Table 1. Demographics, Congenital Anomalies, and Diagnostic Plasma Sterol Levels in a Series of Patients with Smith-Lemli-Optiz Syndrome

* Age at the first anesthetic in this series.

† Severity scores are based on the presence of malformations in each of 10 embryologically distinct areas. 11 

Scores greater than 50 reflect severe disease, 25–50 reflect moderate or typical disease, and less than 25 reflect mild disease.

F, female; M, male; PDA, patent ductus arteriosus; ASD, atrial septal defect; GERD, gastroesophageal reflux disease; Y, yes (showing that the characteristic is present).