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Laparoscopic splenectomy in patients with hereditary spherocytosis: report on 12 consecutive cases

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Abstract

Hereditary spherocytosis is an inherited hemolytic anemia caused by a deficiency in erythrocyte membrane proteins. Removal of the spleen may reduce the intra-splenic hemolytic process of the disease and, therefore, may correct the anemia. Furthermore, it seems to decrease the levels of serum bilirubin, thus reducing the formation of gallbladder stones. Indications and timing of splenectomy, however, are still debated. Twelve patients with severe hereditary spherocytosis operated on with laparoscopic splenectomy were retrospectively reviewed. Median age at diagnosis was 13.8 years (range 8–25 years). Male to female ratio was 5/7. Indications for laparoscopic removal of the spleen included anemia unresponsive to iron supplementation in eight patients (66.6 %) with increase need for red cells transfusions, and jaundice with symptoms related to cholelitiasis in four patients (33.3 %). Laparoscopic splenectomy was associated in four cases to laparoscopic cholecystectomy. Mean operative time was 50 min (range 40–75 min) with no conversion to open surgery. Mean hospital stay ranged from 3 to 7 days. In a 16-month follow-up, no complications were recorded and a persistent correction of anemia was observed. With the advent of laparoscopic surgery, splenectomy has been performed by this mini-invasive approach in referral centers. Laparoscopic splenectomy is an effective technique, when performed in patients with hereditary spherocytosis. Low complication rate and persistent correction of the hematologic disorders can be expected after the laparoscopic splenectomy, provided that a proper technique is performed and an experienced surgical team is available.

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References

  1. Schilling RF (2009) Risks and benefits of splenectomy versus no splenectomy for hereditary spherocytosis—a personal view. Br J Haematol 145:728–732

    Article  PubMed  Google Scholar 

  2. Vecchio R, Cacciola E, Cacciola RR, Marchese S, Intagliata E (2011) Portal vein thrombosis after laparoscopic and open splenectomy. J Laparoendosc Adv Surg Tech A 21(1):71–75. doi:10.1089/lap.2010.0325 (Epub 2010 Dec 29)

    Article  PubMed  Google Scholar 

  3. Vecchio R, Marchese S, Intagliata E, Swehli E, Ferla F, Cacciola E (2013) Long-term results after splenectomy in adult idiopathic thrombocytopenic purpura: comparison between open and laparoscopic procedures. J Laparoendosc Adv Surg Tech A 23(3):192–198. doi:10.1089/lap.2012.0146 (Epub 2012 Dec 11)

    Article  PubMed  Google Scholar 

  4. Glasgow RE, Yee LF, Mulvihill SJ (1997) Laparoscopic splenectomy. The emerging standard. Surg Endosc 11:108–112

    Article  PubMed  CAS  Google Scholar 

  5. Rescorla FJ, Engum SA, West KW, 3rd Tres Scherer LR, Rouse TM, Grosfeld JL (2002) Laparoscopic splenectomy has become the gold standard in children. Am Surg 68:297–301 (discussion 301–302)

    PubMed  Google Scholar 

  6. Bolton-Maggs PHB, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ, On behalf of the General Haematology Task Force of the British Committee for Standards in Haematology (2004) Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 126:455–474

    Article  PubMed  CAS  Google Scholar 

  7. Bolton-Maggs PHB, Langer JC, Iolascon A, Tittensor P, King MJ (2011) Guidelines for the diagnosis and management of hereditary spherocytosis—2011 update. Br J Haematol 156:37–49

    Article  PubMed  Google Scholar 

  8. Oliveira MC, Fernandes RA, Rodrigues CL, Ribeiro DA, Giovanardi MF, Viana MB (2012) Clinical course of 63 children with hereditary spherocytosis: a retrospective study. Rev Bras Hematol Hemoter 34(1):9–13

    Article  PubMed  Google Scholar 

  9. Vecchio R, Marchese S, Swehli E, Intagliata E (2011) Splenic hilum management during laparoscopic splenectomy. J Laparoendosc Adv Surg Tech A 21(8):717–720. doi:10.1089/lap.2011.0165 (Epub 2011 Jul 21)

    Article  PubMed  Google Scholar 

  10. Vecchio R, MacFadyen BV (2002) Laparoscopic common bile duct exploration. Langenbecks Arch Surg 387(1):45–54. doi:10.1007/s00423-002-0289-7 (Epub 2002 Apr 10, Review, PMID:11981684)

    Article  PubMed  Google Scholar 

  11. Perrotta S, Gallagher PG, Mohandas N (2008) Hereditary spherocytosis. Lancet 372(9647):1411–1426

    Article  PubMed  CAS  Google Scholar 

  12. Bolton-Maggs PH (2004) Hereditary spherocytosis; new guidelines. Arch Dis Child 89(9):809–812

    Article  PubMed  CAS  Google Scholar 

  13. Guitton C, Garcon L, Cynober T, Gauthier F, Tchernia G, Delaunay J, Leblanc T, Thuret I, Bader-Meunier B (2008) Hereditary spherocytosis: Guidelines for the diagnosis and management in children Archives de Pe′diatrie 15:1464–1473

    CAS  Google Scholar 

  14. King M-J, Smythe J, Mushens R (2004) Eosin-5-maleimide binding to band 3 and Rh-related proteins forms the basis of a screening test for hereditary spherocytosis. Br J Haematol 124:106–113

    Article  PubMed  CAS  Google Scholar 

  15. Morinis J, Dutta S, Blanchette V, Butchart S, Langer JC (2008) Laparoscopic partial vs total splenectomy in children with hereditary spherocytosis. J Pediatr Surg 43(9):1649–1652

