Hereditary spherocytosis surgery: Difference between revisions

Jump to navigation Jump to search
No edit summary
 
(16 intermediate revisions by 2 users not shown)
Line 2: Line 2:
{{Hereditary spherocytosis}}
{{Hereditary spherocytosis}}
{{CMG}} {{AE}}
{{CMG}} {{AE}}
{{PleaseHelp}}


==Overview==
==Overview==
Generally, the treatment of [[hereditary spherocytosis]] involves presplenectomy care, [[splenectomy]] and management of postsplenectomy [[Complication (medicine)|complications]]. [[Splenectomy]] is very effective in reducing [[hemolysis]], leading to significant prolongation of [[red blood cell]] [[lifespan]]. Partial [[Splenectomy|splenectomies]] can be used in [[Pediatrics|pediatric]] [[Patient|patients]] as it controls [[hemolysis]] and preserves [[Spleen|splenic]] [[Function (biology)|function]]. [[Patient|Patients]] having concomitant [[Gallstone disease|gallstones]] are likely to benefit from combined [[splenectomy]] and [[cholecystectomy]] in terms of [[life expectancy]]. Post [[splenectomy]] [[Complication (medicine)|complications]] may include; [[Infection|infections]] & [[sepsis]] caused by [[encapsulated organisms]] ([[streptococcus pneumoniae]], [[neisseria meningitidis]], [[Haemophilus influenzae|haemophilus influenza]]), [[Deep vein thrombosis|deep venous thrombosis (DVT)]], [[Pulmonary embolism|pulmonary emboli]] and [[portal vein thrombosis]].


==Surgery==
==Surgery==
* Splenectomy is the definitive treatment for HS. <sup>[3]</sup> Except in the unusual autosomal recessive variant of HS, splenectomy usually eliminates hemolysis and the associated signs and symptoms. <sup>[4]</sup> Interestinagly, splenectomy does little to correct the cytoskeletal membrane defects of HS; an atomic force microscopy study by Li et al found that after splenectomy, erythrocytes were larger but still spheroidal-shaped, with a disorganized membrane ultrastructure and reduced surface particle size.  
 
* '''Splenectomy'''
** [[Splenectomy]] is very effective in reducing [[hemolysis]], leading to significant prolongation of the [[red blood cell]] [[lifespan]].<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>
** [[Patient|Patients]] should be selected for [[splenectomy]] on the basis of their [[clinical]] [[Symptom|symptoms]] and presence of [[Complication (medicine)|complications]] such as [[Gallstone disease|gallstones]], not simply on the basis of [[diagnosis]] alone.
** Generally, the treatment of [[hereditary spherocytosis]] involves presplenectomy care, [[splenectomy]], and management of postsplenectomy [[Complication (medicine)|complications]].
** In [[Pediatrics|pediatric]] cases, [[splenectomy]] ideally should not be performed until a [[child]] is older than 6 years because of the increased [[incidence]] of postsplenectomy [[Infection|infections]] with [[encapsulated organisms]] such as [[streptococcus pneumoniae]] and [[haemophilus influenzae]] in young [[Child|children]].
** Partial splenectomies are increasingly used in [[Pediatrics|pediatric]] [[Patient|patients]], as this approach appears to both control [[hemolysis]] and preserve [[Spleen|splenic]] [[Function (biology)|function]]. 
** European guidelines on [[splenectomy]] for [[hereditary spherocytosis]] note that a [[Laparoscopic surgery|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 [[Surgeon|surgeons]].
** [[Child|Children]] or young [[Adult|adults]] with mild [[hereditary spherocytosis]] who also has [[Gallstone disease|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>
** Following [[splenectomy]], the [[clinical]] manifestations and [[Complication (medicine)|complications]] ([[anemia]] & [[Gallstone disease|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>
 
