Kumari H*, Pardeshi S**, Gupta AS***.
(* Junior resident, ** Assistant Professor, *** Professor, Department of Obstetrics & Gynecology, Seth G S Medical College and K E M Hospital, Parel, Mumbai, India).
Syringomyelia is a rare neurological condition in which there is cyst formation within the spinal cord. If the cyst expands it compresses the nerve tissue and patient becomes symptomatic with features like progressive pain, stiffness and weakness, loss of temperature sensation, etc. Causes of Syringomyelia include trauma, meningitis, hemorrhage, tumor, and arachnoiditis. Syringomyelia is also found associated with Arnold Chiari type 1 malformation. Treatment of syringomyelia depends on cause.
Syringomyelia prevalence is about 8.4 cases per 1 lakh population. It is more common in males after 40 years. In pregnancy with syringomyelia, the main challenge is labor analgesia, anesthesia for cesarean section and immobility of the patient. Here we present a case of syringomyelia who had previous two LSCS and partial motor deficit of lower limbs.
A forty year old female, in her sixth pregnancy with two living issues, one intrauterine fetal death and two MTP’s was admitted for elective lower segment cesarean section in view of previous two lower segment cesarean sections. She was admitted at 38.3 weeks of gestation.
She was a known case of syringomyelia (post traumatic) with lumbar disc prolapse and hyperthyroidism. She had a fall in 2005, which resulted in a fracture of D12 vertebra and she was advised bed rest. However her symptoms did not get relieved and hence MRI was done which was suggestive of disc prolapse at the level of L4-L5. She underwent a laminectomy for the same. In 2008, she had a fall again with right intertrochanteric fracture of femur for which she underwent ORIF (open reduction and internal fixation). Subsequently she developed weakness in the lower limbs and a repeat MRI was performed which showed a syrinx from D2-3 to D9. She was advised physiotherapy and vocational therapy.
During the same time frame, she was also diagnosed with hyperthyroidism having toxic and visual symptoms and anterior neck swelling. She was started on tablet carbimazole 60 mg. During pregnancy dose was tapered to 10 mg with which she had a normal thyroid profile.
She was admitted for elective lower segment cesarean section. Orthopedic and endocrine opinion was taken. Neurologist was consulted and she had bilateral upper limb power of 5/5, right lower limb power was 3/5, and left lower limb power was also 3/5. Fitness for general anesthesia was given and avoidance of spinal anesthesia was advised as syringomyelia had involved lumbar region. She underwent elective lower segment cesarean section under general anesthesia. She tolerated the procedure well. She delivered a male baby with an Apgar score of 9/10. She was discharged on D5 of post surgery. Her subsequent recovery was unremarkable.
Syringomyelia is a rare, slowly progressive neurological condition where there is a syrinx within the spinal cord. The safest mode of delivery and anesthetic management in patients with syringomyelia is still controversial. There are concerns regarding worsening of syrinx in vaginal delivery but there is no evidence to prove the same. In a case study done by Ghaly, there was difficulty in intubating the patient for general anesthesia prior to cesarean section as syringomyelia had involved the cervical cord, which was not the case with our patient.
In a case report by Margarido, the patient who had post traumatic syringomyelia was given epidural anesthesia instead of general anesthesia and her outcome was good.
In a case report by Acosta Diez the patient who only had low sensory perception had an instrumental vaginal delivery and even though she had received epidural analgesia she did not show any worsening of neurological symptoms whereas in our case as patient was for elective lower segment cesarean section labor analgesia was not indicated.
In a case report by Park and Jason D patient had associated Arnold Chiari malformation with syringomyelia and she had worsening of neurological symptoms in labor. She underwent cesarean section with epidural anesthesia and postpartum there was no worsening of neurological symptoms. In contrast our patient who had post traumatic syringomyelia with no associated malformation general anesthesia was given as her syrinx was involving the lumbar area. In another case report by Jason D patient had associated Arnold Chiari malformation and patient underwent operative vaginal delivery with epidural anesthesia.
Hasaballa, reported a patient of syringomyelia with paresthesia’s and weakness who was managed conservatively, her neurological symptoms regressed and she conceived and delivered uneventfully vaginally thereafter.
By and large syringomyelia doesn’t start de novo and is usually secondary to traumatic insult or infection. The route of delivery is decided considering the degree of motor deficit in the lower limbs. As such pregnancy does not alter the course of syringomyelia. There is much concern regarding route of anesthesia as spinal anesthesia is best avoided in order to prevent further trauma to the spinal cord which may alter the course of the disease. It is universally accepted that epidural anesthesia or general anesthesia is best suited for these patients as can be seen in above mentioned case reports. Epidural analgesia can be safely used in most of the patients with syringomyelia for labor analgesia.[7,8,9]
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- Roelofse JA, Shipton EA, Nell AC. Anaesthesia for caesarean section in a patient with syringomyelia. A case report. S Afr Med J. 1984;65(18):736–7.
Kumari H, Pardeshi S, Gupta AS. Syringomyelia In Pregnancy. JPGO 2019. Vol 6 No. 6. Available from: https://www.jpgo.org/2019/06/syringomyelia-in-pregnancy.html