Hereditary spastic paraplegia (HSP), also known as familial spastic paraparesis or Strümpell-Lorrain disease, is a rare group of inherited disorders characterized by progressive spastic weakness in the lower limbs due to axonal degeneration of the corticospinal tracts.1 Its prevalence is estimated to range from 1.3 to 9.6 per 100,000.2,3 We report on a patient with HSP who successfully underwent an Ivor-Lewis esophagectomy. This is the first report in the literature describing the anesthetic management of complex thoracoabdominal surgery in a patient with HSP.
The patient provided written informed consent for this publication.
A 52-year-old, 85-kg man presented for an elective Ivor-Lewis esophagectomy for a T1b adenocarcinoma of the gastroesophageal junction identified within an endoscopically resected nodule discovered during surveillance for esophageal metaplasia. Preoperative staging showed no evidence of metastatic disease. The patient’s medical history included HSP, hypertension, obesity (body mass index 30.5 kg/m2), sleep apnea with both central and obstructive components requiring nightly continuous positive airway pressure treatment, and previous ankle fracture surgery performed under general anesthesia, details of which were unavailable.
He was diagnosed with HSP at 24 years of age and uses a wheelchair due to spasticity in his legs. He has symptoms of bladder urgency but no other neurological symptoms and no cognitive impairment. He had very good functional capacity because he was able to wheel several kilometers at a time in his wheelchair without significant dyspnea or angina.
The patient was assessed in the anesthetic clinic before surgery. The patient was offered either an open or minimally invasive surgical approach and elected for an open procedure. Anesthetic management was planned with the aim of optimizing analgesia, minimizing respiratory complications, and reducing the risks associated with HSP. Epidural and intrathecal anesthesia was considered, but after discussion of risks and benefits, the decision was made to use general anesthesia combined with regional wound infusions of local anesthetic.
After premedication with intravenous (IV) midazolam 2.5 mg, an IV anesthetic induction was performed with fentanyl 50 µg, propofol 200 mg, and cisatracurium 10 mg (0.15 mg/kg). The patient was intubated with a left 41F double-lumen endobronchial tube to allow for isolated left lung ventilation. The depth of anesthesia was measured with entropy monitoring and maintained with sevoflurane (end-tidal concentration 1.7%–1.8%) in air and oxygen (inspired oxygen fraction 0.5) and a fentanyl infusion at 80 µg/h. Neuromuscular blockade was maintained with a cisatracurium infusion at 2 mg/h and measured using train-of-four monitoring placed on the right facial nerve. IV cephazolin 2 g and metronidazole 500 mg were administered, as well as subcutaneous heparin 5000 units.
An open 2-stage esophagectomy was performed with the patient beginning in the supine position. After an upper midline incision, the stomach was mobilized, the gastroepiploic arcade was preserved, the left gastric vessels were ligated, and a standard lymphadenectomy was performed. A pyloroplasty was performed and a feeding jejunostomy fashioned. Bilateral wound infusion catheters were inserted in the retro-rectus plane before wound closure and connected to ON-Q elastomeric pumps (Halyard Health, Inc, Alpharetta, GA).
The patient was then placed in the left lateral decubitus position. After initiation of 1-lung ventilation, a right lateral thoracotomy in the fifth intercostal space was performed. The esophagus was mobilized, lymphadenectomy was performed, and the stomach delivered into the thorax. The esophagus was transected above the azygous vein, and the stomach was divided using a linear stapler to create a gastric conduit. The proximal esophagus was anastomosed to the conduit using a circular stapler. A nasogastric tube was advanced through the anastomosis with the tip sitting in the lower aspect of the conduit. Apical and basal chest drains were inserted and a right paravertebral catheter was placed under direct vision before chest closure.
There were no respiratory or hemodynamic incidents during the operation. The double-lumen endobronchial tube was changed to a single-lumen endotracheal tube at the end of the case. The patient was transferred to the intensive care unit and extubated on postoperative day 1 (POD 1).
The patient received IV acetaminophen, continuous infusions of ropivacaine 0.2% at 5 mL/h via the ON-Q wound infusion catheters until POD 3 and 6 mL/h via the thoracic paravertebral catheter until POD 3, and fentanyl patient-controlled analgesia until POD 7. Cumulative fentanyl consumption in the first and second 24-hour periods was 360 and 520 µg, respectively, sufficient to maintain pain scores <6 on movement and <2 at rest on a numeric rating scale from 0 (no pain) to 10 (disabling pain). Oral prolonged-release oxycodone/naloxone 15/7.5 mg twice daily was commenced on POD 7.
