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Culture Documents
doi: 10.1093/bjaceaccp/mkv014
Advance Access Publication Date: 8 June 2015
Matrix reference
2D02, 2D05, 3D00
Spina bifida
Key points
Caused by incomplete closure of the embryonic neural tube,
• Musculoskeletal disorders can be manifestations of spina bifida has a range of clinical presentations, from spina bifida
a systemic disease. occulta to meningocele and Arnold–Chiari malformation asso-
ciated with hydrocephalus. The total incidence of spina bifida
• There may be relative or absolute contraindications
is 5.8 per 10 000 total births but the incidence of elective termin-
to the use of non-steroidal anti-inflammatory drugs
ation in affected pregnancies is ∼72%, so incidence in live births is
or central neuraxial block.
much lower. Ten year survival now exceeds 80%.1
• Blood loss can be substantial unless a tourniquet is Myelomeningocele is a sac containing meninges and neural
used. elements bulging through a vertebral defect and which is closed
surgically shortly after birth, but the underlying spinal cord dam-
• Many conditions are associated with difficult air-
age remains. Abnormal lower limb growth and denervation can
way management.
lead to developmental abnormalities such as hip dysplasia and
• Some co-morbidities are associated with a hyper- talipes equinovarus (clubfoot). Neuromuscular imbalance occurs
metabolic state that can be confused with malig- when spastic or unopposed muscles work against flaccid mus-
nant hyperpyrexia. cles, causing joint deformity. Surgical intervention in the form
of soft tissue release and bone realignment is then required to
preserve mobility.
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58
Anaesthesia for paediatric lower limb surgery
Infants under 6 months of age generally have joint sublux- the duration of surgery and potentially increases analgesic
ation without associated bony deformity and are treated non- requirements.
surgically in a Pavlick harness for up to 8 weeks until both hips
are stable. Children over 6 months of age or those with inad- Related conditions
equate reduction after conservative treatment require open or
fluoroscopically guided closed manipulation and reduction A thorough history of related diagnoses is essential. Important
under general anaesthesia, with application of a hip spica cast co-morbidities include scoliosis and restrictive pulmonary dis-
to maintain the correct joint position until the hip becomes ease, congenital or acquired cardiac lesions, craniocervical and
stable. After 18 months of age, open reduction is the treatment facial abnormalities, hydrocephalus, dental disease, and obesity.
of choice. Generally no specific anaesthetic precautions are Pre-existing neurological deficits, contractures, deformities, and
required as these tend to be otherwise healthy children. fractures should also be carefully documented to aid the
planning of positioning and handling.
Cerebral palsy
Airway assessment
Preoperative evaluation A thorough evaluation of the airway is imperative to highlight
Preoperative assessment concerns vary with the age and medical any increased risk for inadequate mask ventilation and difficult
condition of the child. Planning is best done in conjunction with tracheal intubation, and to plan an airway management strategy.
the surgeon to be aware of the location of the incision site (to plan For example, in OI there may be craniocervical instability, brittle
regional techniques) the length of the procedure and any individ- teeth, a cleft palate, and pre-existing facial fractures. In arthogry-
ual surgical preferences. The surgeon may also plan multiple posis, there may be micrognathia, gastro-oesphageal reflux, and
interventions in one session and this significantly extends limited neck movement because of contractures.
Laboratory tests should be inserted at sites of sensory loss in the child with
spina bifida, to minimize discomfort. Finally, the use of intraoss-
Abnormalities in full blood count or coagulation studies should
eous needles is contraindicated in OI.
be discussed with the surgical team and consideration given to
postponing the procedure until the abnormality is corrected. If
the starting haemoglobin is low (our institution would consider Airway management
the lower limits of safety to be 70 g litre−1) or significant blood Children with dwarfism, OI, and arthrogryposis present some of
loss is anticipated, cross-matched blood should be available be- the most challenging airway management problems in paediat-
fore starting the procedure. In open procedures where a tourni- ric anaesthesia.
quet is not used the potential for blood loss is significant and In arthrogryposis mask ventilation and intubation can be dif-
the requirement for preoperative blood typing or cross-matching ficult or impossible as the temporomandibular joint and cervical
should be discussed with the surgeon. Preoperative blood testing spine can be stiffened by contractures that worsen with increas-
should also include screening for sickle cell disease where appro- ing age. Micrognathia is an associated feature that may further
priate, especially if tourniquet use is planned.