    Article  PubMed  Google Scholar 

  16. Hollingsworth CL, Rice HE (2010) Hereditary spherocytosis and partial splenectomy in children: review of surgical technique and the role of imaging. Pediatr Radiol 40:1177–1183

    Article  PubMed  Google Scholar 

  17. Buesing KL, Tracy ET, Kiernan C, Pastor AC, Cassidy LD, Scott JP, Waree RE, Davidoff AM, Rescorla FJ, Langer JC, Rice HE, Oldham KT (2011) Partial splenectomy for hereditary spherocytosis: a multi-institutional review. J Pediatr Surg 46:178–183

    Article  PubMed  Google Scholar 

  18. IPEG Guidelines Committe (2005) IPEG guidelines for surgical treatment of pediatric spleen diseases. J Laparoendosc Adv Surg Tech 15(1):92–94

    Google Scholar 

  19. Tracy ET, Rice HE (2008) Partial splenectomy for hereditary spherocytosis. Pediatr Clin North Am 55(2):503–519

    Article  PubMed  Google Scholar 

  20. Casale M, Perrotta S (2011) Splenectomy for hereditary spherocytosis: complete, partial or not at all? Expert Rev Hematol 4(6):627–635

    Article  PubMed  Google Scholar 

  21. Alizai NK, Richards EM, Stringer MD (2010) Is cholecystectomy really an indication for concomitant splenectomy in mild hereditary spherocytosis? Arch Dis Child 95(8):596–599

    Article  PubMed  Google Scholar 

  22. Vecchio R, Intagliata E, Marchese S, Spataro L, Ferla F, La Corte F, Cacciola RR, Cacciola E (2013) Laparoscopic splenectomy associated with cholecystectomy: report on 12 cases. JSLS 17 (in press)

  23. Hafsia R, Zriba S, Gouider E, Ben Salah N, Borji W, Zaouche A (2009) Splenectomy in hereditary hemolytic anemia: 82 tunisian cases. Tunis Med 87(5):323–327

    PubMed  Google Scholar 

  24. Delaitre B, Maignien B, Icard P (1992) Laparoscopic splenectomy. Br J Surg 79(12):1334

    Article  PubMed  CAS  Google Scholar 

  25. Davenport M (2003) Laparoscopic surgery in children. Ann R Coll Surg Engl 85:324–330

    Article  PubMed  Google Scholar 

  26. Vecchio R, Cacciola E, Martino M, Cacciola RR, MacFadyen BV (2003) Modifications of coagulation and fibrinolytic parameters in laparoscopic cholecystectomy. Surg Endosc 17(3):428–433. doi:10.1007/s00464-001-8291-7 (Epub 2002 Dec 4, PMID:12457211)

    Article  PubMed  CAS  Google Scholar 

  27. Nobili C, Romano F, Ciravegna AL, Garancini M, Degrate L, Uggeri F, Uggeri F (2011) Consecutive concomitant laparoscopic splenectomy and cholecystectomy: an Italian experience of 30 patients and proposition of a technique. J Laparoendosc Adv Surg Tech 21(4):313–317 (Epub 2011 Mar 2)

    Article  Google Scholar 

  28. Barbaros U, Dinççağ A, Sümer A, Vecchio R, Rusello D, Randazzo V, Issever H, Avci C (2010) Prospective randomized comparison of clinical results between hand-assisted laparoscopic and open splenectomies. Surg Endosc 24(1):25–32. doi:10.1007/s00464-009-0528-x (Epub 2009 Jun 24, PMID:19551441)

    Article  PubMed  Google Scholar 

  29. Dapri G, Bron D, Himpens J, Casali L, Carnevali P, Koustas P, Cadière GB (2011) Single-access transumbilical laparoscopic splenectomy using curved reusable instruments. Surg Endosc 25(10):3419–3422. doi:10.1007/s00464-011-1678-1 (Epub 2011 Apr 22)

    Article  PubMed  Google Scholar 

  30. Rodeghiero F, Ruggeri M (2012) Short- and long-term risks of splenectomy for benign haematological disorders: should we revisit the indications? Br J Haematol 158(1):16–29. doi:10.1111/j.1365-2141.2012.09146.x (Epub 2012 May 10)

    Article  PubMed  Google Scholar 

  31. Abdullah F, Zhang Y, Camp M, Rossberg MI, Bathurst MA, Colombani PM, Casella JF, Nabaweesi R, Chang DC (2009) Splenectomy in hereditary spherocytosis: review of 1,657 patients and application of the pediatric quality indicators. Pediatr Blood Cancer 52:834–837

    Article  PubMed  Google Scholar 

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Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Surgery, University of Catania, Catania, Italy

    R. Vecchio, Eva Intagliata, F. Ferla & S. Marchese

  2. Hematologic Unit, Department of Biomedical Science, University of Catania, Catania, Italy

    R. R. Cacciola & E. Cacciola

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  1. R. Vecchio
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  2. Eva Intagliata
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  3. F. Ferla
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  4. S. Marchese
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  5. R. R. Cacciola
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  6. E. Cacciola
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Correspondence to Eva Intagliata.

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Vecchio, R., Intagliata, E., Ferla, F. et al. Laparoscopic splenectomy in patients with hereditary spherocytosis: report on 12 consecutive cases. Updates Surg 65, 277–281 (2013). https://doi.org/10.1007/s13304-013-0236-x

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  • DOI: https://doi.org/10.1007/s13304-013-0236-x

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