Respiratory rehabilitation consisted of active nursing, chest physiotherapy, and incentive spirometry. Enteral feeding via the jejunostomy was commenced on POD 1 and oral intake was introduced on POD 5. A bedside cycle ergometer—a stationary cycling apparatus that allows patients to exercise through passive or active-assisted training—was used in lieu of mobilization given the patient’s paraplegia. Both mechanical and pharmacological prophylaxis against venous thromboembolism was continued throughout the postoperative period, and meticulous attention was paid to pressure injury prevention. The patient experienced no postoperative complications and was discharged home on POD 11.
Histological analysis showed high-grade glandular dysplasia surrounding the previous resection site with no residual invasive malignancy and 30 negative lymph nodes.
A variety of anesthetic strategies have been described for patients with HSP. Several case reports described regional anesthesia for patients undergoing obstetric procedures.4–6 McIver et al7 and Dallman8 described the use of general anesthesia for cesarean delivery and laparotomy, respectively, using rocuronium as the muscle relaxant, sevoflurane as the maintenance agent, and neostigmine and glycopyrrolate to reverse the neuromuscular blockade. Ponsonnard et al9 described the use of total IV anesthesia with propofol and remifentanil in a patient undergoing spinal surgery, with rocuronium as the muscle relaxant and sugammadex as the reversal agent. Franco-Hernández et al10 described the use of rocuronium, sevoflurane, and sugammadex in a patient undergoing cholecystectomy and the use of propofol and remifentanil, rocuronium, and sugammadex in a second patient undergoing laparoscopic subtotal colectomy.
Depolarizing muscle relaxants such as succinylcholine are contraindicated in patients with motor neuron diseases due to the risk of life-threatening hyperkalemia.11 We chose the nondepolarizing muscle relaxant cisatracurium over rocuronium because of its more favorable recovery characteristics after a prolonged infusion.12 This patient was very sensitive to the effects of cisatracurium as evidenced by the intubating dose lasting 2 hours instead of the usual 45 minutes before showing any sign of reversibility, and by the low dose required for the ongoing infusion. Had we elected to extubate the patient at the end of the procedure, we would have needed to take this into consideration.
Ivor-Lewis esophagectomy is a complex and high-risk procedure with a mortality of up to 5%13,14 and overall morbidity of over 50% including a 32% risk of pulmonary complications.15 We kept the patient intubated overnight, which allowed for extended recovery of the deflated lung, reduction of risk of residual neuromuscular blockade after a long anesthetic, and avoidance of noninvasive ventilation or unplanned reintubation in the event of early respiratory deterioration. Given the patient’s severe obstructive sleep apnea, continuous positive airway pressure would have been ideal for decreasing the risk of hypoventilation and improving alveolar recruitment; however, we generally prefer to avoid noninvasive ventilation in the early postoperative period after esophagectomy because of the theoretical risk of conduit distension and anastomotic complications.
Postoperatively, we provided multimodal pain management including the use of regional analgesic techniques to allow the patient to fully participate in early chest physiotherapy and thus reduce the risk of pulmonary complications. In previous esophagectomies, we have found the combination of paravertebral catheter, rectus sheath catheters, and systemic analgesia provides excellent pain relief without the need for an epidural catheter. We find that placement of the thoracic paravertebral catheter under direct vision at the time of surgery is time-efficient, safe, and provides very effective for analgesia for the thoracotomy wound. The anesthesiologist provides oversight to ensure correct paravertebral positioning. In other cases, we have used the catheters for up to 5 days without complications. Adequate pain management also allows for early mobilization, an entrenched component of enhanced recovery after surgery protocols for esophagectomy even though the supporting evidence is not strong.16 In our patient, his neurological condition was a major barrier to mobilization, but this was partially overcome by using the bedside ergometer. The combination of a comprehensive analgesic plan, early chest physiotherapy, and early mobilization contributed to an excellent outcome.
We have shown that a patient with HSP can safely undergo a high-risk thoracoabdominal procedure through judicious planning and effective multidisciplinary teamwork. Though HSP provides unique perioperative challenges, it should not preclude operative intervention in the management of esophageal cancer.
Name: Phillip F. Yang, MBBS, MS.
Contribution: This author helped obtain the clinical details and draft the manuscript.
Name: David J. R. Links, MBBS, MS, FRACS.
Contribution: This author helped revise the manuscript.
Name: Johnny Petrovski, MBBS, FANZCA.
Contribution: This author helped revise the manuscript.
This manuscript was handled by: Raymond C. Roy, MD.
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