Hypermetabolic states
Arthrogryposis and OI are associated with a hypermetabolic state
under anaesthesia in ∼33% of cases.7 An increase in body tem- Fig 1 Picture of a child undergoing an orthopaedic procedure. In this case,
perature is accompanied by tachycardia, increased end-tidal car- application of a plaster jacket to aid correction of spinal scoliosis. Written
bon dioxide, and acidosis. This state is distinct from malignant parental permission obtained.
hyperthermia (MH) in that the only treatment usually needed is
active cooling. Dantrolene is not required. The hypermetabolic
state occurs even in the absence of MH trigger agents, does not intra-operative warming may need to be discontinued. There is
manifest with muscle rigidity, urinary myoglobin is not detect- no evidence-based or consensus opinion to suggest a maximum
able and in vitro muscle contracture testing is negative. Intra-op- safe inflation time but steps should be taken to keep this to a min-
erative temperature should be carefully monitored and the usual imum in order to reduce the risk of irreversible cellular ischaemic
paediatric perioperative warming strategies discontinued as damage.
necessary. Minute ventilation should be increased for 5 min after tourni-
quet deflation to compensate for an increase in PaCO2 as venous
stasis resolves.
Tourniquets If a tourniquet is not used either for clinical reasons or
because the site is too proximal—for instance in hip surgery—
The benefits of tourniquets in lower limb surgery are a bloodless
substantial blood loss may occur. Cell-salvage techniques can
operating field and limited intra-operative blood loss. They can
dramatically reduce the requirement for cross-matched blood,
potentially be used for any operative site distal to the mid-thigh
but it is still important to have blood available. Some clinicians
and are widely used for ankle procedures. It is important to accur-
give additional fluids early in the procedure to lower haematocrit
ately size the tourniquet with a width greater than half the limb’s
and reduce red cell loss.
diameter in order to safely distribute pressure. In children the
recommended inflation pressures are determined by blood pres-
sure and limb size – for the lower limb 150 mm Hg above systolic
blood pressure is sufficient. Anaesthetic implications for common
The use of tourniquets in patients with sickle cell anaemia or paediatric orthopaedic procedures
trait is controversial but there is no absolute contraindication
Spica casts
provided that the benefits outweigh risk on a per-patient basis.8
It is important to ensure adequate haemoglobin correction, Typically used after hip surgery or to treat DDH, this procedure
patient warming, hydration, oxygenation, and maintenance of can take up to 90 min. The name originates from Latin ‘ear of
acid–base balance to prevent subsequent localized sickle crisis grain’ referring to the figure-of-eight appearance of plaster ban-
in the operative limb. The limb should be carefully exsanguinated dage wrappings. Spica casts immobilize one or both lower
and then reperfused in stages to avoid triggering a generalized limbs generally with knees in flexion and hips in flexion, abduc-
sickle crisis by the release of a large load of hypoxic, acidotic tion, and external rotation. A fibreglass cast is applied to envelop
blood into the circulation. the mid and lower chest, the hips, the thighs, and one or both legs
Tourniquets are inadvisable in OI because of the risk of frac- to the ankle (Fig. 1).
ture as the tourniquet is inflated. Patients with thin skin, contrac- The child is positioned on a spica table by several assistants
tures, and reduced muscle mass need very careful padding under with one support under the shoulders, one under the pelvis,
the tourniquet if it is considered necessary. and a post at the perineum. Care with the airway is required
On exsanguination and inflation of the tourniquet up to 15% during positioning on the spica table, especially if a supra-glottic
of circulating blood volume is redistributed. This may increase airway is used.
blood pressure by up to 30%, with a parallel increase in central A towel is placed under the cast during moulding to allow
venous pressure. chest expansion and a window is cut to allow for abdominal
Prolonged use of bilateral tourniquets (>90 min) is associated breathing. When the surgeon applies the abdominal and chest
with an increase in core temperature (1–2°C) and tachycardia so components pay close attention to ventilation—a ventilator
loop display is invaluable as an early indicator of over-tight wrap- experience of painful fixator may need an epidural in order to
ping. Epidurals are generally avoided as the lumbar spine is agree to undergo the procedure.
inaccessible once the spica cast has been applied.
