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Journal of Medical Cases

Case Report

Volume 8, Number 10, October 2017, pages 297-300

Spinal Instead of General Anesthesia for Two Pediatric Patients at Risk for Malignant Hyperthermia

Malignant hyperthermia (MH) is an inherited disorder, characterized by a hypermetabolic state following exposure to succinylcholine or volatile anesthetic agents. This rare phenomenon occurs in approximately 0.66 per 100,000 pediatric patients [1]. The pathophysiology relates to the exaggerated intracellular release of calcium from the sarcoplasmic reticulum. Susceptible patients often have mutations in the ryanodine receptor (RYR1) or dihydropyridine (DHP) receptor, leading to unregulated calcium release following exposure to triggering agents [2]. The unregulated increase in intracellular calcium leads to accelerated muscle contraction resulting in hypercarbia, hyperthermia, metabolic acidosis, and rhabdomyolysis. If untreated, the process has a high mortality rate [3]. Treatment includes early recognition, removal of the inciting agent, management of the systemic complications, and the administration of dantrolene [4]. In susceptible patients or in patients with a family history of MH, general anesthesia can be provided with the use of non-triggering agents including nitrous oxide and intravenous agents (propofol, ketamine, dexmedetomidine, opioids, and midazolam) [4].

Alternatively, for specific surgical procedures, spinal anesthesia can be used in place of general anesthesia. Spinal anesthesia was first reported in infants and children in the late 19th and early 20th century when the potential for morbidity and mortality from general anesthesia was significant [5, 6]. Spinal anesthesia saw a resurgence during the 1980s when it was used in former preterm infants as a means of avoiding intraoperative intubation in patients with underlying bronchopulmonary dysplasia (BPD), as well as apnea and respiratory insufficiency following general anesthesia with halothane [7, 8]. However, enthusiasm waned as the risk of apnea was shown to be less likely when newer inhalational anesthetic agents were introduced into clinical practice [9, 10]. Moreover, the introduction of surfactant and the improved management of mechanical ventilation in preterm infants have changed the acuity of BPD, reducing the severity of the underlying respiratory disease [11]. More recently, there has been a renewed interest in the use of spinal anesthesia as an alternative to general anesthesia due to concerns raised by laboratory and animal studies suggesting that exposure to certain anesthetic agents is neurotoxic and may be associated with long-term neurocognitive effects [12, 13]. Although the evidence demonstrating an actual clinical effect on long-term human neurocognitive outcomes is equivocal, our center has initiated a program providing spinal anesthesia instead of general anesthesia for many surgical procedures in infants and children [14, 15]. Spinal anesthesia may also have a role as a means of avoiding general anesthesia in specific patient populations with co-morbid diseases associated with an increased risk of general anesthesia. We present the use of spinal anesthesia as a means of avoiding general anesthesia in two infants with a family history of MH.

At Nationwide Children’s Hospital, formal Institutional Review Board approval is not required for case presentations involving two or fewer patients.

An 8-month-old, 10-kg boy with a hidden penis, penoscrotal webbing, and phimosis, presented for elective buried penis repair, complex scrotoplasty, and circumcision. The boy was otherwise healthy and there was no prior history of general anesthesia. His family history was notable for MH in both the father and paternal grandmother. Due to the potential risks of MH in this patient, spinal anesthesia was discussed with the parents and selected for the operative procedure. On the day of surgery, the infant was held nil per os (NPO) for solids for 6 h and clear liquids for 2 h. A topical anesthetic cream was placed over the lumbar area and the patient was transported to the operating room. Spinal anesthesia was placed using a 1.5-inch, 22-gauge spinal needle, which was inserted at the L3-4 interspace. Following free flow of cerebrospinal fluid (CSF), 1.2 mL of isobaric 0.5% bupivacaine (0.6 mg/kg bupivacaine) with epinephrine 1:200,000 and 10 µg of clonidine was injected. Routine American Society of Anesthesiologists’ (ASA) monitors were placed. Adequate surgical anesthesia with sensory and motor blockade was attained. A peripheral intravenous catheter was placed in the foot after the onset of sensory blockade. No intraoperative medications were required to supplement the spinal anesthesia or provide sedation. No clinically significant respiratory or hemodynamic changes were noted. There were no clinical signs of MH. The surgical procedure lasted for approximately 1 h. The patient was transferred to the post anesthesia care unit (PACU) for recovery. Postoperatively, a single dose of fentanyl (1 µg/kg) was administered for irritability. His postoperative course was unremarkable and he was discharged home after 3 h of monitoring.

