx of c-section c-section Avoid local toxicity and large bore wet tap spinal

Neurosurgical consult: dural puncture shouldnt cause herniation or affect ICP NO complications Maternal neurological stability 1 month later

Spinal Anesthesia

Krzystof: CMI spinal for c-section


CMI was newly diagnosed during her 1st trimester when she was mildly symptomatic NO complications with spinal or delivery NO maternal postpartum symptom exacerbations

Safest Anesthetic Management?

Chantigian: (small series) CMI GA and RA for both vaginal and c-sections
30 deliveries (vaginal x 24 & c-section x 5) 6 Epidural, 2 Single Shot Spinal, 1 Continuous Spinal 3 General Anesthesia (rest of vaginal local + inhalationalall pre-1970)

NO exacerbations NO new neuro sx

Conclusions

CMI and Syringomyelia: varying degrees of craniospinal pressure gradients take caution with ICP Lack of literature no uniform recommendations
vaginal vs cesarean section regional vs general

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

Core Competencies

Patient Care: provided the anesthetic management of a parturient with Chiari Malformation and Syringomyelia Medical Knowledge: in depth review of Chiari Malformation and Syringomyelia specifically with respect to pathophysiology, presentation, and treatment as well as the anesthetic management of a parturient with the above Practice-Based Learning and Improvement: used invasive arterial blood pressure monitoring and ensured adequate depth of anesthesia to avoid increasing ICP Interpersonal and Communication Skills: explained the risks and benefits of both General Anesthesia as well as Regional Anesthesia Professionalism: respect given to the patient and her informed decision making as well as OB support staff Systems-Based Practice: communcation between Obstetrics and Anesthesiology as well as Neurology/Neurosurgery

Reflective Practice

Adequate attention was given to prevention in altered hemodynamics and ICP Earlier communication between Obstetrics and Anesthesia to allow for satisfactory preoperative communication with Neurology Consideration could be given to the possibility of an unanticipated difficult airway usage or close proximity of alternative approach to airway management (eg. fiberoptic, glidescope, etc.)

References

Mueller et al. Chiari I Malformation with or without Syringomyelia and Pregnancy: Case Studies and Review of the Literature. American Journal of Perinatology. 2005 Feb;22(2):67-70. Parker et al. Maternal Arnold-Chiari Type I Malformation and Syringomyelia: A Labor Management Dilemma. American Journal of Perinatology. 2002 Nov;19(8):445-50. Cipolla M. Cerbebrovascular Function in Pregnancy and Eclampsia. Hypertension. 2007;50:14 Miller; Millers Anesthesia 6th ed; Elsevier Inc; 2005; pp. 1654, 2128 Sicuranza et al. Arnold-Chiari Malformation in a Pregnant Woman. Obstetrics and Gynecology. 2003 Nov;102(5 Pt 2):1191-4. Agusti et al. Anesthesia for cesarean section in a patient with Syringomyelia and Arnold-Chiari type I malformation. International Journal of Obstetric Anesthesia. 2004 Apr;13(2):114-6. Hullander et al. Chiari I Malformation presenting as recurrent spinal headache. Anesthesia and Analgesia. 1992 75: 1025-26

References

Barton et al. Oscillopsia and horizontal nystagmus with accelerating slow phases after lumbar puncture in Arnold-Chiari malformation. Annals of Neurology. 1993: 33: 418-21 Semple et al. Arnold-Chiari malformation in pregnancy. Anaesthesia. 1996 Jun;51(6):580-2. Nel et al. Extradural anaesthesia for Caesarean section in a patient with Syringomyelia and Chiari type I anomaly. British Journal of Anaesthesia. 1998; 80: 512-515 Landau et al. Spinal Anesthesia for Cesarean Delivery in a Woman with a Surgically Corrected Type I Arnold Chiari Malformation. Anesthesia and Analgesia. 2003; 97: 253-5 Krzysztof. Spinal anesthesia for Cesarean delivery in a parturient with Arnold-Chiari type I malformation. Canadian Journal of Anesthesia. 2004; 51:639 Chantigian et al. Chiari I Malformation in Parturients. Journal of Clinical Anesthesia. 2002; 14:201-205