Severe hemophiliacs require recombinant factor replacement perioperatively to secure hemostasis. Many require placement of a central venous access device for delivery of factor replacement throughout the preschool years. Patients with thrombocytopenia, platelet function defects, von Willebrand disease, or acquired coagulopathy may need blood product replacement and special precautions prior to invasive procedures.
Children with sickle cell disease (SCD) may require splenectomy, cholecystectomy, or adenotonsillectomy and are at risk perioperatively of an acute sickling event.
Leukemic patients often require blood products or growth factor support before surgery and frequently in the operating theater throughout their treatment course for lumbar punctures, bone marrow biopsies, and central venous access-related issues.
Children who are splenectomized are rendered susceptible to overwhelming infection by Gram-positive encapsulated organisms. They require vaccination prior to splenectomy and should receive postoperative antibiotic prophylaxis up to the age of 16. Where possible, splenectomy should be deferred until >6 years of age, although this latter recommendation does not apply to children with SCD, who are functionally asplenic from a young age.
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