Tendon surgery
This is used to correct imbalance of muscle forces in joint de-
Pain management
formity and/or improve gait. Tendon transfers detach a partial Minor procedures such as botox injection require only simple
or full tendon from its point of insertion, retain its innervation analgesia.
and vasculature and re-attach it to another bone or tendon Procedures involving bone or tendons can be extremely pain-
point. Lengthening procedures involve two tendon cuts offset ful so it is important to provide multi-modal analgesia including
from one another and tendon releases or tenotomies completely consideration of regional or local techniques, paracetamol and
detach the tendon from the insertion point. The child may be NSAIDs. A multi-modal approach reduces opioid use, limiting
placed in a cast after operation to encourage healing in the de- the increased side-effect profile known to occur in children. Pre-
sired joint position. The cast also limits movement and therefore operative single-dose gabapentin has insufficient evidence to
reduces analgesic requirements, which can be substantial. Com- support routine use in paediatric orthopaedic surgery but has
mon sites for tendon manipulation include the hip via groin inci- been shown to significantly decrease postoperative pain and res-
sion, hamstrings via posterior thigh incision ( prone/lateral cue analgesic requirements in adults who undergo lower limb
positioning) and the ankle. Painful postoperative muscle spasm orthopaedic surgery.9
is common after tendon manipulation. It is important to use an age and ability appropriate pain scale
for postoperative assessment such as Wong-Baker Faces for chil-
dren aged over 3 yr, FLACC (Face, Legs, Activity, Cry, Consolabil-
Limb lengthening
ity) for children aged 2 months to 7 yr, or revised FLACC for
The Ilizarov method is used to preserve height in limb length in- children with developmental impairment.10
equality caused by unequal growth, trauma, or osteomyelitis. It is Patients with communication difficulties such as in CP may
a lengthy and painful process of cortical osteotomy (corticotomy) find it difficult to express postoperative pain so regular (rather
distraction and fixation. Operations are prolonged (typically 10 h than as required) analgesia used in combination with careful
or more) and technically difficult as an external fixator is placed monitoring of pain score may be preferable (Fig. 2).
around the osteotomy to retain distraction of the bone. The fixa- Regional anaesthesia can become an increasing challenge as
tor is periodically tightened with approximately 1 cm of new the child with musculoskeletal disease ages beyond infancy. A
bone growth for each month of therapy. secured difficult airway may be jeopardized by repositioning for
After operation, transient nerve palsies are possible so care- central neuraxial block. Caudal or lumbar epidurals are highly
fully document any pre-existing conditions. These children can recommended for major procedures but can be contraindicated
be at high risk of compartment syndrome, especially with tibial in OI-related platelet dysfunction and the abnormal spinal
lengthening. The procedure is very painful but neuraxial techni- anatomy of spina bifida. They can be technically challenging be-
ques are often avoided as there is a need to closely monitor nerve cause of patient positioning, bone condition and scoliosis. The
and vascular recovery and to detect compartment syndrome. spread of injectate can be difficult to predict in children with skel-
High-dose opioids via patient- or nurse-controlled analgesia etal abnormalities. There may be fears that the block would mask
may then be the best option and it is worth discussing a short compartment pain in procedures with a significant risk of post-
course of NSAIDs with the surgical team. A child with a previous operative compartment syndrome.
Dense sensory loss around the operative site in spina bifida perfusion pressure of <30 mm Hg may indicate a diagnosis of com-
may significantly reduce analgesia requirements (Table 1). partment syndrome and the need for fasciotomy.
A 2009 review showed no cases of patients aged <18 yr where
epidural analgesia had completely masked the pain of compart-
Compartment syndrome and regional anaesthesia
ment syndrome or caused delay in diagnosis12 and a literature
Certain procedures such as Ilizarov limb-lengthening carry a risk search by the authors did not find any more recent reports to
of postoperative compartment syndrome distal to the operative March 2014.
site in ∼2% of patients.11 Some teams may therefore wish to The most important aspect of early detection of compartment
avoid regional anaesthesia on the basis that it potentially masks syndrome is clinical vigilance for disproportionate pain, swelling,
the pain of compartment syndrome. Children as a group may paresthaesia, and paralysis of the operative limb.13 If called to as-
be at increased risk of developing compartment syndrome as sess a child with uncontrolled postoperative pain despite good
their physiological mean arterial pressure (MAP) is lower, giving provision of analgesia, jointly review with a surgeon and exam-
a reduced compartmental perfusion pressure (Δp = MAP − ine the painful site. Consider pain distal to the site of surgery
compartment pressure). Compartment pressure can be measured and/or increasing pain that is unresponsive to analgesia as
using a standard invasive arterial pressure monitoring set con- compartment syndrome until proven otherwise. Encourage a
nected to a cannula in the compartment at risk. A compartment low surgical threshold for compartment pressure monitoring.
