[General anesthesia for a boy with Wolf-Hirschhorn syndrome]

Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal abnormality in which there is deletion of the short arm of chromosome no. 4. Features of the condition include severe psychomotor retardation, characteristic facies and various congenital midline fusion anomalies. We report the anesthetic management in a 6-year-old boy with WHS, scheduled for renal biopsy under general anesthesia. Anesthesia was induced and maintained with sevoflurane and nitrous oxide in oxygen. Mask ventilation was performed easily. After establishment of mask ventilation, laryngeal mask airway (LMA) was inserted smoothly without muscle relaxant and an adequate airway was established with a LMA. He has episodes of transient deterioration in renal function with physical stress. To decrease renal effects of perioperative stress, transversus abdominis plane (TAP) block and intravenous patient controlled analgesia (IV-PCA) were administered for postoperative analgesia. The operation ended without any complications. Anesthetic emergence was rapid and he had no pain and decline in renal function.