[Anesthesiologic considerations in Cornelia de Lange syndrome].

BACKGROUND

Retrospective study in pediatric patients with Cornelia de Lange syndrome undergoing different surgical operations, in order to critically evaluate the anesthesiologic aspects of this syndrome.

METHODS

Between January 1997 and December 1998, 42 patients undergoing diagnostic examinations (esophagogastroscopy, oculistic visit) or surgery (total exodontia, fundoplicatio for esophageal reflux, correction of labiopalatoschisis) have been studied. The anaesthesiologic technique has been similar for all the cases.

RESULTS

Any particular difficulty or inconvenience in comparison with patients with the same range of age, or particular intraoperative difficulties have been observed. In only one patient the McCoy laryngoscope was used due to difficult intubation (Cormack and Lehane III).

CONCLUSIONS

Short neck, ogive palatum, cleft palate, micrognatia, urogenital and gastroenteric anomalies, esophageal reflux, together with heart defects (15% of cases), can complicate the peri and postoperative period in these patients. A careful preoperative evaluation and a careful perioperative management, may decrease anesthetic complications in Cornelia de Lange syndrome to the same range of normal patients.