sickle cell disease
TRANSCRIPT
Surgical Aspects ofSickle Cell Disease
BY PROF/ GOUDA ELLABBAN
Pathogenesis
Deoxygenated HBS is insoluble which causes it to polymerize inside the RBCs leading them to become crescent shaped, sickling can produce:1-shorten of red cell2-impaired circulation through small blood vessel leading to infarction.
Sickle Cell Disease
Deoxygenation(or ↓H
20, ↓pH)
Sickle cell
Polymerizationof Hgb S
Precipitating factors
•1-dehydration•2-hypoxia•3-cold(causes vasospasm)•4-acidosis•5-infectionThe HB.S release its Oxygen easily than normal, & patient feel well deposit anemia except during crises or complication.
Types of crises:
1- vasoocclusive crisis ( mostly presents with pain ) 2- hemolytic crisis ( anemia , jaundice ) 3- sequestration ( organ damage ) 4- aplastic crisis .
1-Hemolytic crisis
-Jaundice and anemia
-no pain
-Its occur as result of destruction of RBC in the RES “extravascular hge”
2-Aplastic crisis-due to decrease erythropoietin associated with viral infection especially parovirus
Ischemic crises• Resulting from obstruction of small vessel, Which either A) micro infarct – painful crisis B) macro infarct – organ damage & it may lead to 1-bone pain “most common” & Fat emboli from bone marrow necrosis 2-hand & foot syndrome (dactylitis) d.t to infarction of small bone its quite common in
children 3-ischemic necrosis of femoral head particularly in adult 4-atrophic change of the spleen with painful infarction 5-ichemia of the brain leading to stroke hemiparesis or fits 6-ischemia of the eye lead to blindness 7-kidney with papillary necrosis 8-chronic leg ulcer 9-chest-pluritic pain
Acute condition in SCD• Acute sequestration of
the spleen
The spleen suddenly become engorged with sickle cell leading to hypovolemia & hypotension
•acute chest syndrome
• The patient present with
Fever, chest pain, difficulty in breathing, hypoxia & new x-ray change due to consolidation.
It can be due to, pneumonia, fat embolism from bone necrosis or sickle cell sequestration.
• Priapism
Gallbladder Disease
• Pathophysiology: ongoing hemolysis ➔ chronic bilirubin elevation ➔ pigmented bilirubin stones
• Acute attacks can be confused with a sickle-cell crisis in the liver.
• recurrent or severe pain from gallstones indicate the need of surgery
• laparoscopic procedure is preferred than open to reduce possible complications
Investigations• CBC• Peripheral blood smear• Sickling test• solubility test• HB electrophoresis• LFT• Urinanalysis• Stool analysis• Radiological• Septic work up
• 1- surgical:Blood transfusionSplenectomyBone marrow transplantation “<16
age with sever complication & have HLA-linked match doner”
• 2- medical:Antibiotic, hydroxyurea, analgesic,
iv fluid for dehydration, folic acid supplement.
Management
1-Episodic Transfusiona) Management of severe anemia:
acute splenic sequestration or acute attacks of hypersplenism
Aplastic or hemolytic crisis
b) Management of sudden severe illness:Acute chest syndromeStroke “fresh frozen plasma or human
albumin solutionMulti-organ failure
2-Preparation for surgery
• Preoperative partial exchange transfusion of packed cell to reduce H.b.S level to less than 30%
• 1) acute splenic sequestration crisis• 2) hypersplenism• 3) splenic abscess• 4) trauma, autoimmune thrombocytopenic purpura
hemolytic anemia
Indications for Splenectomy
They are liable to infections!!
Perioperative management in SCD
• The perioperative period is defined as the time before, during and after the operative procedure
• Surgical manifestations of sickle cell disease (SCD)
• Spleen : acute splenic sequestration crisis, splenic infarcts, splenic abscess.
• Hepato-biliary : hepatic crisis, acute hepatic failure, cholelithiasis, choledocholithiasis.
• Gastro-intestinal: peptic ulcers ,ischaemic colitis ,others: e.g. appendicitis, pancreatitis.
• Skin: leg ulcers.
• - General Management : • Care should be taken at all times to
avoid factors that may precipitate a sickle crisis. These include adequate hydration, good oxygenation, avoidance of hypothermia, acidosis, and local vascular stasis, and careful monitoring.. The majority of crises occur postoperatively.
• Preoperative preparation :1. Inform Haematology Team
that a patient with sickle cell disease has been admittedThey will help co-ordinate with the haematology laboratory
2. Take blood for full blood count and electrophoresis (%HbS,
HbA, HbF)
3. Crossmatch 1 unit phenotyped blood for all cases
4. Start intravenous maintenance fluids
12 hours preoperatively for all cases
5. Consider postoperative nasal CPAP
if the child has a history of severe obstructive sleep apnoea - discuss with the anaesthetist / respiratory team.
• Postoperative care
1. Monitor saturation continuously
» give oxygen to maintain saturation > 92%
2. Continue intravenous maintenance fluids
» until the child is tolerating oral fluids
3. Postoperative analgesia
» ensure this is adequate
4. Contact physiotherapy
» encourage early mobilisation
• Postoperative sickling complications • Serious postoperative complications (usually
within 48 hours of surgery) include: – painful crisis – cerebral event – chest crisis
• They may be accompanied by fever and may coexist.
• 1. Inform Haematology Team» if any of the above are suspected
• 2. Treatment of Sickle crisis includes:» intravenous fluids» oxygen therapy» antibiotics» analgesia as indicated » transfusion to Hb>10 g/dl (but <12g/dl)
• Over transfusion and hyperviscosity must be avoided• Exchange transfusion to reduce the HbS<20%-30%
may be indicated in certain situations- discuss with the haematologists / neurologists as indicated
• Analgesia: effective analgesia is required - contact the Pain Team
• Ventilation - serious chest crisis: the child may require ventilation in the event of a serious chest crisis - discuss early with the PICU Team