Multimodal Anesthesia for Glaucoma Surgery in a Child with Mitochondrial Disease and Malignant Hyperthermia-Juniper Publishers
Juniper Publishers-Journal of Anesthesia
Abstract
Mitochondrial diseases (MD) are characterized by
impairments of mitochondrial function that precipitate metabolic
acidosis. An 8-year-old MD female with prior anesthesia exposure
complicated by metabolic acidosis, seizures and propofol infusion
syndrome, presented for glaucoma surgery. We present a multimodal
balanced anesthetic technique used to successfully manage this complex
case.
Introduction
Mitochondrial disorders (MD) are a heterogeneous
group of genetic disorders that impair mitochondrial integrity and
result in deficient energy production. The disorder has an incidence of
1:5000 live births [1], and affects tissues with high-energy
requirements such as the central nervous system, retina, heart and
muscle [2]. Consequently, these patients have multiple co-morbidities
that include cardiac, endocrine, and neurologic dysfunction [3-5].
Current evidence suggests that mitochondrial disease may be a
contributing factor in the pathogenesis of glaucoma [6]. Anesthesia in
this patient population may prove hazardous because the stress of
surgery and fasting can induce marked metabolic aberrations, most
commonly lactic acidosis [7].
Case Presentation
Institutional Review Board approval is not required
for single case reports at Jackson Memorial Hospital and University of
Miami. An 8 year old, 25kg Caucasian female with infantile glaucoma,
mitochondrial disease, renal tubular acidosis type II and family history
of malignant hyperthermia presented for bilateral glaucoma surgery. Of
note, at age 6, eye surgery under GA had been complicated by severe
post-operative metabolic acidosis, propofol infusion syndrome (PRIS) and
prolonged two week PICU stay. At that time, genetic investigation
revealed an abnormality of the 2-oxoglutarate-dehydrogenase enzyme.
A multidisciplinary group comprising physicians from
genetics, neurology, cardiology, nephrology and anesthesiology was
convened and recommended new studies in order to identify the gene
deficit. Also, the group recommended preoperative nutritional
supplementation and electrolyte correction. Her ECG demonstrated
prolongation of the QT interval (426ms) and the 2-D cardiac echo was
normal. Current medications included trileptal, brimonidine and
lantaprost ophthalmic solutions, coenzyme Q10 and a multivitamin
supplement.
Our anesthesia plan centered on a balanced technique
utilizing intravenous agents for sedation and hypnosis in tandem with
extraconal ophthalmic block for analgesia. A vapor-free operating room
was prepared. Monitoring included standard ASA monitors and bispectral
analysis (BIS) (Model 185-0151) with the target range set at 40-60. In
the holding suite the child was sedated with midazolam. Then, in the OR,
infusions of remifentanil (0.1mcg/kg/min) and dexmedetomidine
(1mcg/kg/hr) were started. When BIS levels fell below 60 we administered
cisatracurium (4mg) and performed endotracheal intubation. A left
radial intra-arterial catheter was placed for blood gas and metabolic
panel analysis. Prior to surgery, a periorbital block using 2% lidocaine
was performed.
Serial metabolic analysis demonstrated stable acid/base status
with marginal elevations in serum lactic acid (1.3 to 1.5mg/dL).
Surgery was completed in 110 minutes and passed uneventfully.
At this point the neuromuscular block was antagonized, infusions
were discontinued and the child was extubated and transferred
to PICU for overnight observation. She was discharged from the
hospital on postoperative day 1.
Discussion
Mitochondrial disease (MD) are a divergent group of more
than 100 genetic aberrations in which defects of the organelle
impair oxidative phosphorylation and the production of energy,
resulting in cell injury and metabolic acidosis. It is estimated
that MD afflicts 1:5000 children. Symptoms typically manifest in
tissues that are wholly dependent on mitochondria as a source of
energy [8].
The anesthetic management of children with MD is complex
because studies indicate that volatile inhalational agents depress
mitochondrial function [9,10]. Additionally, severe co morbidities
such as organ failure are potential risks for lactic acidosis. Further
anesthesia considerations include sensitivity to neuromuscular
blocking agents, altered homeostasis and impairment of
mitochondrial integrity triggered by several intravenous
agents. There are no controlled clinical trials to assess the
effects of anesthetic agents and their correlation with intra- and
postoperative complications [3]. Currently, available data stems
from case reports, expert opinion and retrospective chart reviews
[10, 11].
A recent rat model study demonstrated that ketamine
produces alterations in the mitochondrial respiratory chain
complex 1 [12]. Other investigations implicate etomidate and
barbiturates as inhibitors of the same complex [13]. Moreover,
evidence suggests that propofol impairs mitochondrial function,
and that propofol infusions are associated with PRIS because
this agent is highly lipophilic and readily diffuses across cell
membranes. Therefore, propofol should only be administered
as a single bolus [14,15]. On the other hand, dexmedetomidine,
a selective α2 agonist, is known to have beneficial effects on the
mitochondrial membrane [16]. Although the association between
MD and malignant hyperthermia (MH) remains unproven, the
Malignant Hyperthermia Association of the United States (MHAUS)
continues to recommend avoidance of volatile anesthetic agents
and caution in using succinylcholine [17- 19].
In this patient we were concerned about the
development of
lactic acidosis, malignant hyperthermia and PRIS. At the same time,
we aimed to optimize intraoperative surgical conditions and avoid
patient movement. In light of these considerations; we adopted
a balanced intravenous anesthetic technique that eliminated the
need for a volatile inhalational agent. Thus, we integrated the
hypnotic effect of dexmedetomidine and the analgesic property of an
ultra-short acting opioid (remifentanil) with a non-depolarizing
neuromuscular blocking agent having negligible renal elimination
(cisatracurium) and also performed an extraconal eye block (2%
lidocaine). We selected lidocaine because bupivacaine inhibits
carnitine-acylcarnitine translocase and has been reported to
precipitate ventricular dysrhythmias [20]. The eye block was
quintessential because it reduced anesthetic requirements and
assured a smooth transition between the operating room (OR)
and post anesthesia care unit (PACU).
In summary, the anesthesia care for MD children is challenging.
This case report highlights two important points; [1] the value
of a multidisciplinary team to optimize preoperative status, and
[2] the benefit of judicious selection of anesthetic technique in
order to avoid complications, such as lactic acidosis, MH or PRIS.
Finally, this case underscores the benefit of regional anesthesia in
reducing anesthetic requirements and facilitating an uneventful
transition from the operating room to post anesthesia care unit.
For more articles in Journal of Anesthesia
& Intensive Care Medicine please click on:
https://juniperpublishers.com/jaicm/index.php
https://juniperpublishers.com/jaicm/index.php
Comments
Post a Comment