Further Outpatient Care

For patients who require follow-up, family education should be provided at each outpatient visit. Tailor follow-up and education to the underlying disease and the potential side effects of therapy.

Complications

The development of hypersplenism with resulting anemia, leukopenia, and/or thrombocytopenia may be mild or severe and depends on the underlying condition and disease course.

Splenic rupture may occur in acute splenomegaly associated with infectious mononucleosis. The incidence is 1:1000, and it usually occurs in the first 3 weeks of illness.^[33]Therefore, the American Academy of Pediatrics, through its Council on Sports Medicine and Fitness, has recommended that children with acute splenomegaly restrict their participation in sports.^{[34, 35]}Disorders associated with chronic splenomegaly do not have the same splenic friability and risk of rupture.^{[35, 36]}

Splenectomy is uncommonly performed in children with splenomegaly. Nevertheless, should it be clinically indicated, the overall risk of postsplenectomy sepsis is approximately 2%, with increased incidence and mortality in young children.^{[59, 60]}

Ezeofor SN, Obikili EN, Anyanwu GE, Onuh AC, Mgbor SO. Sonographic assessment of the normal limits of the spleen in healthy school children in South-East Nigeria. Niger J Clin Pract. 2014 Jul-Aug. 17 (4):484-8. [QxMD MEDLINE Link]. [Full Text].
Suttorp M, Classen CF. Splenomegaly in Children and Adolescents. Front Pediatr. 2021. 9:704635. [QxMD MEDLINE Link]. [Full Text].
Kahramaner Z, Erdemir A, Arik B, Bilgili G, Tekin M, Genc Y. Reference ranges of liver and spleen dimensions in term infants: sonographic measurements. J Med Ultrason (2001). 2015 Jan. 42 (1):77-81. [QxMD MEDLINE Link].
Pelizzo G, Guazzotti M, Klersy C, et al. Spleen size evaluation in children: Time to define splenomegaly for pediatric surgeons and pediatricians. PLoS One. 2018. 13 (8):e0202741. [QxMD MEDLINE Link]. [Full Text].
Snape J, Baker AR, Rees Y. Pseudo-splenomegaly as a result of subphrenic abscess. Postgrad Med J. 1986 Jan. 62 (723):29-30. [QxMD MEDLINE Link]. [Full Text].
Brown NF, Marks DJ, Smith PJ, Bloom SL. Splenomegaly. Br J Hosp Med (Lond). 2011 Nov. 72(11):M166-9. [QxMD MEDLINE Link].
Schlesinger AE, Hildebolt CF, Siegel MJ, Pilgrim TK. Splenic volume in children: simplified estimation at CT. Radiology. 1994 Nov. 193(2):578-80. [QxMD MEDLINE Link].
Ginzel AW, Kransdorf MJ, Peterson JJ, Garner HW, Murphey MD. Mass-like extramedullary hematopoiesis: imaging features. Skeletal Radiol. 2011 Nov 20. [QxMD MEDLINE Link].
Anand K, Munker R. Is a liver-spleen scan helpful in the evaluation of patients with thrombocytopenia?. Ann Hematol. 2015 Nov. 94 (11):1921-2. [QxMD MEDLINE Link].
Larsson F, Ahlin A, Marshall Heyman M, Abrahamsson J. [Acute splenic sequestration in children with sickle cell disease--an overview]. Lakartidningen. 2016 Dec 20. 113:[QxMD MEDLINE Link].
Ghmaird A, Alnoaiji MM, Al-Blewi S, Zaki S, El-Lewi A, Ahmad N. Splenectomy in Patients with Sickle Cell Disease in Tabuk. Open Access Maced J Med Sci. 2016 Mar 15. 4 (1):107-11. [QxMD MEDLINE Link]. [Full Text].
Owusu-Ofori S, Remmington T. Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev. 2017 Nov 7. 11:CD003425. [QxMD MEDLINE Link]. [Full Text].
Costi R, Castro Ruiz C, Romboli A, Wind P, Violi V, Zarzavadjian Le Bian A. Partial splenectomy: Who, when and how. A systematic review of the 2130 published cases. J Pediatr Surg. 2019 Aug. 54 (8):1527-38. [QxMD MEDLINE Link].
Gutierrez Diaz AI, Svarch E, Arencibia Nunez A, et al. [Partial splenectomy in sickle cell disease]. An Pediatr (Barc). 2015 Apr. 82 (4):228-34. [QxMD MEDLINE Link].
Lopez JM Jr, McGonagill PW, Gross JL, et al. Subcapsular hematoma in blunt splenic injury: A significant predictor of failure of nonoperative management. J Trauma Acute Care Surg. 2015 Dec. 79 (6):957-9; discussion 959-60. [QxMD MEDLINE Link].
Pugi J, Carcao M, Drury LJ, Langer JC. Results after laparoscopic partial splenectomy for children with hereditary spherocytosis: Are outcomes influenced by genetic mutation?. J Pediatr Surg. 2018 May. 53 (5):973-5. [QxMD MEDLINE Link].
Ates U, Tastekin NY, Gollu G, Ergun E, Yagmurlu A. Laparoscopic splenectomy in pediatric age: long-term follow-up. Arch Argent Pediatr. 2017 Dec 1. 115 (6):e385-e390. [QxMD MEDLINE Link]. [Full Text].
