Paediatric Anaesthesia 2001 11: 498±500

Case report
Anaesthetic management of a child with a positive
family history of malignant hyperthermia
for posterior fossa surgery in the sitting position
M.A. WOOTTON FRCA A N D J . LO C K I E BSc, MBChB, FRCA
Department of Anaesthesia, Great Ormond Street Hospital for Children NHS Trust, London, UK

Summary
A 6-year-old boy with a positive family history of malignant
hyperthermia presented for posterior fossa craniectomy and excision
of medulloblastoma. A nontriggering anaesthetic was therefore
planned using infusions of propofol and remifentanil and a vapour
free anaesthetic system delivering an oxygen/air mixture. The surgery
was carried out with the child in the sitting position.

Keywords: malignant hyperthermia; posterior fossa surgery;

remifentanil

Posterior fossa surgery at Great Ormond Street

Introduction
Malignant hyperthermia (MH) is an autosomal
dominant, genetically inherited disorder which
may be triggered by anaesthesia. The commonest
anaesthetic triggering agents are suxamethonium
and the volatile agents. An episode of MH results in
a hypermetabolic state involving increased oxygen
consumption, increased carbon dioxide production
and an increase in temperature of more than 2°C
per h. The basic cellular defect appears to be an
abnormal release of intracellular calcium from the
sarcoplasmic reticulum. If left untreated, the syn-
drome is commonly fatal, though early treatment
with dantrolene and intensive supportive measures
will reduce mortality (1). In cases where there is a
family history of MH and the patients status is
unknown, it is therefore usual to use a nontriggering
anaesthetic.
Correspondence to: Dr J. Lockie, Department of Anaesthesia, Great
Ormond Street Hospital for Children NHS Trust, Great Ormond
Street, London WC1N 3JH, UK.
Hospital is routinely undertaken with the child in
the sitting position. In this particular case, an
anaesthetic involving infusions of propofol and
remifentanil was planned as a nontriggering anaes-
thetic, with the child sitting. The combination of
propofol with remifentanil as a total intravenous
anaesthetic has been shown to be suitable for adult
intracranial surgery (2). We report the successful use
of this combination in a child undergoing posterior
fossa surgery.
Case report
A 6-year-old boy, weighing 23 kg, presented for
craniectomy and excision of a posterior fossa tumour.
Preoperatively, he had suffered from intermittent
headaches with nausea and vomiting for 3 months.
He had presented via his optician who found he had
bilateral papilloedema. He had no other symptoms
or signs. His preoperative blood pressure was 90/
40 mmHg with a pulse of 90 bámin)1 and a Glasgow

