Children who have connective tissue disorders may have multiple organ system involvement. These patients are often treated with aspirin or other nonsteroidal antiinflammatory drugs, which may complicate their perioperative management further by causing a bleeding diathesis resulting from platelet dysfunction. The effect of aspirin and nonsteroidal antiinflammatory drugs on platelet function is long lived, and their administration should be stopped 1 week preoperatively. If these drugs cannot be stopped, then a bleeding time may be performed to evaluate the extent of platelet impairment. Determination of prothrombin and partial thromboplastin times will not reflect this abnormality.

Patients who have connective tissue disorders may have associated dysphagia and esophageal dysmotility, which can predispose patients to pulmonary aspiration of gastric and esophageal contents. Extensive fibrosis of the temporomandibular or cricoarytenoid joint can complicate airway management and endotracheal intubation. Pulmonary infiltration and fibrosis may complicate intraoperative care by causing hypoxemia. Hematologic abnormalities, including anemia of chronic disease, may complicate management even further. Again, the history should focus on the extent of disease, the type of treatment, and the child's response to therapy. Laboratory assessment may include an ECG and a chest radiograph; electrolytes, blood urea nitrogen, creatinine, hemoglobin, hematocrit, and platelet levels; and evaluation of the peripheral blood smear. A patient who has quiescent disease and who has regular follow-up may need nothing other than hematocrit determination.