[Anesthesia and fibrobronchoscopy for the study of chronic stridor in a boy with Robinow syndrome]

Robinow's syndrome involves fetal facial features, short stature, brachymelia, hypoplastic genitals and a normal karyotype. A 10-year-old boy with Robinow's syndrome was scheduled for study of chronic stridor by fiberoptic bronchoscopy. Airway exploration with the patient awake revealed hipertelorism, retromicrognathia, poor dental alignment, macroglossia and class IV Mallampati. After anesthetic induction in spontaneous ventilation with 5% sevoflurane, grade IV Cormack-Lehane conditions were observed. A laryngeal mask was placed without muscle relaxation after which the boy was ventilated manually for several minutes without stridor. Anesthetic maintenance was with 3% sevoflurane in 50% oxygen and air. After recovery of spontaneous ventilation, marked stridor presented along with a decrease in oxygen saturation and expired fractions of sevoflurane and CO2. Pulse oxymetry did not increase with increased FiO2. However, when continuous positive airway pressure (CPAP) was set al 10 cm H2O, pulse oxymetry did increase; likewise, expired and inspired sevoflurane concentrations became equal, and expired CO2 increased. Fiberoptic bronchoscopy revealed an area of intrathoracic tracheomalacia, which collapsed partially during spontaneous expiration and collapsed less when CPAP was started. We discuss the relation between the facial dysmorphia characteristic of this syndrome and the possibility of finding a difficult airway, as well as the diagnosis and treatment of intrathoracic tracheomalacia during anesthesia.