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