Table 1 Suggested analgesia options for commonly performed paediatric lower limb orthopaedic procedures. *In agreement with the surgical
team
Surgical procedure Paracetamol NSAIDs* Caudal Epidural PCA/ Popliteal and Sciatic Femoral
infusion NCA saphenous block block block
Other strategies for limiting compartment syndrome can be Postoperative muscle spasms
surgical such as prophylactic fasciotomies at time of procedure,
Many children with co-morbidities such as CP will suffer chronic
or anaesthetic (e.g. using low concentration local anaesthesia to
spasticity. Their anti-spasticity treatment should be continued
avoid dense sensory, motor, and vasomotor block).
perioperatively to prevent withdrawal and worsening of symptoms.
With careful postoperative observation, epidural anaesthesia
In addition, surgical procedures such as osteotomy or tendon
can be offered appropriately to patients who are not at high risk of
lengthening pre-dispose to painful postoperative muscle spasm.
developing compartment syndrome. This involves good training
Acute postoperative spasms are best managed in a critical
of recovery and ward nurses in red flag signs and symptoms. It is
care environment by control of pain with the techniques de-
important to remember that theoretically even patient- or nurse-
scribed above and then the addition of i.v. benzodiazepine with
controlled opioid analgesia can mask the early stages of compart-
careful monitoring for excessive sedation and respiratory depres-
ment syndrome (Box 1).
sion. Suggested regimens include diazepam 0.1 mg kg−1, or mid-
azolam infusion 10–30 μg kg−1 h−1. Baclofen or dantrolene are also
sometimes used as sole agents or in conjunction with
Box 1 Red flag signs of acute compartment syndrome
benzodiazepines.
Increasing or uncontrolled pain despite good provision of
analgesia
Postoperative care
Pain remote to site of surgery
Paresthaesia or paralysis not attributable to analgesia, and Many minor procedures can be performed as day cases but lim-
not resolving despite cessation of local anaesthetic infusion ited mobility, airway abnormalities, blood loss and the need for
Reduced perfusion of areas distal to painful site significant analgesia may require several nights of in-patient
Tight swelling care. In addition, children with co-morbidities including upper
Pain on passive movement of the site airway abnormalities or significant cardiorespiratory disease
and those having long or complex surgery with significant
blood loss will require postoperative critical care.
4. United Nations Childrens’ Fund. A summary of the United 10. Malviya S, Voepel-Lewis T, Burke C, Merkel S, Tait R. The re-
Nations Convention on the Rights of the Child, 2009 vised FLACC observational pain tool: improved reliability and
5. Oppitz F, Speulda E, Goeters C, Roedl R, Busley R. Anesthesia re- validity for pain assessment in children with cognitive im-
commendations in patients suffering from achondroplasia. Orpha- pairment. Pediatr Anesth 2006; 16: 258–65
nAnesthesia. Available from http://www.orphananesthesia.eu/en/ 11. Zuckerberg AL, Yaster M. Anaesthesia for orthopedic surgery.
component/docman/doc_download/81-achondroplasia.html (ac- In: Motoyama EK, Davis PL, eds. Smith’s Anesthesia for Infants
cessed 6 June 2014) and Children. Missouri: Mosby, 1996; 605–32
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commendations in patients suffering from Arthrogryposis diagnosis of lower limb compartment syndrome in children?
multiplex congenita. OrphanAnesthesia. Available from Pediatr Anesth 2009; 19: 83–91
http://www.orphananesthesia.eu/en/component/docman/ 13. Farrow C, Bodenham A, Troxler M. Acute limb compartment
doc_download/80-arthrogryposis-multiplex-congenita.html syndromes. Contin Educ Anaesth Crit Care Pain 2011; 11: 24–8
(accessed 4 June 2014) 14. Cappello T, Nuelle JAV, Katsantonis N et al. Ketorolac admin-