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Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
Review article

POST-OPERATIVE COMPLICATIONS IN SICKLE CELL DISEASE PATIENTS

Abdulrahman Saleh Al-Mulhim, Hessah Abdulaziz Al-Hussaini, Sara Abdullah Alnajjar, Marwah Abdulaziz Al-
Awas, Alia Ali Alokley, Ilham Abdulrahman Almousa
Medical College - King Faisal University, Kingdom of Saudi Arabia
1. INTRODUCTION
Prevalence of post-operative complications in
Sickle cell disease patients:
Prevalence of sickle cell disease:
The post-operative complications can be broadly Worldwide, sickle cell disease is seen in classified into sickle-cell-related complications and approximately 5% of population [1], and it is most non-sickle-cell related complications. The most common in people originally from sub-Saharan Africa, common sickle-cell-related complication is vaso- India, Saudi Arabia and Mediterranean countries [2]. In occlusive crisis and the most serious is acute chest Saudi Arabia, it is highly prevalent in the Eastern syndrome. There are several factors that increase region. The Saudi Premarital Screening Program the risk of certain complications, like hemoglobin estimated the prevalence of the sickle cell gene in the S level, pre-operative blood transfusion, type of adult population at 17% for sickle-cell trait and 1.2% anesthesia, emergency-versus-elective surgeries, type of surgery and laparoscopic-versus-open Types of operations common in Sickle cell disease
surgeries [4],[6],[7],[8],[9],[10],[11],[12]. patients:
2. MAIN TYPES OF POST-OPERATIVE
All sickle cell disease patients can experience any type COMPLICATIONS:
of surgery as the general population [4]. However, there are certain operations that occur more frequently in Sickle-cell-related
Non-Sickle-cell-
complications [4]
sickle cell disease patients in comparison to general complications [4]
cholecystectomy, splenectomy and hip replacement [5]. Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
3. DEATH:
unpredictable recurrences of acute crises on chronic pain creates a unique pain syndrome [15]. Most of postoperative mortality is mainly due the 4.2. Pathophysiology:
associated comorbid conditions, and second due to In VOC, obstruction of blood flow results in sickle-cell related complications. Bleeding, sepsis and regional hypoxemia and acidosis. The severe pain multi-organ failure are the most common sickle-cell is believed to be caused by increased intra- related causes of death postoperatively [4],[13]. medullary pressure, especially within the juxta- Risk factors:
articular areas of long bones, secondary to an acute The surgical procedure doesn't play a major role in inflammatory response to vascular necrosis of the causing postoperative death. However, high level of bone marrow by sickled erythrocytes [15]. hemoglobin S, preoperative blood transfusion and  Risk factors:
high-risk surgeries (i.e. involving intracranial, There are at least three sets of known predisposing cardiovascular and thoracic procedures) increase the events that seem to predict the frequency and risk of postoperative death among sickle cell disease severity of the acute sickle cell painful crisis: genetic, cellular, and environmental/epigenetic 4. VASO-OCCLUSIVE CRISIS:
factors. Major reported factors that seem to Definition:
precipitate acute painful crises include: Vaso-occlusive crisis (VOC), also known as adult Not related to
Related to surgery [16],[17]
surgery [14]
pain syndrome [14], is one of the common reason Dehydration, stress of 1. General anesthesia any kind (physical, 2. Open surgery (e.g. traumatic, hospitalizations in patients with sickle cell disease. Sickle cell pain is often classified as either acute or chronic. The acute type is caused by ischemic acidosis, sleep apnea, climate, and pregnancy tissue injury resulting from the occlusion of microvascular beds by sickled erythrocytes during an acute crisis. Chronic pain occurs because of the 4.3.
