Abstract
The risks associated with total splenectomy, including overwhelming postsplenectomy infection, have led to an interest in the use of partial splenectomy as an alternative surgical option for children with congenital hemolytic anemias and hypersplenism. Partial splenectomy, a procedure designed to remove enough spleen to improve anemia and avoid complications of splenic sequestration while preserving splenic function, has shown promise in children. Radiologic imaging is essential for the preoperative evaluation and postoperative care for children undergoing partial splenectomy and offers a broad range of critical clinical information essential for care of these complex children. It is imperative for radiologists involved in the care of these children to be familiar with the surgical technique and imaging options for these procedures. This article reviews the surgical technique as well as the current status of various diagnostic imaging options used for children undergoing partial splenectomy, highlighting technical aspects and specific clinical information obtained by each modality.
Similar content being viewed by others
References
Delaunay J (1995) Genetic disorders of the red cell membrane. Crit Rev Oncol Hematol 19:79–110
Tchernia G, Bader-Meunier B, Berterottiere P et al (1997) Effectiveness of partial splenectomy in hereditary spherocytosis. Curr Opin Hematol 4:136–141
King H, Shumacker HB Jr (1952) Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg 136:239–242
Brigden ML, Pattullo AL (1999) Prevention and management of overwhelming postsplenectomy infection—an update. Crit Care Med 27:836–842
Leonard AS, Giebink GS, Baesl TJ et al (1980) The overwhelming postsplenectomy sepsis problem. World J Surg 4:423–432
Lynch AM, Kapila R (1996) Overwhelming postsplenectomy infection. Infect Dis Clin North Am 10:693–707
Tchernia G, Gauthier F, Mielot F et al (1993) Initial assessment of the beneficial effect of partial splenectomy in hereditary spherocytosis. Blood 81:2014–2020
Hoeper MM, Niedermeyer J, Hoffmeyer F et al (1999) Pulmonary hypertension after splenectomy? Ann Intern Med 130:506–509
Kruetzmann S, Rosado MM, Weber H et al (2003) Human immunoglobulin M memory B cells controlling Streptococcus pneumoniae infections are generated in the spleen. J Exp Med 197:939–945
Robinette CD, Fraumeni JF Jr (1977) Splenectomy and subsequent mortality in veterans of the 1939–45 war. Lancet 2:127–129
Morinis J, Dutta S, Blanchette V et al (2008) Laparoscopic partial vs total splenectomy in children with hereditary spherocytosis. J Pediatr Surg 43:1649–1652
Pearson HA (1998) The spleen and disturbances of splenic function. In: Nathan DG, Orkin SH (eds) Natahan and Orki’s hematology of infancy and childhood. Saunders, Philadelphia, pp 1051–1068
Price VE, Dutta S, Blanchette VS et al (2006) 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 46:597–603
Rice HE, Oldham KT, Hillery CA et al (2003) Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg 237:281–288
Stoehr GA, Sobh JN, Luecken J et al (2006) Near-total splenectomy for hereditary spherocytosis: clinical prospects in relation to disease severity. Br J Haematol 132:791–793
Bader-Meunier B, Gauthier F, Archambaud F et al (2001) Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis. Blood 97:399–403
Dutta S, Price VE, Blanchette V et al (2006) A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis. Surg Endosc 20:1719–1724
Freud E, Cohen IJ, Mor C et al (1998) Splenic “regeneration” after partial splenectomy for Gaucher disease: histological features. Blood Cells Mol Dis 24:309–316
Goldthorn JF, Schwartz AD, Swift AJ et al (1978) Protective effect of residual splenic tissue after subtotal splenectomy. J Pediatr Surg 13:587–590
Malangoni MA, Dawes LG, Droege EA et al (1985) Splenic phagocytic function after partial splenectomy and splenic autotransplantation. Arch Surg 120:275–278
Witte MH, Witte CL, Van Wyck DB et al (1983) Preservation of the spleen. Lymphology 16:128–137
Cohen IJ, Katz K, Freud E et al (1992) Long-term follow-up of partial splenectomy in Gaucher’s disease. Am J Surg 164:345–347
Poulin EC, Thibault C (1993) The anatomical basis for laparoscopic splenectomy. Can J Surg 36:484–488
De Odorico I, Spaulding KA, Pretorius DH et al (1999) Normal splenic volumes estimated using three-dimensional ultrasonography. J Ultrasound Med 18:231–236
Cappellini MD, Grespi E, Cassinerio E et al (2005) Coagulation and splenectomy: an overview. Ann N Y Acad Sci 1054:317–324
Mohren M, Markmann I, Dworschak U et al (2004) Thromboembolic complications after splenectomy for hematologic diseases. Am J Hematol 76:143–147
Brink JS, Brown AK, Palmer BA et al (2003) Portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy. J Pediatr Surg 38:644–647
Rattner DW, Ellman L, Warshaw AL (1993) Portal vein thrombosis after elective splenectomy. An underappreciated, potentially lethal syndrome. Arch Surg 128:565–569, discussion 569–570
Chaffanjon PC, Brichon PY, Ranchoup Y et al (1998) Portal vein thrombosis following splenectomy for hematologic disease: prospective study with Doppler color flow imaging. World J Surg 22:1082–1086
Cohen J, Edelman RR, Chopra S (1992) Portal vein thrombosis: a review. Am J Med 92:173–182
Canatan D, Erden I, Aytac S et al (1992) Doppler color flow imaging for the evaluation of postsplenectomy portal vein thrombosis in pediatric hematological diseases. Pediatr Hematol Oncol 9:397–399
Papanicolaou N, Harmatz P, Simeone JF et al (1984) Sonographic demonstration of reversible portal vein thrombosis following splenectomy in an adolescent. J Clin Ultrasound 12:575–577
Skarsgard E, Doski J, Jaksic T et al (1993) Thrombosis of the portal venous system after splenectomy for pediatric hematologic disease. J Pediatr Surg 28:1109–1112
van’t Riet M, Burger JW, van Muiswinkel JM et al (2000) Diagnosis and treatment of portal vein thrombosis following splenectomy. Br J Surg 87:1229–1233
Donnelly LF, Foss JN, Frush DP et al (1999) Heterogeneous splenic enhancement patterns on spiral CT images in children: minimizing misinterpretation. Radiology 210:493–497
Glazer GM, Axel L, Goldberg HI et al (1981) Dynamic CT of the normal spleen. AJR 137:343–346
Partanen K, Soimakallio S, Kivimaki T et al (1984) Dynamic topography of the contrast enhancement of the spleen. Eur J Radiol 4:101–106
Ware RE, Rees RC, Sarnaik SA, Iyer RV, Alvarez OA, Casella JF, Shulkin BL, Shalaby-Rana E, Strife CF, Miller JH, Lane PA, Wang WC, Miller ST, BABY HUG Investigators (2010) Renal function in infants with sickle cell anemia: baseline data from the BABY HUG trial. J Pediatr 156(1):66–70.e1
Thompson BW, Miller ST, Rogers ZR et al (2009) The pediatric hydroxyurea phase III clinical trial (BABY HUG): challenges of study design. Pediatr Blood Cancer 54:250–255
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Hollingsworth, C.L., Rice, H.E. Hereditary spherocytosis and partial splenectomy in children: review of surgical technique and the role of imaging. Pediatr Radiol 40, 1177–1183 (2010). https://doi.org/10.1007/s00247-009-1519-8
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-009-1519-8