498 Ó 2001 Blackwell Science Ltd

 M A N A G E M E N T O F M A L I G N A N T H Y P E R T H E R M IA
Coma Scale score of 15. Current medication
comprised dexamethasone 0.1 mgákg)1 5 h. There
was a history of MH in the family on his mother's
side, although neither he nor his mother had been
investigated. His grandfather's uncle and his grand-
father's ®rst cousin had both died under general
anaesthesia and four other cousins of the grandfather
had tested positive for MH.
The child had had a dental anaesthetic in a clinic
2 years previously. We were unable to obtain any
details of this but MH can occur in patients who
have had previous triggering anaesthetics (3). He
had an MRI scan performed without sedation or
anaesthesia. On the basis of a family history of
anaesthetic related deaths due to MH, the national
MH unit at St James's Hospital, Leeds was contac-
ted. The advice given was that the child had at most
a 6.25% chance of having a MH reaction and that
our usual anaesthetic with fentanyl, thiopentone and
vecuronium maintained with iso¯urane would
probably be safe. However, it was decided that a
trigger free anaesthetic avoiding the possibility of an
MH reaction was more appropriate, as management
of MH during posterior fossa surgery in the sitting
position would have been extremely dif®cult had it
become necessary.
No premedication was prescribed except Ametop
gel. A 22-G intravenous cannula was sited and
anaesthesia was induced with propofol (4 mgákg)1),
fentanyl (1 lgákg)1) and muscle relaxation produced
with vecuronium (0.1 mgákg)1). Full anaesthetic
monitoring was commenced including temperature.
A propofol infusion was commenced at a rate of
10 mgákg)1áh)1. The trachea was intubated with a
size 5 reinforced tube. A 20-G femoral line was
inserted and two 18-G peripheral cannulae were
inserted into his feet. A 22-G radial artery line was
also inserted. The child was slowly raised into a
sitting position and cardiovascular stability con-
®rmed over a period of 10 min. His blood pressure
was stable at 80/40 mmHg and his heart rate
80 bámin)1. He was then moved to the operating
theatre in the sitting position in preparation for
posterior fossa surgery. Anaesthesia was maintained
with an infusion of propofol at 10 mgákg)1áh)1 and
after surgical preparation an infusion of remifentanil
at 0.1 lgákg)1ámin)1 was started. Muscle paralysis
was maintained with intermittent boluses of vecu-
ronium 0.05±0.1 mgákg)1. The lungs were ventilated
Ó 2001 Blackwell Science Ltd, Paediatric Anaesthesia, 11, 498±500
499
with an oxygen/air mix via a fresh breathing system
using an anaesthetic machine, which had been
¯ushed through with oxygen for 1 h. The propofol
infusion rate was adjusted to 8 mgákg)1áh)1 after 1 h
and the remifentanil infusion increased to
0.15 lgákg)1ámin)1 after 2 h in response to an
increase in heart rate to 90 bámin)1 and an increase
in blood pressure to 100/55 mmHg for 5 min.
Otherwise, the patient was stable throughout the
operation and the operating conditions were good.
Endtidal carbon dioxide concentration was main-
tained at 4 kPa (30 mmHg) Intraoperative ¯uids
consisted of 200 ml of Hartmann's solution and
300 ml of colloid. Ten min prior to the termination of
surgery, remifentanil infusion was stopped. When
the skin stitches had been almost completed, a
diclofenac suppository (25 mg) was administered
and propofol infusion was then stopped.
Muscle relaxation was reversed with atropine
(25 lgákg)1) and neostigmine (50 lgákg)1). The
duration of surgery was 4 h and 30 min. The child
woke up within the following 10 min and was
extubated uneventfully and noted to be comfortable
and cooperative. He made a good postoperative
recovery but was a little drowsy over the ®rst 24 h
postoperatively and was troubled by postoperative
nausea.
Discussion
We report a case of a 6-year-old child who was
possibly MH susceptible having a nontriggering
anaesthetic of propofol/remifentanil/vecuronium
for excision of a posterior fossa tumour in the sitting
position.
Previous work has shown that an infusion of
remifentanil compares favourably with alfentanil
and fentanyl for supratentorial craniotomies in
adults, both in conjunction with propofol (2) and
iso¯urane maintained anaesthesia (4,5). There is
little change in intracranial pressure with remifen-
tanil, but there is a dose dependent decrease in mean
arterial pressure and cerebral perfusion pressure,
similar to that found with alfentanil. The rate of
infusion for propofol in children is recommended to
be higher than that for adults (6). In our case, we
were combining the propofol with remifentanil and
we particularly wanted to avoid hypotension in a
sitting neurosurgical case. A target controlled pump
5 00 M . A . W O O T T O N A N D J . L O C KI E
was not available and we are not aware of any data
for reliable formulae in this age group for this
combination of infusions. It was decided to use a
small bolus dose of fentanyl with induction until the
propofol infusion was started and the patient was in
the sitting position before we added the remifenta-
nil. We chose the combination of remifentanil and
propofol rather than continuing with fentanyl for
analgesia because we considered that we could
achieve more responsive cardiovascular control with
remifentanil than with fentanyl. During this 4 h and
30 min anaesthetic, the systolic blood pressure
remained at 80 ‹ 10 mmHg, with the exception of
a 5 min period of increased blood pressure (100/
55 mmHg). The preoperative blood pressure was
90/40 mmHg. The pulse remained steady at
70±80 bámin)1, with an increase to 90 bámin)1 in
association with the rise in blood pressure.
On emergence from anaesthesia, which was
within 10 min of cessation of the infusions of
propofol and remifentanil, the child was both com-
fortable and cooperative. It would seem therefore
that the combination of propofol and remifentanil
infusions for neurosurgery in children is one that
can be used successfully.
Acknowledgements
We are very grateful to Mr P. McEneaney, Senior
Operating Department Practitioner, for his help with
this dif®cult case.
References
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Accepted 16 January 2001
Ó 2001 Blackwell Science Ltd, Paediatric Anaesthesia, 11, 498±500