Shan GD, Chen WG, Hu FL, et al. A spontaneous hematoma arising within an intrapancreatic accessory spleen: A case report and literature review. Medicine (Baltimore). 2017 Oct. 96 (41):e8092. [QxMD MEDLINE Link]. [Full Text].
Weledji EP. Benefits and risks of splenectomy. Int J Surg. 2014. 12 (2):113-9. [QxMD MEDLINE Link]. [Full Text].
Rorholt M, Ghanima W, Farkas DK, Norgaard M. Risk of cardiovascular events and pulmonary hypertension following splenectomy - a Danish population-based cohort study from 1996-2012. Haematologica. 2017 Aug. 102 (8):1333-41. [QxMD MEDLINE Link]. [Full Text].
Luu S, Spelman D, Woolley IJ. Post-splenectomy sepsis: preventative strategies, challenges, and solutions. Infect Drug Resist. 2019. 12:2839-51. [QxMD MEDLINE Link]. [Full Text].
Okabayashi T, Hanazaki K. Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease. World J Gastroenterol. 2008 Jan 14. 14 (2):176-9. [QxMD MEDLINE Link]. [Full Text].
Iolascon A, Andolfo I, Barcellini W, et al. Recommendations regarding splenectomy in hereditary hemolytic anemias. Haematologica. 2017 Aug. 102 (8):1304-13. [QxMD MEDLINE Link]. [Full Text].
Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD. Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol. 1991 Jul. 157 (1):119-21. [QxMD MEDLINE Link].
Sills RH. Splenic function: physiology and splenic hypofunction. Crit Rev Oncol Hematol. 1987. 7(1):1-36. [QxMD MEDLINE Link].
Pizzi M, Fuligni F, Santoro L, et al. Spleen histology in children with sickle cell disease and hereditary spherocytosis: hints on the disease pathophysiology. Hum Pathol. 2017 Feb. 60:95-103. [QxMD MEDLINE Link].
Mebius RE, Kraal G. Structure and function of the spleen. Nat Rev Immunol. 2005 Aug. 5(8):606-16. [QxMD MEDLINE Link].
McIntyre OR, Ebaugh FG. Palpable spleens in college freshmen. Ann Intern Med. 1967 Feb. 66(2):301-6. [QxMD MEDLINE Link].
Ebaugh FG, McIntyre OR. Palpable spleens: ten-year follow-up. Ann Intern Med. 1979 Jan. 90(1):130-1. [QxMD MEDLINE Link].
Ancliff P, Hann I. Splenomegaly. Sills RH, ed. Practical Algorithms in Pediatric Hematology and Oncology. Basel, Switzerland: Karger; 2003. 50-1.
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. [QxMD MEDLINE Link].
Pitney WR. The tropical splenomegaly syndrome. Trans R Soc Trop Med Hyg. 1968. 62(5):717-28. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
AAP. Kimberlin DW, ed. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove, IL: American Academy of Pediatrics; 2015.
Rice SG; American Academy of Pediatrics Council on Sports Medicine and Fitness. Medical conditions affecting sports participation. Pediatrics. 2008 Apr. 121(4):841-8. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Amaki J, Sekiguchi T, Hiraiwa S, et al. Three cases of spontaneous splenic rupture in malignant lymphoma. Int J Hematol. 2018 Dec. 108 (6):647-51. [QxMD MEDLINE Link].
Dumic I, Patel J, Hart M, Niendorf ER, Martin S, Ramanan P. Splenic Rupture as the First Manifestation of Babesia Microti Infection: Report of a Case and Review of Literature. Am J Case Rep. 2018 Mar 23. 19:335-41. [QxMD MEDLINE Link]. [Full Text].
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.
Peck-Radosavljevic M. Hypersplenism. Eur J Gastroenterol Hepatol. 2001 Apr. 13(4):317-23. [QxMD MEDLINE Link].
Jandl JH, Aster RH. Increased splenic pooling and the pathogenesis of hypersplenism. Am J Med Sci. 1967 Apr. 253(4):383-98. [QxMD MEDLINE Link].
Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol. 2002 May. 17(5):526-34. [QxMD MEDLINE Link].
Beutler E, Gelbart T. Estimating the prevalence of pyruvate kinase deficiency from the gene frequency in the general white population. Blood. 2000 Jun 1. 95 (11):3585-8. [QxMD MEDLINE Link].
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.
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. [QxMD MEDLINE Link].
Nixon RK Jr. The detection of splenomegaly by percussion. N Engl J Med. 1954 Jan 28. 250(4):166-7. [QxMD MEDLINE Link].
Castell DO. The spleen percussion sign. A useful diagnostic technique. Ann Intern Med. 1967 Dec. 67(6):1265-7. [QxMD MEDLINE Link].
Grover SA, Barkun AN, Sackett DL. The rational clinical examination. Does this patient have splenomegaly?. JAMA. 1993 Nov 10. 270(18):2218-21. [QxMD MEDLINE Link].
Fenando A, Tatineni S, Raziq FI, Alratroot A. Subcapsular haematoma of the spleen complicating acute pancreatitis. BMJ Case Rep. 2019 Sep 11. 12 (9):[QxMD MEDLINE Link]. [Full Text].
