Anesthetic Management of a Parturient with Amyotrophic Lateral Sclerosis Undergoing Cesarean Section

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Anesthetic Management of a Parturient with Amyotrophic Lateral Sclerosis Undergoing Cesarean Section

Amyotrophic lateral sclerosis (ALS) is a well-known and devastating neuromuscular disease. It is characterized by the degeneration of both upper and lower motor neurons, leading to muscle weakness, atrophy, and, in most cases, respiratory failure within 3 to 5 years. For parturients with ALS undergoing cesarean section, anesthetic management presents unique challenges.

Case Presentation

A 31-year-old woman at 37 weeks of pregnancy (weighing 65 kg and 165 cm tall) with progressive ALS was scheduled for cesarean section. She was diagnosed 13 months prior. Her symptoms included dysarthria and difficulty lifting her head from the bed. The motor power of her right and left proximal upper extremities was grade 3 and grade 4, respectively. She also had mildly impaired swallowing and respiratory dysfunction, requiring a non-invasive ventilator (using bi-level positive pressure – BiPAP synchrony) at midnight. A pre-operative examination revealed an amyotrophic lateral sclerosis functional rating scale revised (ALSFRS-R) score of 28. Her family history was notable for a hereditary feature. Her mother’s brother was diagnosed with lower motor neuron syndrome, and her grandfather’s brother had neck weakness. Both died 3 and 1 year after the onset of symptoms, respectively. She had previously delivered a boy at the age of 27 but experienced postpartum urinary retention for 40 days after vaginal delivery due to meperidine. Further pulmonary function tests showed a restrictive pattern with low vital and total lung capacity (forced vital capacity was 52% of normal). Arterial blood gas analysis with the non-invasive ventilator showed a PaCO2 of 34.1 mmHg and an oxygen saturation of 98.7%.

Anesthetic Plan

After pre-operative multidisciplinary consultation, it was decided to implement a bilateral ultrasound-guided transversus abdominis plane (TAP) block combined with incision infiltration before delivery. After delivery, total intravenous anesthesia (TIVA) with remifentanil and propofol by target-controlled infusion would be given to achieve proper analgesia.

Intraoperative Management

Upon entering the operating room, standard monitors were applied, including a 5-lead electrocardiogram with ST-segment analysis, non-invasive blood pressure, and pulse oximetry. Intraoperatively, she was provided with oxygen using her BiPAP ventilator. In the supine position and under ultrasound guidance, a 17 G Touhy needle was positioned via an in-plane approach at the level of the mid-axillary line between the sub-costal margin and iliac crest. Then, 20 ml of 0.5% ropivacaine was injected through the needle on the left side (with adequate spread between the internal oblique and transversus abdominis), and another 20 ml on the right side (as shown in Figure 1). Partial onset of sensory blockade was noted within 10 minutes. At the time of surgical incision, 0.5% lidocaine was injected around the incision. A live girl weighing 2650 g was born with an Apgar score of 10-10-10 (1-5-10 minutes). Anesthesia was maintained with 2 mg/mL (plasma target concentration, Marsh model) of propofol and 3 ng/mL (plasma target concentration, Minto model) of remifentanil, which relieved intraoperative discomfort. During the operation, the minimal oxygen saturation (SpO2) was 98%, and a subsequent arterial blood gas measurement showed a PaCO2 of 31 mmHg and a PaO2 of 106 mmHg. The intensity of postoperative pain was less than 4 on the numerical rating scale (NRS) at 2 and 24 hours without opioid treatment. She was discharged on postoperative day (POD) 3 without complications.

Discussion on ALS and Anesthetic Choices

ALS is a degenerative disease of the motor ganglia in the anterior horn of the spinal cord and spinal pyramidal tracts. It is the most common form of motor neuron disease, with an incidence of 1.5 to 2.5 per 100,000 per year [2]. Autosomal-dominant familial cases account for nearly 10% of ALS cases. Recently, mutations in the fused in sarcoma (FUS)/translocated in liposarcoma gene have been identified as a cause of familial ALS [3]. In this patient, polymerase chain reaction analyses of total RNA revealed a minor mutation on the FUS (NM_004960) gene.

Cesarean section is often a better option than labor for parturients with ALS due to the progressive weakness of respiratory muscles. These parturients may struggle to meet respiratory demands during and after birth.

When choosing an anesthetic method for an ALS patient, anesthesiologists need to consider the method that will be least harmful in terms of disease progression. There is a concern that local anesthetic administration near the nerve or needle trauma could exacerbate pre-existing disease symptoms [4]. Hence, neuraxial block was avoided as a conservative and safe approach.

General anesthesia (GA) has its risks. Patients with bulbar involvement can face postoperative aspiration and respiratory inadequacy. According to the literature [1], the mortality rate may increase when the ALSFRS-R score is less than 39 points. Therefore, peripheral nerve blockade (to minimize the potential effects of local anesthetics on the spinal cord) and TIVA without muscle relaxants (for a smooth recovery after GA) were chosen. Few other studies have recommended the use of ropivacaine in patients with ALS for peripheral nerve blockade. Belavy et al [5] used 0.5% ropivacaine (40 mL) for bilateral TAP blocks after cesarean section. TAP block can work on the skin, subcutaneous, and muscle levels but has no effect on visceral pain. Intravenous anesthetics can block visceral pain. Under sub-anesthetic concentrations, propofol, sevoflurane, remifentanil, and ketamine can decrease visceral pain-evoked potentials amplitudes. So, the rapid reversible short-acting analgesic (remifentanil) and amnestic (propofol) agents were selected to relieve visceral pain. No muscle relaxant was used in this parturient. Depolarizing neuromuscular blockers like succinylcholine should be strongly avoided as they can cause a lethal elevation of serum potassium in neuromuscular disorders like ALS. Non-depolarizing neuromuscular blockers should also be used sparingly and at the lowest possible doses with neuromuscular function monitoring.

In conclusion, this case highlights that the use of peripheral nerve blockade (when possible) can minimize the potential effects of local anesthetics on the spinal cord. The overall strategy, which includes the use of rapidly reversible short-acting analgesic and amnestic agents with no neuromuscular relaxants, can lead to a successful outcome in the anesthetic management of parturients with ALS undergoing cesarean section.

References:

  1. Kollewe K, Mauss U, Krampfl K, Petri S, Dengler R, Mohammadi B. ALSFRS-R score and its ratio: a useful predictor for ALS-progression. J Neurol Sci 2008;275:69–73. doi: 10.1016/j.jns.2008.07.016.
  2. Soriani MH, Desnuelle C. Epidemiology of amyotrophic lateral sclerosis. Rev Neurol 2009;165:627–640. doi: 10.1016/j.neurol.2009.04.004.
  3. Suzuki N, Kato S, Kato M, Warita H, Mizuno H, Kato M, et al. FUS/TLS-immunoreactive neuronal and glial cell inclusions increase with disease duration in familial amyotrophic lateral sclerosis with an R521C FUS/TLS mutation. J Neuropathol Exp Neurol 2012;71:779–788. doi: 10.1097/NEN.0b013e318264f164.
  4. Hobaika AB, Neto AP. Comment on “Anaesthetic management of a patient with amyotrophic lateral sclerosis for transurethral resection of bladder tumour”. Indian J Anaesth 2013;57:635–636. doi: 10.4103/0019-5049.123355.
  5. Belavy D, Cowlishaw PJ, Howes M, Phillips F. Ultrasound-guided transversus abdominis plane block for analgesia after caesarean delivery. Br J Anaesth 2009;103:726–730. doi: 10.1093/bja/aep235.

doi: 10.1097/CM9.0000000000000809

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