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Bleeding diathesis in Noonan syndrome.

Joost M Staudt1, Chantal M A M Van der Horst, Marjolijn Peters

  • 1Department of Plastic and Reconstructive Surgery and Hand Surgery, Academical Medical Centre, Amsterdam, The Netherlands. joost@staudt.nl

Scandinavian Journal of Plastic and Reconstructive Surgery and Hand Surgery
|October 7, 2005
PubMed
Summary

Noonan syndrome patients may experience bleeding issues, even after minor surgery. Early coagulation screening is recommended for individuals with Noonan syndrome to detect potential hemostatic defects.

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Area of Science:

  • Medical Genetics
  • Hematology
  • Pediatric Surgery

Background:

  • Noonan syndrome is a genetic disorder affecting multiple body systems.
  • Surgical interventions are sometimes necessary for individuals with Noonan syndrome.
  • Hemostatic defects are not always highlighted in Noonan syndrome literature.

Observation:

  • An 18-year-old female with Noonan syndrome developed subcutaneous hematomas post-otoplasty.
  • Coagulation tests revealed a bleeding diathesis in the patient.
  • This complication suggests an underlying hemostatic issue.

Findings:

  • Approximately one-third of patients with Noonan syndrome exhibit hemostatic abnormalities.
  • Bleeding diathesis can manifest in Noonan syndrome patients, particularly after procedures.

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  • Subcutaneous hematomas indicate impaired blood clotting.

    • Implications:

    • Routine coagulation screening should be considered for all Noonan syndrome patients.
    • Early detection of hemostatic defects can prevent surgical complications.
    • Enhanced awareness of bleeding risks is crucial for managing Noonan syndrome.