* '''Complications of splenectomy'''
** [[Splenectomy]] has a number of known risks of which [[Patient|patients]] (or parents) should be aware<ref name="pmid28550188">{{cite journal| author=Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L et al.| title=Recommendations regarding splenectomy in hereditary hemolytic anemias. | journal=Haematologica | year= 2017 | volume= 102 | issue= 8 | pages= 1304-1313 | pmid=28550188 | doi=10.3324/haematol.2016.161166 | pmc=5541865 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28550188  }}</ref>
** Operative risks (eg, [[infection]], [[bleeding]], or [[injury]] to adjacent [[Organ (anatomy)|organs]] such as the [[stomach]] or [[Tail of pancreas|tail of the pancreas]]); these are relatively infrequent. 
** [[Infection|Infections]], including overwhelming [[sepsis]], from [[encapsulated organisms]] ([[streptococcus pneumoniae]], [[neisseria meningitidis]], [[Haemophilus influenzae|hemophilus influenzae]]) that can no longer be removed by normal [[Spleen|splenic]] clearance mechanisms, as well as certain other [[Microorganism|microorganisms]] including [[Plasmodium|plasmodia]], [[Babesia]], [[Bordetella]] and [[Capnocytophaga]] [[species]] (from [[Animal bite|animal bites]]).<ref name="pmid285501882">{{cite journal| author=Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L et al.| title=Recommendations regarding splenectomy in hereditary hemolytic anemias. | journal=Haematologica | year= 2017 | volume= 102 | issue= 8 | pages= 1304-1313 | pmid=28550188 | doi=10.3324/haematol.2016.161166 | pmc=5541865 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28550188  }}</ref>
** These risks are thought to be highest in the [[Infant|first year]] following [[splenectomy]] and in individuals undergoing [[splenectomy]] before five to six years of [[Ageing|age]]. 
** However, risks of [[sepsis]] are likely to have declined with improved options for preoperative [[Vaccination|vaccinations]] and postoperative [[Prophylaxis|prophylactic]] [[penicillin]]. This was illustrated in a 1991 study from the Danish National Patient Registry that demonstrated a dramatic reduction in serious [[Streptococcus pneumoniae|streptococcus pneumoniae infections]] following [[Pneumococcal vaccine|pneumococcal vaccination]].<ref name="pmid2058391">{{cite journal| author=Konradsen HB, Henrichsen J| title=Pneumococcal infections in splenectomized children are preventable. | journal=Acta Paediatr Scand | year= 1991 | volume= 80 | issue= 4 | pages= 423-7 | pmid=2058391 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2058391  }}</ref>
** Individuals who did not receive appropriate pre-splenectomy [[Vaccination|vaccinations]] should have a thorough review of their [[immunization]] history and should receive [[Vaccination|vaccinations]].
** [[Venous thromboembolism|Venous thromboembolic (VTE)]] [[Complication (medicine)|complications]] including [[Deep vein thrombosis|thromboses of the deep veins]], [[Pulmonary embolism|pulmonary emboli]], [[Splenic vein thrombosis|splenic]] or [[portal vein thrombosis]], as well as [[thrombosis]] in other unusual sites.<ref name="pmid27118929">{{cite journal| author=Davidsen C, Larsen TH, Gerdts E, Lønnebakken MT| title=Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report. | journal=Thromb J | year= 2016 | volume= 14 | issue=  | pages= 9 | pmid=27118929 | doi=10.1186/s12959-016-0083-3 | pmc=4845368 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27118929  }}</ref><ref name="pmid19373154">{{cite journal| author=Perkins LA, Jones SF, Bhargava RS| title=Dural venous thrombosis following splenectomy in a patient with hereditary spherocytosis. | journal=South Med J | year= 2009 | volume= 102 | issue= 5 | pages= 542-5 | pmid=19373154 | doi=10.1097/SMJ.0b013e31819e90b5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19373154  }}</ref>
** [[Venous thromboembolism]] events appear to be more common in individuals with [[hereditary spherocytosis]] 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.
** [[Prophylaxis|Thromboprophylaxis]] at the time of [[surgery]] should be used based on standard practices; there is no indication for extended [[Prophylaxis|thromboprophylaxis]] beyond the usual duration. 
** [[Arterial thrombosis|Arterial thrombotic events]] may also be increased relative to individuals with [[hereditary spherocytosis]] who do not undergo [[splenectomy]], with the same caveat that applies to [[venous thromboembolism]] (patients who undergo [[splenectomy]] may have more severe underlying [[disease]]).<ref name="pmid18485083">{{cite journal| author=Schilling RF, Gangnon RE, Traver MI| title=Delayed adverse vascular events after splenectomy in hereditary spherocytosis. | journal=J Thromb Haemost | year= 2008 | volume= 6 | issue= 8 | pages= 1289-95 | pmid=18485083 | doi=10.1111/j.1538-7836.2008.03024.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18485083  }}</ref><ref name="pmid9400518">{{cite journal| author=Schilling RF| title=Spherocytosis, splenectomy, strokes, and heat attacks. | journal=Lancet | year= 1997 | volume= 350 | issue= 9092 | pages= 1677-8 | pmid=9400518 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9400518  }}</ref>
** It is not clear whether [[Pulmonary artery hypertension|pulmonary artery hypertension (PAH)]] is a [[Complication (medicine)|complication]] of [[splenectomy]] in [[hereditary spherocytosis]].<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==

Latest revision as of 03:59, 19 December 2018

Hereditary spherocytosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hereditary spherocytosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hereditary spherocytosis surgery On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hereditary spherocytosis surgery

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hereditary spherocytosis surgery

CDC on Hereditary spherocytosis surgery

Hereditary spherocytosis surgery in the news

Blogs on Hereditary spherocytosis surgery

Directions to Hospitals Treating Hereditary spherocytosis

Risk calculators and risk factors for Hereditary spherocytosis surgery

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Generally, the treatment of hereditary spherocytosis involves presplenectomy care, splenectomy and management of postsplenectomy complications. Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of red blood cell lifespan. Partial splenectomies can be used in pediatric patients as it controls hemolysis and preserves splenic function. Patients having concomitant gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy. Post splenectomy complications may include; infections & sepsis caused by encapsulated organisms (streptococcus pneumoniae, neisseria meningitidis, haemophilus influenza), deep venous thrombosis (DVT), pulmonary emboli and portal vein thrombosis.

Surgery

References

  1. 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)
  2. 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. 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.
  4. 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)
  5. 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.
  6. 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.
  7. Konradsen HB, Henrichsen J (1991). "Pneumococcal infections in splenectomized children are preventable". Acta Paediatr Scand. 80 (4): 423–7. PMID 2058391.
  8. 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.
  9. 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.
  10. 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.
  11. Schilling RF (1997). "Spherocytosis, splenectomy, strokes, and heat attacks". Lancet. 350 (9092): 1677–8. PMID 9400518.
  12. Smedema JP, Louw VJ (2007). "Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis". Cardiovasc J Afr. 18 (2): 84–9. PMID 17497044.

Template:WS Template:WH