A 6-year-old boy with a history of arthrogryposis is presenting for an Achilles tendon release and tendon transfer.
Physical examination shows contracture of all four extremities, limited range of motion of the neck, and limited mouth opening.
Arthrogryposis is a nonprogressive congenital disorder. In most cases, it is not a genetic condition. However, genetic conditions may be found in about 30% of patients. Arthrogryposis is caused by fetal akinesia secondary to many different conditions (neurologic, muscular, or connective tissue abnormalities). Most frequently it affects the wrist, elbow, shoulder, hip, knee, and ankle joints, but in a severe form, all joints might be involved. This condition is not usually progressive, and physical therapy, occupational therapy, and surgical intervention may significantly improve the patient’s quality of life.
Some arthrogryposis patients may have significant scoliosis and respiratory compromise because of restrictive lung disease. You may need to evaluate their respiratory reserve and create a plan for postoperative respiratory support. Significant involvement of cervical vertebrae and the temporomandibular joint may result in a difficult intubation. Careful physical examination and history taking are necessary for airway assessment.
Positioning may be very difficult. Good padding and protection of pressure points are crucial. Refrain from excess force during positioning.
Consider awake intubation vs intubation after induction of anesthesia.
Use caution with patients who have respiratory insufficiency.
There are reports of significant postoperative hyperthermia. A trigger-free anesthetic may provide a less confusing picture when the patient develops hyperthermia postoperatively.
Intravenous access may be very difficult to obtain.
If the patient has significant respiratory issue, the use of regional anesthesia for postoperative pain control is recommended. Use a popliteal nerve block combined with a saphenous nerve block or a caudal epidural block.
Most patients can be extubated immediately, but some patients may need to be admitted to the intensive care unit for postoperative ventilation if they have significant restrictive pulmonary disease.
DOs and DON’Ts
✓ Do a detailed airway assessment and prepare for a possible difficult intubation.
⊗ Do not use excess force during positioning.
✓ Do use a trigger-free anesthetic to reduce confusion with malignant hyperthermia when patient develops hyperthermia postoperatively.
⊗ Do not assume that any hyperthermia is malignant hyperthermia.
There is an association with postoperative hyperthermia in these patients. However, there is no proven connection between arthrogryposis and malignant hyperthermia.
Positive end-expiratory pressure does not improve the restrictive pulmonary disease that is sometimes seen with severe cases of arthrogryposis.