Pediatric Asplenia Follow-up
- Author: Joseph C Turbyville, MD; Chief Editor: Harumi Jyonouchi, MD more...
Further Inpatient Care
- The most difficult and crucial aspect of asplenia is establishing the diagnosis.
- Although this task is relatively simple in patients with accompanying anomalies, especially complex cyanotic cardiac problems, and in those with a family history of the condition, the patient with isolated asplenia or hyposplenia may not be easily identified.
- The diagnosis is often made at autopsy.
- Patients require regular monitoring with an established provider.
- All immunizations, including routine childhood vaccinations and additional immunizations, are recommended (see Medical Care).
- These vaccinations should be administered at the earliest opportunity.
- Close observation and monitoring is mandatory, especially in the first few years of childhood, to educate the family and to ensure compliance with antibiotic prophylaxis.
Prognosis
- With early diagnosis and aggressive treatment, the long-term prognosis of a child with isolated congenital asplenia is good.
- The risk of overwhelming sepsis, although it does not end, significantly decreases in individuals older than 5 years.
- The primary care physician plays an integral role in the identification and long-term treatment of patients with asplenia.
- Congenital asplenia, polysplenia, and hypoplasia may be underdiagnosed. An increased awareness of their existence may be crucial and life saving in immunocompromised individuals with these conditions.
Patient Education
- See Medical Care.
Fremont RD, Rice TW. Splenosis: a review. South Med J. Jun 2007;100(6):589-93. [Medline].
Leahy RT, Philip RK, Gibbons RJ, et al. Asplenia in ATR-X Syndrome: a second report. Am J Med Genet A. 2005;15;139 (1):37-9. [Medline].
Pollak U, Bar-Sever Z, Hoffer V, Marcus N, Scheuerman O, Garty BZ. Asplenia and functional hyposplenism in autoimmune polyglandular syndrome type 1. Eur J Pediatr. Feb 2009;168(2):233-5. [Medline].
Casey B, Devoto M, Jones KL, Ballabio A. Mapping a gene for familial situs abnormalities to human chromosome Xq24-q27.1. Nat Genet. Dec 1993;5(4):403-7. [Medline].
US Preventive Services Task Force. Screening for sickle cell disease in newborns: recommendation statement. Am Fam Physician. May 1 2008;77(9):1300-2. [Medline].
Committee on Infectious Diseases American Academy of Pediatrics. Red Book: 2006 Report of the Committee on Infectious Diseases. 27th Ed. 2006.
Updated recommendation from the Advisory Committee on Immunization Practices (ACIP) for revaccination of persons at prolonged increased risk for meningococcal disease. MMWR Morb Mortal Wkly Rep. Sep 25 2009;58(37):1042-3. [Medline].
Elsayes KM, Narra VR, Mukundan G, et al. MR imaging of the spleen: spectrum of abnormalities. Radiographics. 2005;Jul-Aug;25(4):967-82. [Medline]. [Full Text].
Garson A, Bricker JT, McNamara DG. The Science and Practice of Pediatric Cardiology. Vol 3. 1990:1288-97.
Gill DG, Kara M, Moore L. Archives of diseases in childhood. Vol 66. 1991:1366.
Gorg C, Eickhorn M, Zugmaier G. The small spleen: sonographic patterns of functional hyposplenia or asplenia. J Clin Ultrasound. 2003;Mar-Apr;31(3):152-5. [Medline].
Harrod VL, Howard TA, Zimmerman SA, Dertinger SD, Ware RE. Quantitative analysis of Howell-Jolly bodies in children with sickle cell disease. Exp Hematol. Feb 2007;35(2):179-83. [Medline].
Katcher AL. Familial asplenia, other malformations, and sudden death. Pediatrics. 1980;65(3):633-635. [Medline].
Kevy SV, Tefft M, Vawier GF, Rosen FS. Hereditary splenic hypoplasia. Pediatrics. Nov 1968;42(5):752-7. [Medline].
Lane PA, O'Connell JL, Lear JL, et al. Functional asplenia in hemoglobin SC disease. Blood. Apr 15 1995;85(8):2238-44. [Medline].
Lin XZ, Chang TM, Tsai HM, et al. Liver, spleen and tumor volume measured by personal computer. Hepatogastroenterology. Mar-Apr 1999;46(26):838-42. [Medline].
Lindor NM, Smithson WA, Ahumada CA, et al. Asplenia in two father-son pairs. Am J Med Genet. Mar 13 1995;56(1):10-1. [Medline].
Long WA. Ivemark syndrome (cardiosplenic or heterotaxy syndrome). In: Fetal and Neonatal Cardiology. 1990:616-19.
Mebius RE, Kraal G. Structure and function of the spleen. Nat Rev Immunol. Aug 2005;5(8):606-16. [Medline].
Melles DC, de Marie S. Prevention of infections in hyposplenic and asplenic patients: an update. Neth J Med. Feb 2004;62(2):45-52. [Medline].
Moller JH, Neal WA. Congenital cardiac anomalies. In: Fetal, Neonatal, and Infant Cardiac Disease. 1990:767-71.
Morgan MS. Prophylaxis should be considered even for trivial animal bites. BMJ. May 10 1997;314(7091):1413. [Medline].
Pearson HA. The spleen and disturbances of splenic function. Hematology. 1993;2:1058-9.
Price VE, Blanchette VS, Ford-Jones EL. The prevention and management of infections in children with asplenia or hyposplenia. Infect Dis Clin North Am. Sep 2007;21(3):697-710, viii-ix. [Medline].
Price VE, Dutta S, Blanchette VS, 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].
Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia. A review of cardiac and non- cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J. Aug 1975;37(8):840-52. [Medline].
Stiehm ER. Immunodeficiency disorders. In: Immunologic Disorders in Infants and Children. 4th ed. 1996:326-327.
Tham KT, Teague MW, Howard CA, Chen SY. A simple splenic reticuloendothelial function test: counting erythrocytes with argyrophilic inclusions. Am J Clin Pathol. May 1996;105(5):548-52. [Medline].
Tribioli C, Lufkin T. The murine Bapx1 homeobox gene plays a critical role in embryonic development of the axial skeleton and spleen. Development. Dec 1999;126(24):5699-711. [Medline].
Waldman JD, Rosenthal A, Smith AL, et al. Sepsis and congenital asplenia. J Pediatr. Apr 1977;90(4):555-9. [Medline].
Wang JK, Hsieh KH. Immunologic study of the asplenia syndrome. Pediatr Infect Dis J. Nov 1991;10(11):819-22. [Medline].

