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The anesthetic management of a patient with Cohen syndrome

Li Meng1, Joseph J Quinlan, Erin Sullivan

  • 1Department of Anesthesiology, University of Pittsburgh, Pittsburgh, Pennsylvania.

Anesthesia and Analgesia
|August 31, 2004
PubMed

Insights

Cohen syndrome is a rare genetic disorder with distinct facial and developmental features. Anesthetic management presents challenges due to patient characteristics and potential airway difficulties.

Area of Science:

  • Genetics and Rare Diseases
  • Pediatric Anesthesiology

Background:

  • Cohen syndrome is an autosomal recessive disorder.
  • Characterized by intellectual disability, hypotonia, microcephaly, and distinctive facial features.
  • Rarely reported, with approximately 100 cases documented globally.

Observation:

  • Patients often exhibit uncooperative behavior and facial malformations.
  • These factors can complicate airway management and intubation during general anesthesia.
  • Associated conditions include strabismus, myopia, optic atrophy, blindness, and cardiac defects.

Findings:

  • The study details the anesthetic management of a patient with Cohen syndrome.
  • Highlights the challenges encountered in providing anesthesia for these individuals.
  • Emphasizes the need for careful pre-anesthetic evaluation and planning.

Implications:

  • Informs anesthetic strategies for patients with Cohen syndrome.
  • Contributes to the limited literature on managing this rare genetic disorder.
  • Aids clinicians in anticipating and mitigating potential anesthetic risks.

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