Management:
destruction of bones, joints and visceral organs as a Postoperative pain management is helped by result of recurrent crises. The effect of giving multiple analgesia. VOC treatment mainly Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
consists of intravenous morphine, which has many the pulmonary vasculature, a variety of inciting dose-related side effects. Non-steroidal anti- events triggers deoxygenation of hemoglobin S inflammatory drugs have been proposed to provide (Hgb S) leading to Hgb S polymerization and pain relief and decrease the need for opioids [18]. sickling of the red blood cell that results in 4.4. Prevention:
vasoocclusion, ischemia, and endothelial injury [28]. Sickling is avoided by preventing risk factors Multiple factors may be present and contribute to mentioned above. Hydroxyurea can decrease the ACS. They include inflammation (secondary to infection or fat embolus), regional alveolar hypoxia 5. Acute chest syndrome:
5.1. Definition:
Risk factors:
The acute chest syndrome (ACS) is the most The following factors increase the risk of common form of acute pulmonary disease in patients with Sickle cell disease, occurring in almost one-half of patients [19],[20]. It is the most frequently reported cause of death in adults, and it is a risk factor for early mortality [21],[22],[23]. ACS may present as a  Open surgery (ACS mostly occur within first 24 postoperative complication in children with Sickle cell disease [24],[25]. A working definition of ACS in a Management:
patient with sickle cell disease is the presence of a The supportive management of an episode of ACS new pulmonary infiltrate (not due to atelectasis, requires a number of active interventions, involving at least one complete lung segment), including adequate pain control and fluid respiratory symptoms (chest pain, dyspnea, management [27], use of bronchodilators when wheezing, or cough) and temperature >38.5ᵒC wheezing is present, incentive spirometry to prevent the development of pulmonary atelectasis, 5.2. Pathophysiology:
and use of supplemental oxygen [30]. Specific The pathogenesis of acute pulmonary disease in therapies for ACS include blood transfusion, SCD is complex, and the establishment of a specific cause in any single case is difficult. Within Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
antibiotics and venous thromboembolism 7. Overwhelming postsplenectomy infection:
Definition:
Prevention:
Overwhelming postsplenectomy infection (OPSI) is life-threatening complication of splenectomy. In Careful perioperative management, including general, it results in 3.2% for invasive infection appropriate presurgical transfusion support and and an overall mortality of 1.4% among adult postoperative pulmonary care can minimize the patients and in 1.7% of children. More than half of these infections occur within the first two years after splenectomy [36]. OPSI presents with mild Hydroxyurea can prevent the recurrence of ACS symptoms of flu-like such as low grade fever, chills, muscle aches, and nausea followed by subsequent rapid deterioration may occur in the 6. Stroke:
Stroke is an important and common complication form of fatigue, colored skin, body weight loss, of Sickle cell disease, affecting children as well as abdominal pain, diarrhea, constipation, nausea, and adults. Clinically evident stroke, usually brain infarction, associated with stenosis or occlusion of meningitis which can lead to fulminant sepsis, the intracranial arteries of the Circle of Willis, disseminated intravascular coagulation, and multi- Japanese word for "hazy" or "like a puff of smoke" Etiology:
that describes the appearance of an abnormal Encapsulated bacteria are the primary causative microvasculature on angiography believed secondary to internal carotid artery stenosis or  Streptococcus pneumonia (causes 70% of bacteremic episodes following splenectomy) collateralization) [34]. Sickle cell disease confers a greatly increased risk of ischemic and primary  Neisseria meningitides (meningococcus) Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
Management and prevention:
Appropriate and timely immunization [36]: Anti-pneumococcal, anti-meningococcal and anti- haemophilus influenza vaccines should be received Pathophysiology:
Cefazoline or clindamycin should be administered, homoeostasis will be disturbed, leading to decreased clearance of particulate antigens from the circulation, diminished clearance of opsonized Penicillin V can be used postoperatively for 2 bacterial antigens, diminished primary humoral years in adults and 5 years in children. Duration of response to neoantigens, and diminished antibody penicillin V can be extended according to patient response to polysaccharides. Furthermore, IgM memory B cells, which are responsible for protecting against infection from encapsulated Patients should understand the potential severity of bacteria, require the spleen for their survival [37]. OPSI and the possibility of rapid progression. Risk factors:
 The time interval after splenectomy (most cases In case of infection symptoms and fever higher occur within 2 years postsplenectomy) [37] than 38°C, early antibiotic therapy with amoxicillin  Sickle cell disease itself carries the highest risk for clavulanate should be administered (or cefuroxime  Some surgeries can reduce the risk of OPSI, such 8. Fever and infection:
Definition:
splenectomy during exploratory laparotomy for Fever above 38ºC (100.4ºF) is common in the first few days after major surgery. Most early postoperative fever is caused by the inflammatory stimulus of surgery and resolves spontaneously. Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
However, postoperative fever can be a 8.4. Risk factors:
manifestation of a serious complication [40]. Pathophysiology:
Prolonged hospital stay (>24 hr) that may Patients with Sickle cell disease are more influenced by Sickle cell disease can increase the susceptible to infections because of functional risk of fever (0.5%) and respiratory complication asplenia. Autoinfarction caused by sickling of red blood cells results in a nonfunctioning spleen, which is unable to filter bacteria from the blood Simple transfusion is not associated with high stream, and increase the risk of invasive infection incidence of infection in comparison to aggressive by encapsulated organism accordingly. Other than having an infectious cause, fever is also a manifestation of cytokine release in response to a It decreases the risk of splenic sequestration in variety of stimuli. Fever-associated cytokines, Sickle cell and reduces the need for blood including interleukin (IL)-1, IL-6, tumor necrosis transfusions. So, infection rates did not increase factor (TNF)-alpha, and interferon (IFN)-gamma, after the procedure during the follow-up period are produced by a variety of tissues and cells. There is some evidence that IL-6 is the cytokine 8.5. Management and prevention:
most closely correlated with postoperative fever All measures of immunization, antibiotic prophylaxis and prompt treatment of infection are Differential diagnosis [40]:
Immediate Acute
Subacute
Delayed unnecessary treatments, including medications,
nasogastric tubes and intravascular and urinary catheters should be discontinued in the febrile Causes . Medications
patient. Treating the fever with acetaminophen is 9. Bleeding, thrombosis and embolism:
These postoperative complications are not common in patients with sickle cell disease in comparison to Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
the above mentioned complications. Some studies Anesthesia in Sickle Cell Disease. Blood mentioned that preoperative blood transfusion increases the risk of these complications [4]. In 5. Adam S, Jonassaint J, Kruger H, Kail M, Orringer addition, the risk of excessive blood loss varies EP, Eckman JR et al. Surgical and Obstetric according to the procedure [43]. A case report Outcomes in Adults with Sickle Cell Disease. Am mentioned that prolonged laparoscopic surgeries can increase the risk of portal vein thrombosis [44]. 6. Hirst C, Williamson L. Preoperative Blood 10. Conclusion:
Transfusions for Sickle Cell Disease. Cochrane Database Syst Rev 2012 Jan 18;1:CD003149. It has been approved that sickle cell diseases patients 7. Aziz AM, Meshikhes AW. Blood Transfusion in are more susceptible to serious post-operative complications compared to the general population. These complications can be minimized by prompt perioperative assessment and intervention. Assessment of Perioperative Transfusion Therapy REFERENCES
and Complications in Sickle Cell Disease Patients 1. Sickle-cell Disease and Other Haemoglobin Disorders [Online]. 2011 Jan; Available from: URL:http://www.who.int/mediacentre/factsheets/f Cholecystectomy in Adult Patients with Sickle Cell Disease. Surg Laparosc Endosc Percutan 2. Sickle-cell Anaemia [Online]. 2006 April 24; Available from: URL:http://apps.who.int/gb 10. Al-Mulhim AS, Al-Mulhim AA. Laparoscopic Cholecystectomy in 427 Adults with Sickle Cell 3. Jastaniah W. Epidemiology of Sickle Cell Disease Disease: A Single-center Experience. Surg in Saudi Arabia. Ann Saudi Med. 2011 May- 4. Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK et al. Surgery and Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