Stylianos S. To save a child's spleen: 50 years from Toronto to ATOMAC. J Pediatr Surg. 2019 Jan. 54 (1):9-15. [QxMD MEDLINE Link].
Ingle SB, Hinge Ingle CR, Patrike S. Epithelial cysts of the spleen: a minireview. World J Gastroenterol. 2014 Oct 14. 20 (38):13899-903. [QxMD MEDLINE Link]. [Full Text].
Pochedly C, Sills RH, Schwartz AD, eds. Disorders of the Spleen: Pathophysiology and Management. New York, NY: Marcel Dekker; 1989.
Clarke RT, Van den Bruel A, Bankhead C, Mitchell CD, Phillips B, Thompson MJ. Clinical presentation of childhood leukaemia: a systematic review and meta-analysis. Arch Dis Child. 2016 Oct. 101 (10):894-901. [QxMD MEDLINE Link].
Di Giorgio A, De Angelis P, Cheli M, et al. Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: a multicentre national study. Dig Liver Dis. 2019 Mar 6. [QxMD MEDLINE Link].
Yang AC, Bier L, Overbey JR, et al. Early manifestations of type 1 Gaucher disease in presymptomatic children diagnosed after parental carrier screening. Genet Med. 2017 Jun. 19 (6):652-8. [QxMD MEDLINE Link].
McGovern MM, Wasserstein MP, Bembi B, et al. Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation. Orphanet J Rare Dis. 2021 May 10. 16 (1):212. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD. Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol. 1991 Jul. 157 (1):119-21. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Altered immunocompetence - general best practice guidelines for immunization: best practices guidance of the Advisory Committee on Immunization Practices (ACIP). Vaccine recommendations and guidelines of the ACIP. Available at https://www.cdc.gov/vaccines/hcp/acip-recs/general-recs/immunocompetence.html. Reviewed Aug 20, 2019; Accessed: Dec 31, 2019.
Lane PA. The spleen in children. Curr Opin Pediatr. 1995 Feb. 7(1):36-41. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Rosman CWK, Broens PMA, Trzpis M, Tamminga RYJ. A long-term follow-up study of subtotal splenectomy in children with hereditary spherocytosis. Pediatr Blood Cancer. 2017 Apr 18. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Hassan ME, Al Ali K. Massive splenomegaly in children: laparoscopic versus open splenectomy. JSLS. 2014 Jul-Sep. 18 (3):[QxMD MEDLINE Link].
Utria AF, Goffredo P, Keck K, Shelton JS, Shilyansky J, Hassan I. Laparoscopic Splenectomy: Has It Become the Standard Surgical Approach in Pediatric Patients?. J Surg Res. 2019 Aug. 240:109-114. [QxMD MEDLINE Link].
Van Der Veken E, Laureys M, Rodesch G, Steyaert H. Perioperative spleen embolization as a useful tool in laparoscopic splenectomy for simple and massive splenomegaly in children: a prospective study. Surg Endosc. 2016 Nov. 30 (11):4962-7. [QxMD MEDLINE Link].
Chapman J, Azevedo AM. Splenomegaly. StatPearls. 2019 Jan. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Sul HJ, Kang Dy. Congenital neuroblastoma with multiple metastases: a case report. J Korean Med Sci. 2003 Aug. 18 (4):618-20. [QxMD MEDLINE Link]. [Full Text].
Tamayo SG, Rickman LS, Mathews WC, et al. Examiner dependence on physical diagnostic tests for the detection of splenomegaly: a prospective study with multiple observers. J Gen Intern Med. 1993 Feb. 8 (2):69-75. [QxMD MEDLINE Link].

Media Gallery

of 0

Author

Trisha Simone Natanya Tavares, MD Contract Clinical Hematologist and Oncologist, Department of Pediatric Hematology and Oncology, Presbyterian Healthcare; Volunteer Hematology/Oncology Physician, St Vincent de Paul Medical Center

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

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) Professor Emeritus of Pediatrics, Albany Medical College; Chief of Pediatric Oncology, Homi Bhabha Cancer Hospital, Varanasi, India

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) is a member of the following medical societies: Children's Oncology Group, International Society of Pediatric 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.

Richard H Sills, MD Professor of Pediatrics, State University of New York 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.

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 Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group

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.

Sections Pediatric Splenomegaly
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
References

Pediatric Splenomegaly Follow-up

Further Outpatient Care

Complications

References

Tables

Contributor Information and Disclosures

What would you like to print?