ARTHROGRYPOSIS 2017 SUPPLEMENT
Anaesthesiology for Children With Arthrogryposis
Artem N. Savenkov, MD,* Giorgio E. Pajardi, MD,w Olga E. Agranovich, MD, PhD,*
Dmitry Zabolskiy, MD,* and Harold J.P. van Bosse, MDz
Abstract: Patients with arthrogryposis often require anesthesia
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for surgical procedures. Intubation can be challenging due to
lack of visualization. Anesthetic maintenance is fairly routine.
Pheripheral blocks are an important adjunct to postoperative
pain management.
Key Words: arthrogryposis, arthrogryposis multiplex congenital,
amyoplasia, congenital contracture syndromes, distal arthrog-
ryposis, Cormack-Lehane, malignant hyperthermia, peripheral
nerve blocks
(J Pediatr Orthop 2017;37:S27–S28)
N early all patients with arthrogrypotic conditions
will undergo at least 1 surgical procedure requiring
anesthesia. Many of the underlying conditions pose a chal-
lenge for anesthetic treatment, including issues of difficult
intubation, appropriate anesthetic agents, and postoperative
pain management. We will discuss information presented at
the Second International Symposium on Arthrogryposis in
Saint Petersburg, Russia in September 2014.
INTUBATION
Patients with arthrogryposis often have facial and neck
involvement. This may include micrognathia, Pierre Robin
sequence (micrognathia, cleft palate, and retroglossoptosis),
limited mouth opening, and limited neck extension. Fur-
thermore, the glottis may be difficult to visualize. In 1 series
of children with arthrogryposis, the Cormack and-Lehane
scale1 of glottis visualization on direct laryngoscopy was
used. Three children generically labeled “arthrogryposis”
were Cormack-Lehane classification grade II (grade IIa
partial view of glottis, grade IIb—only posterior extremity of
glottis or arytenoid cartilages seen), and 6 children with the
Freeman-Sheldon distal arthrogryposis were grade III (only
epiglottis seen, none of glottis viewed). Intubations in this
From the *Arthrogryposis Clinic, Turner Scientific and Research
Institute for Children’s Orthopedics, Pushkin, Saint Petersburg,
Russia; wIstituto di Chirurgia Plastica, Università degli Studi di
Milano, Milan, Italy; and zShriners Hospitals for Children,
Philadelphia, PA.
Authors have no sources of support or funding to declare.
The authors declare no conflicts of interest.
Reprints: Harold J.P. van Bosse, MD, Shriners Hospital for Children,
3551 North Broad Street, Philadelphia, PA 19140. E-mail:
hvanbosse@shrinenet.org.
Copyright r 2017 Wolters Kluwer Health, Inc. All rights reserved.
DOI: 10.1097/BPO.0000000000000998
J Pediatr Orthop  Volume 37, Number 5 Supplement 1, July/August 2017
Copyright r 2017 Wolters Kluwer Health, Inc. All rights reserved.
series were all performed with neuromuscular blocking
agents. The authors recognize that some prefer to avoid
those agents out of concerns of sensitivity to depolarizing
agents in children with arthrogryposis multiplex congenita
(AMC), but note that only forms of arthrogryposis mul-
tiplex congenita with underlying myopathies are well as-
sociated with this phenomenon.2 Mask ventilation was
found to be unrestricted, allowing for an unrushed in-
tubation. Occasionally, for patients with limited jaw
openings identified well in advance of surgery, a program
of gradual mouth opening dilation is fruitful, and also
allows for better dental hygiene. As in all cases with
difficult airways or limited mouth opening, fiber-optic–
assisted intubation should be considered as an option, and
at least be available to the anesthesiologist in case of a
complicated intubation.
ANESTHETIC MAINTENANCE
Recent literature suggests that the association be-
tween arthrogryposis multiplex congenita and malignant
hyperthermia is weak, and the risk is small.3 Recom-
mendations still are to avoid succinylcholine, but the
volatile inhalational agents apparently do not pose a risk,
outside of conditions such as the muscular dystrophies or
myotonia.
One strategy is to maintain anesthesia with 2%
sevoflurane in a mixture of 40% oxygen and 60% nitrous
oxide. Analgesia is provided by intravenous fentanyl
2 mg/kg and acetaminophen 125 to 250 mg rectally ac-
cording to weight.
PERIPHERAL NERVE BLOCKS
For upper extremity surgery, axillary, supra-
clavicular, and infraclavicular blocks modulate intra-
operative pain with initial postoperative pain relief. The
blocks are often applied under ultrasonographic guid-
ance. For lower extremity surgery, paravertebral, lumbar
epidural, iliofascial, femoral nerve, and sciatic nerve
blocks provide a similar function. With the use of in-
dwelling catheters, pain can be controlled up to 14 days,
particularly useful for aggressive postoperative passive
range of motion of joints therapy. Medications used in-
clude ropivacaine (0.2%, 0.375%, 0.5%, and 0.75%),
bupivacaine (0.5%), and lidocaine (2%). Epidural and
spinal blocks are probably only suitable for 2 or 3 days,
and the patient needs to be in a monitored unit, at bedrest
to prevent catheter dislodgement, and with a bladder
catheter, as they will not be able to void spontaneously.
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Savenkov et al J Pediatr Orthop  Volume 37, Number 5 Supplement 1, July/August 2017

An alternative is a subcutaneously tunneled catheter that
slowly delivers local anesthesia to the surgical site (On-Q
Pain Relief System, Halyard Health Inc.). Nerve blocks,
epidural catheters, and subcutaneous catheters allow for a
decrease in the amount of narcotic that is needed post-
operatively.
REFERENCES
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Anaesthesia. 1984;39:1105–1111.
2. Lonsdale H, Owen J. Anaesthesia for paediatric lower limb surgery.
BJA Educ. 2016;16:58–65.
3. Benca J, Hogan K. Malignant hyperthermia, coexisting disorders,
and enzymopathies: risks and management options. Anesth Analg.
2009;109:1049–1053.

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Copyright r 2017 Wolters Kluwer Health, Inc. All rights reserved.