Gallbladder Stones in Sickle Cell Anemia SCA Complications
Gallbladder Stones in Sickle Cell Anemia
SCA Complications • • • Anemia Aplastic crisis Sequestration crisis Hemolytic crisis Dactylitis Painful crisis. VOC Acute chest syndrome Chronic lung disease priapism
• • • CVA Ocular Gallbladder disease Renal Cardiomyopathy Skeletal Leg ulcers Infections Growth failure , delayed puberty psychological
Causes of GB stones in pediatric : • • Hemolytic ds ( sickle , spherocytosis ) Obesity Ileal resection CF Chronic liver ds Crohn ds Prolonged TPN. .
Gallstones types • Pigment stones: >70%, black-brown pigment , Ca bilirubinate & glycoprotein , • Cholesterol stones : 15 -20 %
• CHOLELITHIASIS is very common in patients with sickle cell anemia • 17 -33 % of SCA complications • incidence is much lower in patients with Hb. SC and Hb. S-beta thalasemmia • Mainly Pigment • Associated with the promoter of uridine diphosphate (UDP) glucoronosyltransferase 1 A 1 →unconjugated hyberbili.
• 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.
Diagnosis • • CBC : ↑ WBC ↑ amylase ↑ Bilirubine ↑ ALP X-ray : 50% of pigment stones are radiopaque U/S MRCP ERCP
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
• 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. .
- Slides: 10