Perioperative care of the child with arthrogryposis.

BACKGROUND

Arthrogryposis multiplex congenita (AMC) is a congenital symptom complex characterized by multiple joint contractures. AMC results from fetal akinesia due to extrinsic factors (oligohydramnios) or primary neurogenic or myopathic conditions of the fetus.

METHODS

We retrospectively reviewed our perioperative experience in 12 patients with AMC.

RESULTS

Intraoperative issues noted in our patients included difficulties with peripheral IV placement (n = 3), difficult tracheal intubation (n = 4), and intraoperative hyperthermia (n = 4). Difficulties with peripheral IV placement were more common in younger patients (2.3 +/- 0.6 vs 9.2 +/- 3.5 years). Although easy bag-valve-mask ventilation was present in all 12 patients, difficulty with tracheal intubation was noted in four patients. In these four patients, the airway was secured using an LMA (n = 3) or a facemask (n = 1). The patients in whom difficulties with tracheal intubation were encountered were older than patients in whom no difficulties were noted (11.0 +/- 2.9 vs 5.8 +/- 3.9 years). Four patients developed intraoperative temperatures > or =37 degrees C; however, there was no evidence of hypercarbia or tachycardia suggestive of malignant hyperthermia. Postoperative issues included stridor (n = 2) and postoperative atelectasis (n = 3).

CONCLUSIONS

The perioperative care of patients with AMC should consider not only the physical manifestations of the disease process, but also the implications of the underlying neuromuscular disease. The most common perioperative issues included difficulties with airway management, problematic intravenous access, and intraoperative hyperthermia.