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 : %

CHOLELITHIASIS is very common in patients with sickle cell anemia % 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.

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..