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Anaesthesiology for Children With Arthrogryposis

Savenkov, Artem N. MD*; Pajardi, Giorgio E. MD; Agranovich, Olga E. MD, PhD*; Zabolskiy, Dmitry MD*; van Bosse, Harold J.P. MD

Journal of Pediatric Orthopaedics: July/August 2017 - Volume 37 - Issue - p S27–S28
doi: 10.1097/BPO.0000000000000998
Arthrogryposis 2017 Supplement

Patients with arthrogryposis often require anesthesia 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.

*Arthrogryposis Clinic, Turner Scientific and Research Institute for Children’s Orthopedics, Pushkin, Saint Petersburg, Russia

Istituto di Chirurgia Plastica, Università degli Studi di Milano, Milan, Italy

Shriners 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:

Nearly all patients with arthrogrypotic conditions will undergo at least 1 surgical procedure requiring anesthesia. Many of the underlying conditions pose a challenge 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.

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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. Furthermore, 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 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 multiplex congenita with underlying myopathies are well associated with this phenomenon.2 Mask ventilation was found to be unrestricted, allowing for an unrushed intubation. 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.

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Recent literature suggests that the association between arthrogryposis multiplex congenita and malignant hyperthermia is weak, and the risk is small.3 Recommendations 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 µg/kg and acetaminophen 125 to 250 mg rectally according to weight.

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For upper extremity surgery, axillary, supraclavicular, and infraclavicular blocks modulate intraoperative pain with initial postoperative pain relief. The blocks are often applied under ultrasonographic guidance. For lower extremity surgery, paravertebral, lumbar epidural, iliofascial, femoral nerve, and sciatic nerve blocks provide a similar function. With the use of indwelling catheters, pain can be controlled up to 14 days, particularly useful for aggressive postoperative passive range of motion of joints therapy. Medications used include 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.

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 postoperatively.

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1. Cormack RS, Lehane J. Difficult tracheal intubation in obstetrics. 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.

arthrogryposis; arthrogryposis multiplex congenital; amyoplasia; congenital contracture syndromes; distal arthrogryposis; Cormack-Lehane; malignant hyperthermia; peripheral nerve blocks

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