Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Splenomegaly Treatment & Management

  • Author: Alexander Gozman, MD; Chief Editor: Robert J Arceci, MD, PhD  more...
 
Updated: Oct 05, 2015
 

Medical Care

Because splenomegaly is usually the result of a systemic disease, the primary goal is treatment of the underlying process. In certain circumstances, splenectomy may be the therapy of choice for symptoms or complications caused by the enlarged organ. However, because of the risk of overwhelming sepsis in children who are asplenic, the risks and benefits must be carefully weighed when splenectomy is being considered.[11, 12]

  • The new PCV7 conjugated pneumococcal vaccine and the older PPV23 polyvalent pneumococcal vaccines and the Haemophilus influenzae vaccine should be administered to all children who are asplenic and to those about to undergo splenectomy. The polyvalent pneumococcal vaccine is effective only if the patient is aged 2 years or older. Meningococcal vaccine, MCV4, is often administered to these patients in this age group as well. To maximize antibody formation, vaccines should be administered at least 10 days before splenectomy. [26]
  • Daily penicillin is recommended to prevent pneumococcal septicemia in asplenic children younger than 5 years. Antibiotic prophylaxis is often administered for several years after splenectomy in patients older than 5 years, but the role of prophylaxis in these children is less well documented than it is in others.
  • In patients with homozygous sickle cell anemia or sickle beta-zero thalassemia, oral penicillin prophylaxis should be started when the diagnosis is established. This therapy should be administered until at least age 5 years. The role of penicillin prophylaxis in patients with hemoglobin sickle cell disease is controversial. Patients with sickle beta+ thalassemia do not appear to need penicillin prophylaxis.
  • Febrile illnesses in asplenic children should be approached as potentially life-threatening events and evaluated thoroughly, with a low threshold for treatment with intravenous antibiotics that cover Streptococcus pneumoniae and H influenzae. The increasing prevalence of drug-resistant S pneumoniae and the decreasing incidence of H influenzae infections are complicating factors in determining the optimal choice of antibiotics.
Next

Surgical Care

Splenic trauma is the most common indication for splenectomy, although attempts at splenic preservation are increasingly important. Nonsurgical management for splenic trauma has success rates of 52-98%, with failure usually occurring in the first 96 hours. Splenic cysts, tumors, and vascular lesions may also require surgical removal. Whenever possible, splenic tissue is preserved to decrease the risk of septicemia, but total splenectomy is occasionally necessary.[27, 28] For elective surgery, laparascopic splenectomy is preferrable to open splenectomy, except for in patients with massive splenomegaly.[29]  (However, a retrospective study by Hassan and Al Ali on 32 children with massive splenomegaly suggested that laparoscopic splenectomy for this condition is safe and effective, with patients experiencing reductions in pain and blood loss, improved recovery, and a shorter hospital stay; operative time, though, was significantly longer than with open splenectomy.[30] )

Splenectomy can cure hypersplenism but is not usually indicated because the secondary cytopenias rarely cause serious problems. However, in patients with portal hypertension, vascular shunts may be necessary to prevent esophageal variceal bleeding.

Splenectomy may be helpful in improving cytopenias in several medical conditions, including congenital anemias (eg, hereditary spherocytosis, elliptocytosis) and autoimmune disorders (eg, immune thrombocytopenic purpura, autoimmune hemolytic anemia, hypersplenism). In thalassemia major, splenectomy may initially decrease the transfusion requirements caused by hypersplenism. However, the benefit must be carefully weighed against the risk of sepsis.

In Gaucher disease, splenectomy may be necessary when the mechanical strain of the enlarged spleen requires intervention.

Splenectomy may be indicated in children with sickle cell anemia and a history of splenic sequestration crisis in order to prevent recurrences of the crisis.

As part of exploratory laparotomy, splenectomy was once an important component of staging of Hodgkin disease. This procedure is rarely used because of improvements in imaging modalities, the high risk of postsplenectomy sepsis, and the increased use of chemotherapy in patients, which allows treatment decisions to be made on the basis of radiologic evaluation alone. Furthermore, data suggest that splenectomy increases the risk of second malignancy in patients treated for Hodgkin disease.

Li et al described the successful use of suture suspension in the performance of single-incision laparoscopic splenectomy. In the study, which involved nine children with splenomegaly due to hereditary spherocytosis, suture suspension reportedly improved splenic hilum exposure.[31]

Previous
Next

Consultations

A pediatric hematologist and/or oncologist is the usual consultant when the cause of splenomegaly is not obvious or when a primary hematologic or oncologic disorder is suspected.