A 9-month-old, 10.5-kg boy with a right ectopic testicle and penile adhesions, presented for elective right orchidopexy and lysis of penile adhesions with circumcision. The infant was otherwise healthy with no prior anesthesia or surgical history. His family history was notable for MH in a maternal nephew. Due to the potential risks of MH in this patient, spinal anesthesia was discussed with the parents and selected for the operative procedure. On the day of surgery, the infant was held NPO for solids for 6 h and clear liquids for 2 h. A topical anesthetic cream was placed over the lumbar area and the patient was transported to the operating room. Spinal anesthesia was provided using a 1.5-inch, 25-gauge spinal needle, which was inserted at the L3-4 interspace. Following free flow of CSF, 1.2 mL of isobaric 0.5% bupivacaine (0.6 mg/kg bupivacaine) with epinephrine 1:200,000 and 10 µg of clonidine was injected. Routine ASA monitors were placed. Adequate anesthesia with sensory and motor blockade was attained. A peripheral intravenous catheter was inserted in the foot after the onset of sensory blockade. No intraoperative medications were required to supplement the spinal anesthesia or provide sedation. No clinically significant respiratory or hemodynamic changes were noted. There were no clinical signs of MH. The surgical procedure lasted approximately 1.25 h. The patient was transferred to the PACU and discharged home after 1 h of monitoring following an unremarkable postoperative course.

Although used most commonly to avoid the deleterious effects of general anesthesia on the central control of ventilation, to avoid the need for endotracheal intubation, or to avoid the potential neurocognitive effects of general anesthetic agents, there are many co-morbid conditions which may increase the risk of general anesthesia [16]. In the two patients described, spinal anesthesia was chosen instead of general anesthesia due to the potential for increased morbidity given the patients’ family history of MH. Spinal anesthesia effectively provided surgical anesthesia for the urologic procedures, thereby avoiding the need for general anesthesia. Neither patient required intraoperative sedative or analgesic medications and maintained hemodynamic stability without signs of MH. The postoperative PACU stay of the first patient was prolonged because a dose of fentanyl (1 µg/kg) was administered, mandating longer monitoring per our institutional guidelines. Our second patient highlights another benefit of spinal anesthesia as he required no postoperative analgesic or sedation medications, and therefore was discharged rapidly from the PACU to the phase two recovery area.

Previous reports of the use of spinal anesthesia in MH susceptible adult patients are outlined in Table 1 [17-23]. Adverse effects were rare with hypotension noted in one parturient and respiratory difficulties in another patient related to a high spinal block (T3) and the concomitant administration of dantrolene. Our two patients represent the first reports of the use of spinal anesthesia in potentially MH-susceptible pediatric patients. Overall, these reports and our experiences demonstrate the safe and effective use of spinal anesthesia as an alternative to general anesthesia in patients at risk for MH due to their previous anesthetic history or family history.

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Table 1. Use of Spinal Anesthesia in MH Susceptible Patients [17-23]

Although generally considered safe in patients with MH, anecdotal reports suggest the onset of MH following neuraxial (spinal or epidural) anesthesia [24-26]. These anecdotal reports include MH-like symptoms after neuraxial administration of both ester (tetracaine) and amide (bupivacaine) local anesthetic agents. Sheu et al describe the potential development of MH following spinal anesthesia with tetracaine in a 62-year-old patient undergoing transurethral resection of the prostate [27]. Caffeine-halothane contracture testing was not available and while MH was unable to be confirmed, the author reported a hypermetabolic state demonstrating features of acute rhabdomyolysis. Albeit rare, the potential to trigger MH with neuraxial anesthesia mandates appropriate intraoperative and postoperative monitoring of temperature and physiologic function. Dantrolene should be available for specific treatment of MH.

Adverse effects related to spinal anesthesia are generally low, especially in the pediatric population. Relative contraindications include uncorrected coagulopathy, localized infection at the insertion site, and evidence of intracranial hypertension. Given the later development of the sympathetic nervous system, hypotension is rare and in fact, blood pressure stability is better with spinal anesthesia than general anesthesia [28]. Post-dural puncture headache (PDPH) remains uncommon in pediatric patients less than 10 - 12 years of age when compared to the adult population [29]. Other neurological complications that may occur from spinal anesthesia are rare, including subdural hematoma formation. Anecdotal reports suggest that such complications generally occur in the setting of co-morbid defects of coagulation function [30].

Developed over the past 24 months, the following outlines our standard practice for spinal anesthesia from our institution. The intrathecal space is accessed using a 1.5-inch, 22-gauge or a 1.5-inch, 25-gauge spinal needle (Becton Dickinson, Franklin Lakes, NJ, USA). The local anesthetic used is preservative-free isobaric bupivacaine (0.5%) in a dose of 0.2 mL/kg (1 mg/kg). The maximum dose is generally 1 mL (5 mg) in most infants. However, for longer procedures or larger children, a higher total volume can be used (1.2 mL or 6 mg). To prolong the duration of the surgical block and maximize postoperative analgesia, the syringe is washed with 1:1,000 epinephrine prior to filling it with the local anesthetic agent and clonidine (1 µg/kg) is added to the solution [31, 32].

With its excellent safety profile, spinal anesthesia is a useful alternative to general anesthesia in patients with a past history or family history of MH. Our pediatric cases highlight the benefits of spinal anesthesia instead of general anesthesia in patients with co-morbid conditions. With concerns for respiratory complications following general anesthesia with the volatile agents and ongoing controversy regarding the potential long-term neurocognitive outcomes of general anesthesia, spinal anesthesia may have a larger role in the anesthetic management of pediatric patients.

Conflict of Interest

No conflict of interest for any authors.

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