Journal of Obstetric Anaesthesia and Critical Care

CASE REPORT
Year
: 2022  |  Volume : 12  |  Issue : 1  |  Page : 62--63

Neuraxial anesthesia for a laboring patient with hereditary spastic paraplegia: A case report


Joshua Falescky, Madina Gerasimov, Judith Aronsohn, Gregory Palleschi 
 Department of Anesthesiology, Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, United States

Correspondence Address:
Dr. Madina Gerasimov
Northwell, Northshore University Hospital, 300 Community Dr, Manhasset, NY - 11030
United States

Abstract

Hereditary spastic paraplegia (HSP) is a rare, inherited condition affecting the corticospinal tract, typically characterized by bilateral lower extremity weakness and stiffness. HSP presents challenges to anesthetic management, particularly in the obstetric population due to the paucity of existing recommendations in the literature. Although case reports exist regarding the successful use of general and neuraxial anesthesia for cesarean section, none currently exist with regard to labor analgesia. Here, we report the use of labor epidural anesthesia in a 29-year-old woman with HSP. She delivered via cesarean delivery after laboring for 27 h with an epidural catheter in place. The patient tolerated epidural placement well with no adverse neurologic sequelae. The utilization of neuraxial techniques avoids the risks associated with airway manipulation in a parturient and obviates the need for the use of muscle relaxants in the setting of upper motor neuron disease. Epidural anesthesia appears to be a safe approach to the management of labor and cesarean delivery in a parturient with HSP.



How to cite this article:
Falescky J, Gerasimov M, Aronsohn J, Palleschi G. Neuraxial anesthesia for a laboring patient with hereditary spastic paraplegia: A case report.J Obstet Anaesth Crit Care 2022;12:62-63


How to cite this URL:
Falescky J, Gerasimov M, Aronsohn J, Palleschi G. Neuraxial anesthesia for a laboring patient with hereditary spastic paraplegia: A case report. J Obstet Anaesth Crit Care [serial online] 2022 [cited 2022 Jun 29 ];12:62-63
Available from: https://www.joacc.com/text.asp?2022/12/1/62/339550


Full Text



 Introduction



Hereditary spastic paraplegia (HSP) is a group of upper motor neuron diseases, with variable patterns of inheritance.[1] HSP commonly manifests as a gait disturbance due to lower limb spasticity with progression to paraparesis or paraplegia. HSP presents unique anesthetic challenges, especially in the obstetric population. The current literature consists of several case reports describing the successful use of general and neuraxial anesthesia for cesarean delivery of parturients with HSP.[2],[3],[4] However, there are no reports describing the use of epidural analgesia for labor in these patients. Here, we describe the successful use of an epidural technique for both labor analgesia and subsequent cesarean section.

 Case Description



A 29-year-old woman G1P0 was admitted for induction of labor at 39 weeks gestation. Her medical history was significant for HSP, mild asthma, and hearing loss. Height and weight were 156 cm and 81 kg, respectively. The diagnosis of HSP was verified by genetic testing and found to involve two pathogenic variants of the SPG11 genotype (c.6753G>A, p.Trp2251, and c.5815G>T, p.Glu1939), which follows autosomal recessive inheritance. Baseline neurologic deficits included lower extremity weakness with spastic gait, ataxia, and joint pain, requiring the use of a wheelchair. A multidisciplinary meeting took place involving the obstetric, anesthesiology, and neurology services. We decided to proceed with the induction of labor with the placement of an epidural catheter for labor analgesia.

Fifteen hours following the induction, the cervix was dilated at 1 cm. The patient was placed in the sitting position for epidural catheter insertion and 3 mL of 1% lidocaine were infiltrated into the subcutaneous tissue. A 17ga Tuohy needle was inserted at the L3-4 interspace. Using the loss of resistance to air technique, the epidural space was located on the first attempt, and an epidural catheter was threaded, without complication, to 13 cm. A test dose of 3 mL of 1.5% lidocaine with epinephrine (1:200,000) was administered with no evidence of intravascular or intrathecal placement. An 8 mL bolus of 0.0625% bupivacaine and 2 μg/mL fentanyl solution were used to reach a sensory loss at the T10 level. Adequate analgesia was maintained with patient-controlled epidural analgesia and continuous infusion of the same solution at 10 mL/h.

