Advertisement

Hematological Problems in Pediatric Surgery

  • Ciara O’RaffertyEmail author
  • Owen Patrick Smith
Living reference work entry
  • 210 Downloads

Abstract

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.

Keywords

Sickle cell disease Thalassemia Thrombocytopenia Leukemia Neutropenia Splenectomy Hemophilia Venous thromboembolism Disseminated intravascular coagulation Transfusion 

References

  1. Ai J, Advani A. Current status of antibody therapy in all. Br J Haematol. 2015;168(4):471–80.CrossRefPubMedGoogle Scholar
  2. Arcesi RJ, Hann IM, Smith OP. Paediatric haematology. 3rd ed. Oxford: Blackwell Publishing; 2006.CrossRefGoogle Scholar
  3. Astermark J, Donfield SM, DiMichele DM, Gringeri A, Gilbert SA, Waters J, Berntorp E, et al. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the Feiba Novoseven comparative (Fenoc) study. Blood. 2007;109(2):546–51.CrossRefPubMedGoogle Scholar
  4. Berntorp E, Dolan G, Hay C et al. European retrospective study of real-life haemophilia treatment. Haemophilia. 2016.  10.1111/hae.13111. [Epub ahead of print].
  5. Blood L-JL. In: Oldham KT, Colombani PM, Foglia RP, Skinner MA, editors. Principles of practice of pediatric surgery. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 297–312.Google Scholar
  6. Bolton-Maggs PHB. D Poles et al. on behalf of the Serious Hazards of Transfusion (SHOT) Steering Group. The 2015. Annual SHOT Report (2016). www.shotuk.org/shot-reports/
  7. Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol. 2004;126(4):455–74.CrossRefPubMedGoogle Scholar
  8. Bolton-Maggs PH, Chalmers EA, Collins PW, et al. A review of inherited platelet disorders with guidelines for their management on behalf of the Ukhcdo. Br J Haematol. 2006;135(5):603–33.CrossRefPubMedGoogle Scholar
  9. Evidence-based management of sickle cell disease: expert panel report. 2014. Available at http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines/
  10. Hutchinson RJ. Surgerical implications of hematological disease. In: Grosfeld JL, O’Neill JA, Coran AG, Fonkalsrud EW, editors. Pediatric surgery. Philadelphia: Mosby; 2006. p. 178–93.CrossRefGoogle Scholar
  11. Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. Haemophilia. 2008;14(4):671–84.CrossRefPubMedGoogle Scholar
  12. Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Br J Haematol. 2009;145(1):24–33.CrossRefPubMedGoogle Scholar
  13. McCann S, Foa R, Smith OP, Conneally E. Case-based haematology. Oxford: Blackwell Publishing; 2005.Google Scholar
  14. Newall F, Johnston L, Ignjatovic V, Monagle P. Unfractionated heparin therapy in infants and children. Pediatrics. 2009;123(3):e510–8.CrossRefPubMedGoogle Scholar
  15. Oldenburg J, Albert T. Novel products for haemostasis–current status. Haemophilia. 2014;20(Suppl 4):23–8.CrossRefPubMedGoogle Scholar
  16. Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–86.CrossRefPubMedGoogle Scholar
  17. Puri P, Höllwarth ME. Pediatric surgery: diagnosis and management. Heidelberg: Springer; 2009.CrossRefGoogle Scholar
  18. Raja RA, Schmiegelow K, Frandsen TL. Asparaginase-associated pancreatitis in children. Br J Haematol. 2012;159(1):18–27.CrossRefPubMedGoogle Scholar
  19. Rodeghiero F, Ruggeri M. Itp and international guidelines: what do we know, what do we need? Presse Med. 2014;43(4 Pt 2):e61–7.CrossRefPubMedGoogle Scholar
  20. Sadelain M, Brentjens R, Rivière I, Park J. CD19 CAR Therapy for Acute Lymphoblastic Leukemia. Am Soc Clin Oncol Educ Book. 2015:e360-3. doi:10.14694/EdBook_AM.2015.35.e360Google Scholar
  21. Smith OP, Hann I. Essential paediatric haematology. London: Martin Dunitz Publishers; 2002.CrossRefGoogle Scholar
  22. Standards for the clinical care of children and adults with thalassaemia in the UK; 2008.Google Scholar
  23. Thachil J, Toh HC, Levi M, Watson HG. The withdrawal of activated protein C from the use in patients with severe sepsis and dic [amendment to the Bcsh guideline on disseminated intravascular coagulation]. Br J Haematol. 2012;157(4):493–4.CrossRefPubMedGoogle Scholar
  24. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312(10):1033–48.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2016

Authors and Affiliations

  1. 1.Department of HaematologyOur Lady’s Children’s HospitalCrumlin, Dublin 12Ireland
  2. 2.University College DublinDublin 4Ireland

Personalised recommendations