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


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
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:
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
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
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.
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:
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:
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
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
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URL: 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:

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