Article Type: Case Report
Received : October 01, 2018
Accepted : October 06, 2018
Published : October 12, 2018
*Correspondence to: A. Mehra*, Senior Resident, Department of Anaesthesiology and Intensive care, Maulana Azad Medical College and
associated Lok Nayak hospital, New Delhi, India, email:firstname.lastname@example.org
Article Type: Case Report
Received : October 01, 2018
Accepted : October 06, 2018
Published : October 12, 2018
Syringomyelia is an unusual neurological condition characterised by the presence of fluid filled cystic cavity or syrinx within the spinal cord resulting in neurological manifestations with male preponderance. Objectives in the anaesthetic management of these patients is to avoid factors increasing intracranial pressure (ICP) as even in absence of clinical or radiological findings, it can be assumed that intracranial pressure is relatively high which is the basis of pathophysiology of formation of syrinx.Here we present a 27 years old parturient, a known case of cervical syringemyelia who underwent successful emergency LSCS under general anaesthesia.
Keywords: Parturient, cervical syringomyelia, general anaesthesia,caesarean section, obstetric.
Syringomyelia has been used to refer to a syndrome caused by cavitation of the spinal cord adjacent to the central canal, or to any cyst of the spinal cord or dilatation of the central canal and/or its extension into the cord substance. It has a prevalence of 8.4 per 100,000 with male preponderance in the third or fourth decade of life.Syringomyelia has been divided into two groups, communicating with CSF pathways (communicating syringomyelia) and non-communicating. The communicating form commonly associated with Arnold-Chiari Malformation type I (ACM I)whereas non communicating syrinx can developed following cord hematoma, ischemia, venous obstruction, shearing stress with mechanical disruption and proteinaceous fluid secretion. It may also be idiopathic[5, 6]. Most common site is lower cervical or upper thoracic region with gradual expansion up to the medulla (syringobulbia).The symptoms and signs of syringomyelia are related to the location, size, and extent of the cavity and usually consist of any combination of motor, sensory and autonomic dysfunction according to progression of disease.
Objectives in the anaesthetic management of these patients are proper preanaesthetic check-up for typical history and examination to elicit any preoperative motor, sensory and autonomic dysfunction, and any associated anomalies. Intraoperative goal is directed to avoid factors increasing intracranial pressure (ICP), avoidance of succinylcholine,use of no depolarizing neuromuscular blocker with neuromuscular monitoring, avoidance of spinal anaesthesia, proper positioning and care of the skin with tropic ulcers and joints with osteoarthritic changes, anticipation of difficult airway due to deformed joints, kyphoscoliosis and other causes, providing optimal ventilation[7-11]. We report successful anesthetic management of a 27 years old primigravida, known case of cervical syringemyelia who presented for emergency lower segment caesarean section(LSCS).
A 27 years old parturient with 38 wks of gestation anda known case of cervical syringomyelia presented to obstetric emergency in labor. She was planned for emergency LSCS as it was advised by neurologistto avoid normal vaginal delivery to eliminate risk of increase in ICP by mother’s bearing down effort.
On preanaesthetic check-up (PAC) patient gave history of stiffness and numbness in both upper and lower limbs with weakness in right upper limb for past three years for which she had consulted neurologist andafter investigations including MRI spine was diagnosed as a case of cervical syringomyelia. She was started on oral Gabapentin, calcium and multivitamins two years ago for symptomatic improvementwhich was stopped at advent of pregnancy. Following stoppage of treatment there was increase in weakness and numbness of all the four limbs. No surgical intervention was ever made for the treatment of syringomyelia.She also gave history of cervical lymphnode tuberculosis 2 years back for which she took antitubercular drug therapy (ATT) for 1 year. She also had productive cough for last 2-3 days at presentation. Rest of the past medical and surgical history was not significant. Airway examination, general and systemic examination revealed crepitation in bilateral middle and lower zone of chest on auscultation. Neurological examination revealed decreased pain and temperature sensation in bilateral upper limbs from dermatome C4 to T1 and right lower limb from dermatome L2 to L5. Muscle power was grade 4/5 in right upper limb and 5/5 in other limbs. Reflexes were intact in all the limbs. Spine examination appeared normal.
