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3/24/2019 Arthrogryposis multiplex congenita: An anesthetic challenge

Anesth Essays Res. 2012 Jan-Jun; 6(1): 78–80. PMCID: PMC4173428


doi: 10.4103/0259-1162.103380: 10.4103/0259-1162.103380 PMID: 25885508

Arthrogryposis multiplex congenita: An anesthetic challenge


Vinayak Seenappa Pujari, Shivakumar Shivanna, Tejesh C. Anandaswamy, and A. C. Manjunath

Department of Anesthesiology, M S Ramaiah Medical College and Hospitals, Bangalore, India


Corresponding author: Dr. Pujari Vinayak, Department of Anesthesiology, M S Ramaiah Medical College and
Hospitals, Bangalore - 560 054, India. E-mail: drvinayak@hotmail.com

Copyright : © Anesthesia: Essays and Researches

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-
Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided
the original work is properly cited.

Abstract
Children with arthrogryposis multiplex congenita often require multiple orthopedic corrective procedures.
We present a case of a child with arthrogryposis multiplex congenita posted for contracture release of both
lower limbs that were successfully managed with total intravenous anesthesia and caudal epidural
analgesia with Bupernorphine as an additive.

Keywords: Arthrogryposis multiplex congenital, buprenorphine, caudal epidural analgesia, pediatric


anesthesia

INTRODUCTION
Arthrogryposis multiplex congenita (AMC) is a rare condition characterized by multiple joint contractures
found throughout the body at birth.[1] AMC may be associated with multiple congenital anomalies.[2]
Patients of AMC require anesthesia most often to correct orthopedic deformities and for muscle biopsy for
definitive diagnosis. AMC is a challenge to the anesthesiologist as it is associated with multiple anesthetic
implications.

The following is a case report of successful management of a 10-year-old girl with AMC posted for
orthopedic corrective surgery.

CASE REPORT
A 10-year-old girl with AMC presented for release of ankle and knee contractures of both lower limbs.
The parents were well informed about the disease and had been fully worked up elsewhere. The most
significant problems were lower limb and upper limb deformities. She was confined to the wheel chair and
had to be mobilized by her parents.

Her past history included two orthopedic surgeries done at the ages of 2 and 4 years, the anesthetic details
were unavailable. The patient required post-op ventilation after both the surgeries for a day.

On examination patient had a mallampati grade-3 airway. Her weight was 25 kgs and securing a good IV
access seemed difficult.

After discussion of all the possible options it was decided to manage the case with total intravenous
anesthesia (TIVA) with caudal epidural analgesia.

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3/24/2019 Arthrogryposis multiplex congenita: An anesthetic challenge

The patient was premedicated with syrup Promethazine 125 mg on the night and morning of surgery. The
routine monitoring was instituted and a 22 G IV cannula was inserted in the left forearm with difficulty.
The patient was given inj Midazolam 1 mg IV for smooth separation from the parents. The difficult airway
cart was kept ready, patient was induced with inj Fentanyl 50 mcg, inj Propofol 50 mg iv and after
ensuring smooth mask ventilation inj Rocuronium 25 mg iv was given. Direct laryngoscopy done with
MAC 2 blade, Lehane and Cormack grade-3 view was seen, which improved to grade-2 view with
backward and downward pressure on the larynx. The child was intubated with a six cuffed endotracheal
tube.

Anesthesia was maintained with an infusion of inj Propofol started at 5 mg/Kg/hr to achieve a BIS level of
50–60. She was ventilated with 33% oxygen and 66% nitrous oxide mixture with ventilation to maintain
end tidal carbon dioxide between 30–33%. The caudal epidural space was identified with a 23 G
hypodermic needle and 20 mL of 0.20% Bupivacaine and 30 mcg of Bupernorphine.

The left external jugular vein was cannulated with 18 G IV cannula. The vitals were stable throughout the
3 hr procedure. The Propofol infusion was reduced to 3 mg/Kg/ hr 20 min after of the start of surgery. The
total additional inj Rocuronium used was 15 mg. Inj Paracetamol 500 mg IV was infused. A convection
warmer was used through out surgery and the skin temperature was between 34–36°C.

The limbs were operated upon sequentially and tourniquet used for both. The Propofol infusion was
tapered and stopped at the last suture. The total duration of the surgery was three hours. The
neuromuscular blockade was reversed with inj Neostigmine 1.25 mg and inj Glycopyrollate 0.3 mg and
extubated after full reversal of neuromuscular blockade.

The patient was comfortable and had no pain she was monitored in the recovery room for an hour and was
then sent back to her room. The patient did not require any additional analgesics for the next 8 hr. NSAIDs
were used for postoperative analgesia. The child had an uneventful postoperative course and discharged
after 2 days.

