Gallbladder Stones in Sickle Cell Anemia

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Gallbladder Stones in Sickle Cell Anemia
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SCA Complications

• Anemia
• Aplastic crisis
• Sequestration crisis
• Hemolytic crisis
• Dactylitis
• Painful crisis. VOC
• Acute chest syndrome
• Chronic lung disease
• priapism

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• CVA
• Ocular
• Gallbladder disease
• Renal
• Cardiomyopathy
• Skeletal
• Leg ulcers
• Infections
• Growth failure , delayed puberty
• psychological

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Causes of GB stones in pediatric

• Hemolytic ds ( sickle , spherocytosis )
• Obesity
• Ileal resection
• CF
• Chronic liver ds
• Crohn ds
• Prolonged TPN
• ..

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Gallstones types

• Pigment stones gt70, black-brown pigment , Ca
  bilirubinate glycoprotein ,
• Cholesterol stones 15-20

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• CHOLELITHIASIS is very common in patients with
  sickle cell anemia
• 17-33 of SCA complications
• incidence is much lower in patients with HbSC and
  HbS-beta thalasemmia
• Mainly Pigment
• Associated with the promoter of uridine
  diphosphate (UDP) glucoronosyltransferase 1A1
  ?unconjugated hyberbili.

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• C/P acute Rt, UQ sever colicky pain , fever ,
  jaundice fat intolerance, Rt, shoulder pain ,
  leukocyosis
• Symptomatic(mainly) and asymptomatic
• Complications cholycystitis ,cholangitis,
  jaundice , pancriatitis and gallstone ileus .

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Diagnosis

• CBC ? WBC
• ? amylase
• ? Bilirubine
• ? ALP
• X-ray 50 of pigment stones are radiopaque
• U/S
• MRCP
• ERCP

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Intervention

• conservative preoperative transfusion is
  recommended ..
• Cholecystectomy is the most frequently performed
  surgical procedure in SCA
• In order to avoid the subsequent confusion of
  acute cholecystitis pain and acute VOC,
  cholecystectomy has become a feasible approach in
  the management of gallstones

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• one study has recommended caution with regards to
  laparoscopic cholecystectomy due to perioperative
  complications in the context of sickle cell
  disease, advocating open cholecystectomy instead.
  
• another showed ,morbidity is the same in the open
  and laparoscopic in SCA,may reflect the
  importance of the underlying disease, rather than
  the procedure, in determining perioperative
  outcome.
• Open Vs lap chole is still debatable..