11. Al-Mulhim AS. Laparoscopic Splenectomy for Massive Splenomegaly in Benign Hematological Diseases. Surg Endosc 2012 Nov;26(11):3186-9. Ketoprofen for Sickle-cell Disease Vaso-occlusive Laparoscopic Splenectomy and/or Crises in Adults. Blood Cholecystectomy for Children with Sickle Cell Disease. Pediatr Surg Int 2009 May;25(5):417-21. 13. Manci EA, Culberson DE, Yang YM, Gardner Complications of Sickle Cell Anemia. A Need for TM, Powell R, Haynes J Jr et al. Causes of Increased Recognition, Treatment, and Research. Death in Sickle Cell Disease: An Autopsy Study. Am J Respir Crit Care Med 2001; 164(11):2016- GA, Dampier C, Osunkwo I et al. Beyond the Complications of Sickle Cell Disease. Semin Definitions of the Phenotypic Complications 21. Gray A, Anionwu EN, Davies SC, Brozovic M. Patterns of mortality in sickle cell disease in the ScientificWorldJournal 2012;2012:949535. United Kingdom. J Clin Pathol 1991; 44:459. 15. Yale SH, Nagib N, Guthrie T. Approach to the 22. Platt OS, Brambilla DJ, Rosse WF, et al. Vaso-occlusive Crisis in Adults with Sickle Cell Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med Sani 23. Gladwin MT, Schechter AN, Shelhamer JH, CM, Habibou A. Laparoscopic Cholecystectomy Ognibene FP. The acute chest syndrome in sickle in Sickle Cell Patients in Niger. Pan Afr Med cell disease. Possible role of nitric oxide in its pathophysiology and treatment. Am J Respir Crit 17. Al-Ebrahim KE. Cardiac Surgery and Sickle Cell 24. Platt OS. The acute chest syndrome of sickle cell Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
25. Wales PW, Carver E, Crawford MW, Kim PC. 32. Ballas SK, Lieff S, Benjamin LJ, et al. Definitions Acute chest syndrome after abdominal surgery in of the phenotypic manifestations of sickle cell children with sickle cell disease: Is a laparoscopic approach better? J Pediatr Surg 2001; 36:718. 33. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises complications of sickle cell disease. N Engl J Med in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell 27. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome 34. Gebreyohanns M, Adams RJ. Sickle Cell Disease: in sickle cell disease. National Acute Chest Primary Stroke Prevention. CNS Spectr 2004 Syndrome Study Group. N Engl J Med 2000; 35. Strouse JJ, Jordan LC, Lanzkron S, Casella JF. 28. Boyd JH, DeBaun MR, Morgan WJ, et al. Lower The Excess Burden of Stroke in Hospitalized airway obstruction is associated with increased Adults with Sickle Cell Disease. Am J Hematol morbidity in children with sickle cell disease. 36. Romano F, Garancini M, Ciravegna AL, Uggeri 29. Graham LM. Sickle cell disease: pulmonary F, Degrate L, Maternini M. The Implications for management options. Pediatr Pulmonol Suppl Patients Undergoing Splenectomy: Postsurgery Risk Management. Dove Press Journal: Open 30. Powars D, Weidman JA, Odom-Maryon T, et al. Access Surgery 2011 Aug; 2011(4): 21-34. Sickle cell chronic lung disease: prior morbidity 37. Lammers AJ. The Value of Prophylactic and the risk of pulmonary failure. Medicine Vaccinations and Antibiotic Treatment in Post- splenectomy Patients: A Review. Dove Press 31. Godeau B, Schaeffer A, Bachir D, et al. Bronchoalveolar lavage in adult sickle cell patients with acute chest syndrome: value for 38. Pasternack MS. Prevention of Sepsis in the diagnostic assessment of fat embolism. Am J Int j clin surg adv 2013; 1(2):41-50 Post-operative Complications in Sickle Cell Disease Patients Abdulrahman Saleh Al-Mulhim et al
URL:http://www.uptodate.com/contents/preventio Postsplenectomy Infection Syndrome in Adults - A Clinically Preventable Disease. World J CORRESPONDENCE:

Gastroenterol 2008 January 14; 14(2): 176–179. Dr. Abdulrahman Saleh Al-Mulhim,
Associate Professor of Surgery
40. Weed HG, Baddour LM. Postoperative Fever Department of Surgery
Medical College - King Faisal University,
[Online]. 2012 March 21. Available from: Hofuf, P.O. Box 1164Al-Hassa 31982
Kingdom of Saudi Arabia
E-mail address: [email protected]
URL:http://www.uptodate.com/contents/postopera tivefever?source=search_result&search=postopera tive+fever+in+sickle+cell+disease&selectedTitle= 41. Wali YA, al Okbi H, al Abri R. A Comparison of Two Transfusion Regimens in the Perioperative Disease Undergoing Adenotonsillectomy. Pediatr Splenectomy Prevents Splenic Sequestration Crises in Sickle Cell Disease. J Pediatr Surg 2009 43. Sathappan SS, Ginat D, Di Cesare PE. Management of Orthopedic Patients with Sickle C ell Disease. Orthopedics 2006 Dec;29(12):1094- Following Laparoscopic Surgery in a Patient with Int j clin surg adv 2013; 1(2):41-50

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