After taking appropriate consent patient was shifted to operating room. Standard monitoring including ECG, pulseoximetry, temperature and non invasive automated blood pressure was initiated. An 18 gauge peripheral i.v. cannula was secured and 500ml of Ringers Lactate was given as preload.General anaesthesia with modified rapid sequence intubation (RSI)was doneusinginjection thiopentone 250 mg androcuronium 60 mg i.v. followed by intubation under direct laryngoscopy (Cormack Lehane grade I) with 7.5 mminternal diameter endotracheal tube (ETT). Anaesthesia was maintained using O2:N2O in ratio of 1:1 with Isoflurane of ≤0.4% volume concentration. After delivery of baby fentanyl 100 µg,dexamethasone 8 mg , diclofenac 75 mg, paracetamol 1 g, ondansetrone 4 mg and slow i.v. infusion ofoxytocin15 IU were given. There was minimal cardiovascular response to tracheal intubation or surgical stimulation. Normocapnia was maintained throughout the procedure with a peak airway pressure < 15 cmH2O. PEEP of 5cmH2O was applied. Transverse abdominis plane (TAP) block with 5ml 1% lignocaine and 10ml 0.25% bupivacaine was given on each side.Reversal was done with injection neostigmine 3 mg i.v.andglycopyrrolate 0.6 mg i.v.and patient was extubated.She was shifted to intensive care unit (ICU) for postoperative close monitoring.Both periperative and postoperative period were uneventful. Postoperative analgesia consisted of diclofenac 75mg 8 hourly and tramadol 50mg as required.Patient was shifted to postnatal ward next day evening and dischargedon fourth day after surgery with advice to follow in obstetric as well as neurology OPD. There was no deterioration of neurological signs and symptoms till the time of discharge of patient from hospital.
Syringomyelia has been used to refer to a syndrome caused by cavitation of the spinal cord adjacent to the central canal, or to any cyst of the spinal cord or dilatation of the central canal and/or its extension into the cord substance. Ollivies d’ Angers (1827) coined the term syringomyelia from two Greek words meaning “channel” and “marroin”. It has a prevalence of 8.4 per 100,000 and occurs more frequently in men than women in the third or fourth decade of life. Rarely, it may develop in childhood or late adulthood.Syringomyelia has been divided into two groups, communicating with CSF pathways (communicating syringomyelia) and that do not communicate (non-communicating). The communicating form is the most common where continuity between syrinx and subarachnoid space and its CSF exists and most of them are linked to congenital or acquired anamolies involving foramen magnum of which Arnold-Chiari Malformation type I (ACM I) is the commonest. Non communicating syrinx developed anywhere in the cord and factors involved include hematoma, ischemia, venous obstruction, shearing stress with mechanical disruption and proteinaceous fluid secretion. It may also be idiopathic[5, 6].
The pathophysiology of syringomyelia is still debated. Most people agree that the syrinx is subject to continuous pulsatile pressure from outside the spinal cord and to sudden rises in pressure associated with coughing, sneezing, and Valsalva maneuvers. Shunting the contents of the syrinx to a low-pressure extraspinal site offers the best chance of maintaining the pressure within the syrinx at a reduced level and dissipating the effects of sudden pressure changes. There is clinically variable presentation in syringomyelia. Cord cavitation causes slow but relentlessly progressive symptoms and signs, depending on its location and extent.
Syrinx typically arises in the lower cervical or upper thoracic region with very gradual expansion, initially into the anterior and lateral cord. Extension upwards into the medulla often occurs (syringobulbia), whereas involvement of the lumbar region of the spinal cord is uncommon.The symptoms may consist of any combination of motor and sensory dysfunction. Disassociated sensory findings of impaired pain and temperature with the preservation of light touch sensation with “cape-like” suspended sensory loss are the classic presentation. The earliest sensory changes usually affect the hands along with neck, shoulders, upper chest, and back. Motor dysfunction often accompanies the sensory loss, resulting in progressive atrophy of the musculature of the upper extremities. This often manifests in skeletal malformations, principally kyphoscoliosis. The progressive atrophy is seen with other lower motor neuron findings such as muscle fasciculation and areflexia. Later in the disease course, upper motor neuron signs arise in the legs due to involvement of the lateral columns of the spinal cord. The last neurologic functions to be lost are vibratory and position senses in the lower extremities, which occur after the syrinx extends to affect the posterior columns of the spinal cord. Less common manifestations include preganglionic Horner syndrome secondary to involvement of the intermediolateral cell column of the lower cervical and first thoracic segment of the spinal cord containing sympathetic neurons.
Various problems may arise during anesthetic management of these patients includingdamage to the spinal cord due to raised intracranial pressure, tachyarrhythmias, widely fluctuating arterial pressure, and sudden cardiac or respiratory arrest due to abnormalities in the autonomic nervous system.Ventilation-perfusion abnormalities if respiratory muscles are involved, difficult venous access due to trophic lesions in the skin, exaggerated responses to nondepolarising muscle relaxants, impaired thermal regulation, and exaggerated hyperkalemia on use of succinylcholine are also seen. Patient requires careful positioning of the patient due to disorganised joints and fixed flexion deformities[7-13].The physiological changes and medical interventions experienced during labour and deliveryexposes the mother to the risk of neurological deterioration.
