Hereditary spherocytosis surgery: Difference between revisions
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==Surgery== | ==Surgery== | ||
* Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of the red cell life span.<ref>{{Cite journal | |||
| author = [[P. H. B. Bolton-Maggs]], [[R. F. Stevens]], [[N. J. Dodd]], [[G. Lamont]], [[P. Tittensor]] & [[M.-J. King]] | |||
| title = Guidelines for the diagnosis and management of hereditary spherocytosis | |||
| journal = [[British journal of haematology]] | |||
| volume = 126 | |||
| issue = 4 | |||
| pages = 455–474 | |||
| year = 2004 | |||
| month = August | |||
| doi = 10.1111/j.1365-2141.2004.05052.x | |||
| pmid = 15287938 | |||
}}</ref><ref name="CasalePerrotta2014">{{cite journal|last1=Casale|first1=Maddalena|last2=Perrotta|first2=Silverio|title=Splenectomy for hereditary spherocytosis: complete, partial or not at all?|journal=Expert Review of Hematology|volume=4|issue=6|year=2014|pages=627–635|issn=1747-4086|doi=10.1586/ehm.11.51}}</ref> | |||
* Patients should be selected for splenectomy on the basis of their clinical symptoms and presence of complications such as gallstones, not simply on the basis of diagnosis alone. | |||
* Following splenectomy, the clinical manifestations and complications (anemia & gallstones) are much reduced in severe hereditary spherocytosis and abolished in milder cases, but at the risk of increased life threatening sepsis from encapsulated organisms, particularly streptococcus pneumoniae.<ref name="Bolton-MaggsStevens2004">{{cite journal|last1=Bolton-Maggs|first1=P. H. B.|last2=Stevens|first2=R. F.|last3=Dodd|first3=N. J.|last4=Lamont|first4=G.|last5=Tittensor|first5=P.|last6=King|first6=M.-J.|title=Guidelines for the diagnosis and management of hereditary spherocytosis|journal=British Journal of Haematology|volume=126|issue=4|year=2004|pages=455–474|issn=0007-1048|doi=10.1111/j.1365-2141.2004.05052.x}}</ref><ref>{{Cite journal | |||
| author = [[Sayeeda Huq]], [[Mark A. C. Pietroni]], [[Hafizur Rahman]] & [[Mohammad Tariqul Alam]] | |||
| title = Hereditary spherocytosis | |||
| journal = [[Journal of health, population, and nutrition]] | |||
| volume = 28 | |||
| issue = 1 | |||
| pages = 107–109 | |||
| year = 2010 | |||
| month = February | |||
| pmid = 20214092 | |||
}}</ref> | |||
* Children or young adults with mild hereditary spherocytosis who also has gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy.<ref name="Bolton-MaggsStevens2004">{{cite journal|last1=Bolton-Maggs|first1=P. H. B.|last2=Stevens|first2=R. F.|last3=Dodd|first3=N. J.|last4=Lamont|first4=G.|last5=Tittensor|first5=P.|last6=King|first6=M.-J.|title=Guidelines for the diagnosis and management of hereditary spherocytosis|journal=British Journal of Haematology|volume=126|issue=4|year=2004|pages=455–474|issn=0007-1048|doi=10.1111/j.1365-2141.2004.05052.x}}</ref> | |||
=== Splenectomy: === | === Splenectomy: === | ||
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●It is not clear whether pulmonary artery hypertension (PAH) is a complication of splenectomy in HS.<ref name="pmid17497044">{{cite journal| author=Smedema JP, Louw VJ| title=Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis. | journal=Cardiovasc J Afr | year= 2007 | volume= 18 | issue= 2 | pages= 84-9 | pmid=17497044 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17497044 }}</ref> | ●It is not clear whether pulmonary artery hypertension (PAH) is a complication of splenectomy in HS.<ref name="pmid17497044">{{cite journal| author=Smedema JP, Louw VJ| title=Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis. | journal=Cardiovasc J Afr | year= 2007 | volume= 18 | issue= 2 | pages= 84-9 | pmid=17497044 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17497044 }}</ref> | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
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Overview
- Generally, the treatment of HS involves presplenectomy care, splenectomy, and management of postsplenectomy complications.
- In pediatric cases, splenectomy ideally should not be performed until a child is older than 6 years because of the increased incidence of postsplenectomy infections with encapsulated organisms such as S pneumoniae and H influenzae in young children.
- Partial splenectomies are increasingly used in pediatric patients, as this approach appears to both control hemolysis and preserve splenic function.
Surgery
- Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of the red cell life span.[1][2]
- Patients should be selected for splenectomy on the basis of their clinical symptoms and presence of complications such as gallstones, not simply on the basis of diagnosis alone.
- Following splenectomy, the clinical manifestations and complications (anemia & gallstones) are much reduced in severe hereditary spherocytosis and abolished in milder cases, but at the risk of increased life threatening sepsis from encapsulated organisms, particularly streptococcus pneumoniae.[3][4]
- Children or young adults with mild hereditary spherocytosis who also has gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy.[3]
Splenectomy:
- Generally, the treatment of HS involves presplenectomy care, splenectomy, and management of postsplenectomy complications.
- In pediatric cases, splenectomy ideally should not be performed until a child is older than 6 years because of the increased incidence of postsplenectomy infections with encapsulated organisms such as S pneumoniae and H influenzae in young children.