Previous
Next

Activity

See the list below:

  • According to the American Academy of Pediatrics and the Council of Sports Medicine and Fitness, children with acutely enlarged spleens should avoid contact, collision, or limited-contact sports. [26] Viral-related splenomegaly rarely lasts longer than 2 months.
  • The duration for which contact restriction should persist after acute infectious mononucleosis remains uncertain. In the absence of trauma the incidence of splenic rupture is approximately 1:1000, usually occurring in the first 3 weeks of illness. No evidence-based guidelines are available. More conservative suggestions recommend that children with infectious mononucleosis and resolution of splenomegaly noted on examination have 3 weeks of rest with a 4-week graded return to activity. However, it has also been suggested that contact sports can resume as soon as 4 weeks after onset of illness. [32]
  • Even with large spleens, disorders associated with chronic splenomegaly have reduced splenic friability with minimal risk of rupture. In children with chronically enlarged spleens, decision to restrict activity should be made on a case-by-case basis. [33]
Previous
 
 
Contributor Information and Disclosures
Author

Alexander Gozman, MD Assistant Professor, Department of Pediatrics, Division of Hematology/Oncology, Albany Medical Center

Alexander Gozman, MD is a member of the following medical societies: American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Richard H Sills, MD Professor of Pediatrics, Upstate Medical University

Richard H Sills, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP Professor of Pediatrics, Albany Medical College; Chief, Division of Pediatric Hematology-Oncology, John and Anna Landis Endowed Chair for Pediatric Hematology-Oncology, Medical Director, Melodies Center for Childhood Cancer and Blood Disorders, Albany Medical Center

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD Director, Children’s Center for Cancer and Blood Disorders, Department of Hematology/Oncology, Co-Director of the Ron Matricaria Institute of Molecular Medicine, Phoenix Children’s Hospital; Editor-in-Chief, Pediatric Blood and Cancer; Professor, Department of Child Health, University of Arizona College of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

J Martin Johnston, MD Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, International Society of Paediatric Oncology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Wayne Hioe, MD, and Mundeep K Kainth, DO, to the development and writing of this article.

References
  1. Arkles LB, Gill GD, Molan MP. A palpable spleen is not necessarily enlarged or pathological. Med J Aust. 1986 Jul 7. 145(1):15-7. [Medline].

  2. Brown NF, Marks DJ, Smith PJ, Bloom SL. Splenomegaly. Br J Hosp Med (Lond). 2011 Nov. 72(11):M166-9. [Medline].

  3. Sills RH. Splenic function: physiology and splenic hypofunction. Crit Rev Oncol Hematol. 1987. 7(1):1-36. [Medline].

  4. Mebius RE, Kraal G. Structure and function of the spleen. Nat Rev Immunol. 2005 Aug. 5(8):606-16. [Medline].

  5. McIntyre OR, Ebaugh FG. Palpable spleens in college freshmen. Ann Intern Med. 1967 Feb. 66(2):301-6. [Medline].

  6. Ebaugh FG, McIntyre OR. Palpable spleens: ten-year follow-up. Ann Intern Med. 1979 Jan. 90(1):130-1. [Medline].

  7. Ancliff P, Hann I. Splenomegaly. Sills RH, ed. Practical Algorithms in Pediatric Hematology and Oncology. Basel, Switzerland: Karger; 2003. 50-1.

  8. Genton B, al-Yaman F, Beck HP, et al. The epidemiology of malaria in the Wosera area, East Sepik Province, Papua New Guinea, in preparation for vaccine trials. I. Malariometric indices and immunity. Ann Trop Med Parasitol. 1995 Aug. 89(4):359-76. [Medline].

  9. Pitney WR. The tropical splenomegaly syndrome. Trans R Soc Trop Med Hyg. 1968. 62(5):717-28. [Medline].

  10. Farley DR, Zietlow SP, Bannon MP, Farnell MB. Spontaneous rupture of the spleen due to infectious mononucleosis. Mayo Clin Proc. 1992 Sep. 67(9):846-53. [Medline].

  11. Castagnola E, Fioredda F. Prevention of life-threatening infections due to encapsulated bacteria in children with hyposplenia or asplenia: a brief review of current recommendations for practical purposes. Eur J Haematol. 2003 Nov. 71(5):319-26. [Medline].

  12. Price VE, Dutta S, Blanchette VS, Butchart S, Kirby M, Langer JC, et al. The prevention and treatment of bacterial infections in children with asplenia or hyposplenia: practice considerations at the Hospital for Sick Children, Toronto. Pediatr Blood Cancer. 2006 May 1. 46(5):597-603. [Medline].

  13. Wilson DB. Acquired platelet defects. Nathan DG, Orkin SH, Ginsburg D, Look AT. Nathan and Oski's hematology of infancy and childhood. 6th ed. Philadelphia, PA: WB Saunders; 2003. Vol 2: 1599.

