Mayo Clinic Children’s Center

Anesthetic Complications of Children with Arthrogryposis Syndromes: A Retrospective Case-Control Study

Stephen J. Gleich, M.D.1; Michael Tien, B.S.2; Darrell R. Schroeder, M.S.3; Randall Flick, M.D., M.P.H.1; Michael E. Nemergut, M.D., Ph.D.1

1Departments of Anesthesiology and Pediatrics; 2Mayo Medical School; 3Department of Health Sciences Research, Biomedical Statistics and Informatics Mayo Clinic, Rochester, MN

© 2016 Mayo Foundation for Medical Education and Research

BackgroundArthrogryposis syndromes are a heterogeneous group of disorders, characterized by congenital joint contractures, requiring multiple surgeries during childhood to address skeletal and visceral abnormalities. Prior reports suggest these children have increased perioperative risk, including hyperthermia and hypermetabolic events, discrete from malignant hyperthermia (MH), difficult airway management, and difficult IV placement (1-3).

Study AimWe sought to compare children with Arthrogryposis Multiplex Congenita (AMC) vs. the less severe, Distal Arthrogryposis Syndromes (DAS) and to evaluate possible intraoperative hyperthermia of AMC patients. We hypothesized that children with AMC demonstrated greater difficulty with airway management, IV access, and had a greater prevalence of intraoperative hyperthermia.

MethodsChildren age 0-25 years with arthrogryposis syndromes who underwent anesthesia from 1972-2014 were identified. The medical records were reviewed for demographics, arthrogryposis type, and anesthetic complications. AMC subjects were compared to those with DAS.

To further evaluate the potential risk of hyperthermia and hypermetabolic responses of AMC patients, we performed a post-hoc case-control analysis. Patients with AMC were matched in a 1:2 ratio to patients without arthrogryposis to evaluate the primary outcome of maximum intraoperative temperature.

Table 1. Patient Characteristics

AMC (n=45) DAS (n=16) P-Value

Sex Male Female

26 (57.8%)19 (42.2%)

9 (56.3%)7 (43.7%)

0.92

Pulmonary Disease 18 (40.0%) 2 (12.5%) 0.04*

Neurologic Disease 30 (66.7%) 5 (31.3%) 0.01*

Cardiac Disease 12 (26.7%) 3 (18.8%) 0.53

AMC = Arthrogryposis Multiplex CongenitaDAS = Distal Arthrogryposis Syndromes

Results45 patients with AMC and 16 patients with DAS underwent 266 and 106 unique anesthetics, respectively.

Children with AMC had a greater co-existing disease burden (P<0.01), underwent longer mean duration of anesthesia (P=0.01), and had more difficult IV access (P<0.01). There was no difference in intraoperative hyperthermia/hypermetabolic events (P=0.90) or difficult airway (P=0.20) between groups.

Case-Control AnalysisVia post-hoc case-control analysis, there was no evidence of intraoperative hyperthermia among AMC patients compared to controls.

• Maximum intraoperative temperature difference +0.04˚C (95% CI -0.14-0.22, P=0.63)

ConclusionsChildren with arthrogryposis syndromes present challenges to the anesthesia and surgical teams, including a greater burden of co-existing disease and difficult IV access. Contrary to prior reports, we did not find evidence of an increased risk of intraoperative hyperthermia/hypermetabolic responses or difficulty in airway management.

References1. Oda Y, Yukioka H, Fujimori M. Anesthesia for arthrogryposis multiplex

congenita--report of 12 cases. J Anesth 1990; 4(3): 275-8.

2. Martin S, Tobias JD. Perioperative care of the child with arthrogryposis. Paediatric Anaesthesia 2006; 16: 31-7.

3. Nguyen NH, Morvant EM, Mayhew JF. Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports. J Clin Anesth 2000; 12(3): 227-30.

Table 2. Anesthetic Management

AMC (n=266) DAS (n=106) P-Value

ASA Physical Status ASA I ASA II ASA III ASA IV

40 (15.0%)120 (45.1%)101 (38.0%)

5 (1.9%)

43 (40.6%)44 (41.5%) 19 (17.9%)

0 (0.0%)

< 0.01*

Airway Difficulty Score† 0 1 2

153 (89.5%)15 (8.8%)3 (1.8%)

55 (96.5%)1 (1.8%)1 (1.8%)

0.20

Difficult Intravenous Catheterization

32 (12.0%) 2 (1.9%) < 0.01*

Hemodynamic Instability 20 (7.5%) 2 (1.9%) 0.04*

Intraoperative Hyperthermia 23 (8.6%) 8 (7.5%) 0.90

Postoperative Disposition Outpatient General Care Floor Intensive Care Unit

97 (36.5%)119 (44.7%)50 (18.8%)

58 (54.7%)37 (34.9%) 11 (10.4%)

< 0.01*

AMC = Arthrogryposis Multiplex CongenitaDAS = Distal Arthrogryposis Syndromes†Not all procedures required airway management