Three hours later, the patient was started on an oxytocin infusion. At 27 h after induction, the patient was dilated at 9 cm. As she had not progressed within the past 4 h, she was diagnosed with the arrest of the active phase of labor. In addition, the patient was noted to have a fever of 38.6°C and a category II tracing, characterized by prolonged decelerations and fetal tachycardia. Her clinical picture was concerning for chorioamnionitis, which was later confirmed on the placental pathology report.

Following another multidisciplinary meeting, we decided to proceed with cesarean delivery. The epidural was dosed with 15 mL of 2% lidocaine with epinephrine (1:200,000). A healthy baby weighing 3200 g was delivered with APGARs 9 and 9 at 1 and 5 min, respectively. The immediate postoperative course was complicated by uterine atony requiring oxytocin, methylergonovine, and tranexamic acid administration, and transfusion of 2 units of allogeneic blood for estimated blood loss of 1500 mL. Despite the initial complications, she remained hemodynamically stable, and vasopressor infusions were never required. After the closure of the surgical site, 2 mg of morphine was administered via the epidural for postoperative pain control, and the catheter was removed. The remainder of her postoperative course was uneventful, and her neurologic examination was unchanged. The patient was discharged home on postoperative day 2.

 Discussion



Hereditary spastic paraplegia presents significant challenges, given the rarity of the disease and the paucity of existing recommendations regarding obstetric anesthesia management. Prior case reports demonstrate the use of general anesthesia in a patient with HSP undergoing cesarean delivery.[2] However, general anesthesia requires securing the airway, which may prove challenging in the obstetric population. Furthermore, rapid sequence intubation requires neuromuscular blockade. Succinylcholine is contraindicated in HSP secondary to the upregulation of nicotinic acetylcholine receptors and the potential for fatal hyperkalemia.[1] Non-depolarizing agents, such as rocuronium, may be used, especially with the increasing availability of sugammadex.

Neuraxial anesthesia obviates the need for muscle relaxants and airway concerns associated with general anesthesia. Several case reports have demonstrated the utilization of spinal and epidural anesthesia in HSP patients.[3],[4] A report by McTiernan describes the placement of an epidural catheter in a 30-year-old, wheelchair-bound HSP patient undergoing elective cesarean delivery. The epidural was placed at the L3–L4 interspace and dosed with 20 mL of 2% lidocaine with epinephrine. There were no anesthetic-related complications, and no neurologic changes postoperatively and at 6-month follow-up. Another case report by Thomas et al.[4] described the administration of a spinal anesthetic to an 18-year-old HSP patient for the manual removal of the placenta after vaginal delivery. She received 2.0 mL of 0.5% isobaric bupivacaine with 25 μg fentanyl via a single-shot spinal using a 25 Ga Whitacre needle. No anesthetic complications were appreciated, and neurologic examination returned to baseline within 24 h.

What makes this case unique is that our HSP patient underwent a trial of labor to avoid the morbidity associated with cesarean delivery. In light of prior reports demonstrating the successful use of neuraxial anesthesia in HSP and knowing the current literature is lacking for evidence that local anesthetics affect disease progression in HSP, we proceeded with the insertion of an epidural catheter for labor analgesia. Although adequate analgesia was achieved, her intrapartum course was unfortunately complicated by the arrest of an active phase of labor and chorioamnionitis requiring conversion to cesarean section. The exact etiology behind the arrest of labor is unknown. Possibilities include cephalopelvic disproportion and lack of pelvic floor muscle relaxation in the setting of neurologic spastic disease. The use of epidural anesthesia for conversion to cesarean section was also successful, and unchanged baseline neurologic status was documented postoperatively.

Epidural anesthesia appears to be a safe option for both labor analgesia and cesarean delivery in HSP patients. The utilization of neuraxial techniques avoids the risks associated with airway manipulation in a parturient as well as the need for muscle relaxants in the setting of an upper motor neuron disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2McIver T, Jolley D, Pescod D. General anaesthesia and caesarean section for a patient with hereditary spastic paraparesis (Strumpell's disease). Int J Obstet Anesth 2007;16:190-1.
3McTiernan C, Haagenvik B. Strümpell's disease in a patient presenting for cesarean section. Can J Anaesth 1999;46:679-82.
4Thomas I, Thomas M, Scrutton M. Spinal anaesthesia in a patient with hereditary spastic paraplegia: Case report and literature review. Int J Obstet Anesth 2006;15:254-6.