Ellis-van Creveld syndrome is a rare type of developmental
chondroectodermal dysplasia. We report the case of a 32-year-old woman with this syndrome who was scheduled for
cesarean section. She had no related heart defect. A spinal block was attempted but after confirming that no sensory blockade had been achieved,
general anesthesia was administered. Both the operation and the
anesthetic procedure were without complications. The clinical manifestations of
Ellis-van Creveld syndrome are short-limbed
dwarfism, postaxial polydactyly, fingernail dysplasia,
cleft palate and lips, and heart defects. Diagnosis is based on clinical manifestations and radiography. Treatment involves correction of heart defects and orthopedic problems. Perioperative
airway management problems may develop. A preoperative echocardiogram should be done to assess heart function and ascertain anatomical abnormalities. Thoracic
deformities may make
mechanical ventilation difficult and there is risk of
barotrauma. Intraoperative management requires rapid control of the airway and prevention of bronchial aspiration. Vigilance in preventing hemodynamic instability and myocardial depression is essential. Postoperative
analgesia must be managed carefully and adverse cardiorespiratory events avoided.