BACKGROUND AND OBJECTIVES: Mckusick-Kaufman syndrome is an uncommon disease, typically characterized by hydrometrocolpos, polydactyly and congenital heart defects. These patients are often submitted to different surgical procedures throughout their lives and the anesthesiologist must be prepared to deal with possible complications. This article aimed at reporting the anesthetic management adopted for a patient with this syndrome. CASE REPORT: A 11-year-old, 37 kg, female with Mckusick-Kaufman syndrome, chronic renal failure, hypertensive encephalopathy and severe asthma was scheduled for surgical arterial-venous fistula preparation and removal of infected peritoneal dialysis catheter. Previous prolonged tracheal intubation was reported. Anesthesia was induced with alfentanil (1 mg), propofol (50 mg) and atracurium (25 mg) and was maintained with inhalational sevoflurane (2% to 4%) and intermittent IV alfentanil doses. Trachea was intubated without intercurrences and extubation was performed in the operating room after satisfactory neuromuscular function recovery. CONCLUSIONS: Although Mckusick-Kaufman syndrome is a variable association of congenital defects, some standard anesthetic cares may be defined. This specific case presented complicating factors for anesthesia, and induction with propofol and alfentanil and maintenance with sevoflurane have provided the patient a perioperative period with minimal ventilatory and hemodynamic repercussions.
ANESTHESIA; DISEASES