- Partial splenectomies are increasingly used in pediatric patients, as this approach appears to both control hemolysis and preserve splenic function.
- European guidelines on splenectomy for HS note that a laparoscopic approach is currently considered the gold standard for removal of a normal-sized or slightly enlarged spleen and is preferred to open splenectomy, but it should be performed only by experienced surgeons.
- In children undergoing splenectomy, the gallbladder should be removed concomitantly if the patient has symptomatic gallstones.
Complications — Splenectomy has a number of known risks of which patients (or parents) should be aware:[5]
●Operative risks (eg, infection, bleeding, or injury to adjacent organs such as the stomach or tail of the pancreas); these are relatively infrequent.
●Infections, including overwhelming sepsis, from encapsulated organisms (eg, Streptococcus pneumoniae, Neisseria meningitidis, H. influenzae) that can no longer be removed by normal splenic clearance mechanisms, as well as certain other microorganisms including plasmodia, Babesia, Bordetella, and Capnocytophaga species (from animal bites).[6] These risks are thought to be highest in the first year following splenectomy and in individuals undergoing splenectomy before five to six years of age.
However, risks of sepsis are likely to have declined with improved options for preoperative vaccinations and postoperative prophylactic penicillin. This was illustrated in a 1991 study from the Danish National Patient Registry that demonstrated a dramatic reduction in serious S. pneumoniae infections following pneumococcal vaccination.[7] Individuals who did not receive appropriate pre-splenectomy vaccinations should have a thorough review of their immunization history and should receive vaccinations as discussed separately.
●Venous thromboembolic (VTE) complications including thromboses of the deep veins, pulmonary emboli, splenic or portal vein thrombosis, as well as thrombosis in other unusual sites.[8][9] VTE events appear to be more common in individuals with HS who undergo splenectomy than in those who do not, but the individuals who undergo splenectomy may have had more severe underlying disease, making direct comparisons difficult. Thromboprophylaxis at the time of surgery should be based on standard practices; there is no indication for extended thromboprophylaxis beyond the usual duration.
●Arterial thrombotic events may also be increased relative to individuals with HS who do not undergo splenectomy, with the same caveat that applies to VTE (patients who undergo splenectomy may have more severe underlying disease).[10][11]
●It is not clear whether pulmonary artery hypertension (PAH) is a complication of splenectomy in HS.[12]
References
- ↑ P. H. B. Bolton-Maggs, R. F. Stevens, N. J. Dodd, G. Lamont, P. Tittensor & M.-J. King (2004). "Guidelines for the diagnosis and management of hereditary spherocytosis". British journal of haematology. 126 (4): 455–474. doi:10.1111/j.1365-2141.2004.05052.x. PMID 15287938. Unknown parameter
|month=ignored (help) - ↑ Casale, Maddalena; Perrotta, Silverio (2014). "Splenectomy for hereditary spherocytosis: complete, partial or not at all?". Expert Review of Hematology. 4 (6): 627–635. doi:10.1586/ehm.11.51. ISSN 1747-4086.
- ↑ 3.0 3.1 Bolton-Maggs, P. H. B.; Stevens, R. F.; Dodd, N. J.; Lamont, G.; Tittensor, P.; King, M.-J. (2004). "Guidelines for the diagnosis and management of hereditary spherocytosis". British Journal of Haematology. 126 (4): 455–474. doi:10.1111/j.1365-2141.2004.05052.x. ISSN 0007-1048.
- ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter
|month=ignored (help) - ↑ Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L; et al. (2017). "Recommendations regarding splenectomy in hereditary hemolytic anemias". Haematologica. 102 (8): 1304–1313. doi:10.3324/haematol.2016.161166. PMC 5541865. PMID 28550188.
- ↑ Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L; et al. (2017). "Recommendations regarding splenectomy in hereditary hemolytic anemias". Haematologica. 102 (8): 1304–1313. doi:10.3324/haematol.2016.161166. PMC 5541865. PMID 28550188.
- ↑ Konradsen HB, Henrichsen J (1991). "Pneumococcal infections in splenectomized children are preventable". Acta Paediatr Scand. 80 (4): 423–7. PMID 2058391.
- ↑ Davidsen C, Larsen TH, Gerdts E, Lønnebakken MT (2016). "Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report". Thromb J. 14: 9. doi:10.1186/s12959-016-0083-3. PMC 4845368. PMID 27118929.
- ↑ Perkins LA, Jones SF, Bhargava RS (2009). "Dural venous thrombosis following splenectomy in a patient with hereditary spherocytosis". South Med J. 102 (5): 542–5. doi:10.1097/SMJ.0b013e31819e90b5. PMID 19373154.
- ↑ Schilling RF, Gangnon RE, Traver MI (2008). "Delayed adverse vascular events after splenectomy in hereditary spherocytosis". J Thromb Haemost. 6 (8): 1289–95. doi:10.1111/j.1538-7836.2008.03024.x. PMID 18485083.
- ↑ Schilling RF (1997). "Spherocytosis, splenectomy, strokes, and heat attacks". Lancet. 350 (9092): 1677–8. PMID 9400518.
- ↑ Smedema JP, Louw VJ (2007). "Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis". Cardiovasc J Afr. 18 (2): 84–9. PMID 17497044.