  14. Peck-Radosavljevic M. Hypersplenism. Eur J Gastroenterol Hepatol. 2001 Apr. 13(4):317-23. [Medline].

  15. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol. 2002 May. 17(5):526-34. [Medline].

  16. Tunnessen WW Jr. Splenomegaly. Roberts K, Tunnessen W, eds. Signs and Symptoms in Pediatrics. 3rd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 1999. 475-83.

  17. Baris HN, Cohen IJ, Mistry PK. Gaucher disease: the metabolic defect, pathophysiology, phenotypes and natural history. Pediatr Endocrinol Rev. 2014 Sep. 12 Suppl 1:72-81. [Medline].

  18. Nixon RK Jr. The detection of splenomegaly by percussion. N Engl J Med. 1954 Jan 28. 250(4):166-7. [Medline].

  19. Castell DO. The spleen percussion sign. A useful diagnostic technique. Ann Intern Med. 1967 Dec. 67(6):1265-7. [Medline].

  20. Grover SA, Barkun AN, Sackett DL. The rational clinical examination. Does this patient have splenomegaly?. JAMA. 1993 Nov 10. 270(18):2218-21. [Medline].

  21. Pochedly C, Sills RH, Schwartz AD, eds. Disorders of the Spleen: Pathophysiology and Management. New York, NY: Marcel Dekker; 1989.

  22. Kinney TR, Ware RE, Schultz WH, Filston HC. Long-term management of splenic sequestration in children with sickle cell disease. J Pediatr. 1990 Aug. 117(2 Pt 1):194-9. [Medline].

  23. Robertson F, Leander P, Ekberg O. Radiology of the spleen. Eur Radiol. 2001. 11(1):80-95. [Medline].

  24. Schlesinger AE, Hildebolt CF, Siegel MJ, Pilgrim TK. Splenic volume in children: simplified estimation at CT. Radiology. 1994 Nov. 193(2):578-80. [Medline].

  25. Ginzel AW, Kransdorf MJ, Peterson JJ, Garner HW, Murphey MD. Mass-like extramedullary hematopoiesis: imaging features. Skeletal Radiol. 2011 Nov 20. [Medline].

  26. AAP. Immunocompromised children. Pickering LK, ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th ed. Elk Grove, IL: American Academy of Pediatrics; 2003. 69-81.

  27. Lane PA. The spleen in children. Curr Opin Pediatr. 1995 Feb. 7(1):36-41. [Medline].

  28. Rice HE, Oldham KT, Hillery CA, Skinner MA, O'Hara SM, Ware RE. Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg. 2003 Feb. 237(2):281-8. [Medline].

  29. Ahad S, Gonczy C, Advani V, Markwell S, Hassan I. True benefit or selection bias: an analysis of laparoscopic versus open splenectomy from the ACS-NSQIP. Surg Endosc. 2013 Jan 26. [Medline].

  30. Hassan ME, Al Ali K. Massive splenomegaly in children: laparoscopic versus open splenectomy. JSLS. 2014 Jul-Sep. 18 (3):[Medline].

  31. Li S, Li M, Xu W, Sun C, Liu L. Single-Incision Laparoscopic Splenectomy Using the Suture Suspension Technique for Splenomegaly in Children with Hereditary Spherocytosis. J Laparoendosc Adv Surg Tech A. 2015 Sep. 25 (9):770-4. [Medline].

  32. Eichner ER. Sports medicine pearls and pitfalls--defending the spleen: return to play after infectious mononucleosis. Curr Sports Med Rep. 2007 Apr. 6(2):68-9. [Medline].

  33. Rice SG; American Academy of Pediatrics Council on Sports Medicine and Fitness. Medical conditions affecting sports participation. Pediatrics. 2008 Apr. 121(4):841-8. [Medline].

  34. Goddard SL, Chesney AE, Reis MD, et al. Pathological splenic rupture: a rare complication of chronic myelomonocytic leukemia. Am J Hematol. 2007 May. 82(5):405-8. [Medline].

  35. Jandl JH, Aster RH. Increased splenic pooling and the pathogenesis of hypersplenism. Am J Med Sci. 1967 Apr. 253(4):383-98. [Medline].

  36. Goodman J, Newman MI, Chapman WC. Disorders of the spleen. Greer JP, Foerster J, Lukens J, et al, eds. Wintrobe's Clinical Hematology. 11th ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 2004. 1893-909.

  37. Shurin SB. Splenomegaly. Kliegman R, Nieder M, Super D, et al, eds. Practical Strategies in Pediatric Diagnosis and Therapy. Philadelphia, PA: WB Saunders; 1996. 352-9.

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.