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  • Author
    Karen Olaco
  • Co-author

    Fei Zheng-Ward, MD, Elizabeth Tsai, MD, and Aram Messerlian, MD

  • Title

    Anesthetic Considerations in a Pediatric Patient with Patau Syndrome

  • Abstract

    Patau Syndrome, or Trisomy 13, is the third most common autosomal trisomy disorder with an estimated prevalence of 8 to 15 per 100,000 live births.  Over 90% of infants die within the first year of life.  Patau Syndrome is characterized by multiple craniofacial and congenital organ malformations, particularly involving the cardiac and central nervous systems. These anatomic and functional changes present potential challenges to anesthetic management. We present a case of a 3-year-old female with Patau Syndrome who underwent Monitored Anesthesia Care (MAC) for a magnetic resonance imaging (MRI) of the lumbar spine for possible neurosurgical intervention. In addition to her Trisomy 13, her past medical history included Lennox Gastaut Syndrome (a severe form of epilepsy), obstructive sleep apnea, and numerous cardiac, pulmonary and vascular anomalies. The child was nonverbal and unable to follow commands at baseline.  She also had syndromic facies and bilateral lower extremity rigidity.  On the day of the MRI, the child was premedicated with oral midazolam and peripheral intravenous access was obtained.  Propofol was titrated intravenously to the patient’s level of sedation and the procedure was performed uneventfully.  She was later discharged home in stable condition from the post-anesthesia care unit.  Patau Syndrome patients can have a wide range of congenital abnormalities, and in recent years, one-year survival rates have increased.  Therefore, anesthetic management emphasizes diligent preoperative planning and intraoperative vigilance.  Anesthetic goals include seizure prevention, maintenance of hemodynamic stability, and prevention of and monitoring for apnea.  This patient safely underwent MAC anesthesia for radiological imaging.

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