Cri Du Chat Syndrome (5p− Syndrome)


Risk

  • 1:15,000-50,000 live births

  • Most common human chromosomal syndrome

Perioperative Risks

  • Difficult airway management due to anatomic abnormalities of the airways.

  • 30% can present with congenital heart disease.

  • Aspiration risk.

  • May require temp control.

  • Cardiac events (pts may have cardiac abnormalities).

Worry About

  • Difficult mask ventilation; airway obstruction secondary to hypotonia

  • Difficult intubation

  • Temperature regulation

Overview

  • Microcephaly with profound mental retardation and hypotonia.

  • Characteristic facies with micrognathia, low-set ears, facial asymmetry.

  • Characteristic high-pitched cry may be due to laryngeal abnormalities (narrow diamond-shaped larynx, long floppy epiglottis) or neurogenic defect.

  • CHD: Persistent ductus arteriosus, septal defects, or pulmonic stenosis.

  • Behavioral features include repetition of movements, aggression, self-injury, and hypersensitivity to sound, among others.

  • Repetitive respiratory infections are common.

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