DISCUSSION
Patients with AMC may come for multiple surgeries most often for correction of orthopedic deformities.
The challenges faced by the anesthesiologist are as follows.[1]

1. Difficult airway – micrognathia, high arched palate, cervical spine instability (under development of
first and second cervical vertebra).[2]
2. Malignant hyperthermia (MH) – avoid triggering agents such as Succinyl choline and inhalational
anesthetic agents (some variants have been associated with MH).[3]
3. Myopathy – increased sensitivity and prolonged duration of neuromuscular blockers.
4. Cardio-respiratory – increased sensitivity to induction agents. They may have a high risk of
postoperative respiratory depression and are prone to aspiration.
5. Difficult regional blockade – spine anomalies, difficult access to nerves due to joint contractures.[4]
6. Difficult IV access – reduced subcutaneous tissue and tense skin.
7. Difficult positioning – joint contractures and reduced muscle mass.

In our patient we did not anticipate a difficult airway, but we had to use a muscle relaxant and intubate the
patient as the surgeon wanted to position the patient prone or lateral if he had difficulty during the surgery.
But the surgeon could complete the surgery in supine position. We encountered difficult IV access and had
to cannulate the external jugular vein. We avoided triggering agents for MH[3,5] and we successfully
managed the case using TIVA with propofol and caudal epidural analgesia.[6] Ketamine infusion has been
used for management of these patients, we chose propofol for better cardiovascular stability.[7]

The use of regional anesthesia in these patients is controversial as one of the etiologies of AMC is a self-
limited anterior horn cell disease and may be technically difficult due to anatomical abnormalities. Caudal
analgesia has been successfully used for the management of postoperative pain in Freeman Sheldon
syndrome a variant of AMC.[8] Regional anesthesia in the form of Fascia iliaca block has been
successfully used for muscle biopsy in patients with AMC without any neurological sequale.[9] We have
used a caudal analgesia for supplementing TIVA, thus reducing the requirement of Propofol, muscle

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3/24/2019 Arthrogryposis multiplex congenita: An anesthetic challenge

relaxants and opiates. We used Bupernorphine as an additive to prolong post operative analgesia; our
patient was pain free for 8 hr without any complications. Intrathecal morphine has been used for the
management of post operative pain in patients with AMC. We chose Bupernorphine over Morphine as the
duration of action is longer and without any serious side effects.[10] We could extubate the child and she
did not require post operative ventilation or ICU care.

In conclusion, AMC can present many challenges for anesthetist especially in children. The combination
of TIVA and caudal epidural analgesia with Bupernorphine appears to be a suitable anesthetic technique
for patients with AMC. This technique provided good conditions for rapid recovery from anesthesia with
good postoperative analgesia.

Footnotes
Source of Support: Nil

Conflict of Interest: None declared.

REFERENCES
1. Hall JG. Overview of Arthrogryposis. In: Staheli LT, Hall JG, Jaffe KM, Paholke OD, editors.
Arthrogryposis: A text atlas. New York: Cambridge University Press; 1998. pp. 1–26.

2. Nguyen NH, Morvant EM, Mayhew JF. Anesthetic management for patients with arthrogryposis
multiplex congenita and severe micrognathia: Case reports. J Clin Anesth. 2000;12:227–30. [PubMed:
10869924]

3. Hopkins PM, Ellis FR, Halsall PJ. Hypermetabolism in arthrogryposis multiplex congenita.
Anaesthesia. 1991;46:374–5. [PubMed: 2035784]

4. Quance DR. Anaesthetic management of an obstetrical patient with arthrogryposis multiplex congenita.
Can J Anaesth. 1988;35:612–4. [PubMed: 3203454]

5. Kanaya N, Nakayama M, Nakae Y, Kobayashi I, Tsuchida H, Namiki A. Hyperthermia during


sevoflurane anaesthesia in arthrogryposis multiplex congenita with central nervous system dysfunction.
Paediatr Anaesth. 1996;6:428–9. [PubMed: 8880828]

6. Shiraishi M, Minami K, Shigematsu A. Anesthesia using propofol for a patient with arthrogryposis
multiplex congenital [Article in Japanese] Masui. 2001;50:637–8. [PubMed: 11452472]

7. Oberoi GS, Kaul HL, Gill IS, Batra RK. Anaesthesia in arthrogryposis multiplex congenita: Case report.
Can J Anaesth. 1987;34:288–90. [PubMed: 3581399]

8. Duggar RG, Jr, DeMars PD, Bolton VE. Whistling Face syndrome: General anaesthesia and early
postoperative caudal analgesia. Anesthesiology. 1989;70:545–7. [PubMed: 2923300]

9. Ion T, Cook-Sather SD, Finkel RS, Cucchiaro G. Fascia iliaca block for an infant with arthrogryposis
multiplex congenita undergoing muscle biopsy. Anesth Analg. 2005;100:82–4. [PubMed: 15616056]

10. de Beer DA, Thomas ML. Caudal additives in children—solutions or problems. Br J Anaesth.
2003;90:487–98. [PubMed: 12644422]

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