There are very few case reports on anesthetic management in patients with syringomyelia and here we report our case with a successful outcome. Preferred anesthetic technique for patients with syringomyelia is general anesthesia with avoidance of cerebrospinal fluid (CSF) pressure fluctuations and intracranial pressure (ICP) elevations. Even in patients without clinical or radiological findings, it can be assumed that intracranial CSF pressure is relatively high as it is fundamental to the pathophysiology of syringomyelia[10, 11]. Symptoms worsen when CSF pressure fluctuates, for example during coughing/straining and in active labour with mother’s bearing down effort.Spinal anaesthesia is best avoided in the presence of co-existing Chiari malformations, as there are case reports which described the onset of signs and symptoms up totwo weeks after dural puncture[10, 11].Due to autonomic dysfunction the vasodilatory effects of regional anaesthesia are poorly tolerated and likewise the vasodilatation and cardiac depression of most general anaesthetic agents may be deleterious. Body temperature should be closely monitored especially if autonomic dysfunction is suspected.
Our patient was a known case of cervical syringoyelia, not on any treatment sinceadvent of pregnancy,with history of weakness and numbness in all four limbs with upper respiratory tract infection.General anaesthesia was given with modified RSI using thiopentone and rocuronium with ETT intubation with continuous vigilance for perioperative events. We used rocuronium to facilitate rapid intubation of the trachea as succinylcholine is associated with hyperkalaemia when given to patients with enervated muscles. Our patient demonstrated a normal return to full muscle function following a standard induction dose of rocuronium. Normocapnia was maintained and high airway pressures were avoided to prevent any rise in intracranial pressure.
We avoided spinal anaesthesia to avoid risk of worsening of syrinx. Epidural anaesthesia could be another choice but was avoided for fear of inadvertent dural puncture which could have lead to worsening of patient’s condition.
We conclude that general anaesthesia with RSI using Injection rocuronium can be administered safely in a known case of cervical syringomyelia undergoing emergency LSCS under close perioperative monitoring and avoiding any increase in intracranial pressure.
1. Chen JK, Chen CH, Lee CL, Chen TW, Weng MC, et al. (2004) acute idiopathic syringomyelia: a case report.
Kaohsiung J Med Sci August 20(8): 404-9.
2. Madsen PW, Yezierski RP, Holets VR. (1994) Syringomyelia: clinical observations and experimental studies.
J Neurotrauma; 11:241-54.
3. Brewis M, Poskanzer DC, Rolland C. (1966) Neurological disease in an English city. Acta Neurol Scand; 42:1-89.
4. Williams B. (1969) the distending force in the production of “communicating syringomyelia”. Lancet; 2:189-93.
5. Walton JN. (1985) Brain’s disease of the Nervous System, 9th Ed, Oxford, Oxford University Press,412-16.
6. Williams B. (1980) on the pathogenesis of syringomyelia: a review. J R Soc Med; 73:798-806.
7. Jayaraman L, Sethi N, Sood J. (2011) Anaesthesia for Caesarean Section in a Patient with Lumbar Syringomyelia.
Rev Bras Anestesiol. July-August, 61(4): 469-73.
8. Balaji K, Pratap R, Vijayalaxmi G, Chakravarthy P K, Nagaraju J. |(2013) Anaesthetic management in a patient with Arnold-Chiari
malformation type I and syringomyelia. Journal of Evolution of Medical and Dental Sciences. April 29, 2(17): 2908-11.
9. Mustapha B, Chkoura K, ELhassani M, Ahtil R, Azendour H, et al. (2011) Difficult intubation in a parturient with syringomyelia and
Arnold-Chiari malformation: Use of AirtaqTM laryngoscope. Saudi J Anaesth; 5:419-22.
10. Hullander RM, Bogard TD, Leivers D, Moran D, Dewan DM. (1992) Chiari I malformation presenting as recurrent spinal headache.
Anesthesia and Analgesia; 75:1025-29.
11. Barton JJS, Sharpe JA. (1993) Oscillopsia and horizontal nystagmus with accelerating slow phases following lumbar puncture in the
Arnold-Chiari malformation. Annals of Neurology; 33:418-21.
12. Nawaz Y, McAtamney D. (2010) Anaesthetic Management of Caesarean Section ina Patient with Syringomyelia.
The Internet Journal of Anaesthesiology; 28(2):1-5.
13. Cooperman LH. (1970) Succinylcholine induced hyperkalaemia in neuromuscular disease. JAMA; 213:1867-71.