Sickle small cavity disease (SCD) ...

Sickle small cavity disease (SCD) is one of the principally common genetic disorders in the United States. (1) Primary symptoms of this chronic disease include chronic hemolytic anemia and tissue damage secondary to vaso-occlusion through sickle-shaped red blood cells (Figure 1) Patients with SCD have an increased risk of developing cholelithiasis (ie, gallstones). (2) Cholelithiasis should be considered as a potential diagnosis when a child with SCD experiences renewed abdominal pain. (3) Evaluation criteria used to confirm the diagnosis of cholelithiasis include radiological evaluation using biliary scintigram and ultrasound and laboratory inferences that indicate elevations in serum

[FIGURE 1 OMITTED]

* bilirubin,

* alkaline phosphatase,

* hepatic enzyme evens and

* white line cell count. (4)

An estimated 50% of patients in sickle enclosed space crisis (ie, pain crisis) experience acute abdominal pain, which frequently is difficult to differentiate from acute cholecystitis. (5) undivided group of researchers noted that acute abdominal pain symptoms in children with SCD repeatedly were diagnosed as vaso-occlusive crisis rather than biliary colic associated with cholelithiasis. (6)

Cholecystectomy (ie, removal of the gallbladder) is the standard surgical intervention for the diagnosis of cholelithiasis in patients without a chronic illness. In contrast, cholecystectomy has been considered a controversial surgical intervention for children with SCD because these children usually display asymptomatic cholelithiasis. generally most physicians advocate elective cholecystectomy for children with SCD who display asymptomatic cholelithiasis to interrupt symptoms of acute cholecystitis and the risk of requiring exigency cholecystectomy that may place them at risk of complications. (7) Typically, the indication for undergoing a cholecystectomy is the appearance of symptomatic gallstones in patients, regardless of age. Prophylactic cholecystectomy also is appropriate for asymptomatic individuals who have a chronic illness (eg SCD diabetes) and those who have an increased risk of developing symptomatic gallstones. (8)

Cholelithiasis typically is observ les commonly in healthy children who do not have a chronic illness. Gallstones are observ with greater frequent occurrence among children with SCD. Age and chronic hemolysis are identified as the major factors that increase the risk of formation of black pigment sign gallstones in children with SCD (9) Additionally, increase of pigment gallstones in children with SCD appears to be age at the disposal of and occurs in approximately 15% of children younger than 10 years of age (10) and 50% of children by way of 20 years of age. (11) The incidence of gallstones varies with age across the life span for patients with SCD For instance, the incidence is

* 12% in the two- to four-year-old age arrange (12)

* 9% in the five- to seven-year-old age assemblage (13)

* 43% in the 15- to 18-year-old age cluster (14)

* 83% in populace older than 30 years of age, (15) and

* 60% to 85% in the community age 33 or older. (16)

GALLBLADDER ANATOMY

The gallbladder's shape has been compared to a pear (Figure 2) It is approximately 7 cm protracted and holds 30 mL to 50 mL of bile. (17) Branches of the cystic artery originating from the right hepatic artery provide the gallbladder's offspring supply. The common and right hepatic arteries provide the for the use of all bile duct with its children supply. During cholecystectomy, the triangle of Calot conduce tos as a surgical landmark to identify important forms (18) These structures include the cystic pipe the common hepatic duct, and the inferior border of the liver. The often met with hepatic duct, gallbladder, and universal bile duct function as a storage site for bile and a conduit for bile melt from the liver to the duodenum

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PIGMENT GALLSTONES

Children with chronic hemolytic disorders generally have an increased risk of developing pigment gallstones. (19) Pigment gallstones have been observ in 15% of children with SCD who are younger than 10 years of age. (20) and 50% of children with SCD through 20 years of age. The frequent occurrence of pigment gallstones is higher in this population because of a sustained increase in heme catabolism among children with SCD (21)

Approximately 10% to 20% of gallstones are referr to as pigment stones. These are compos of poorly soluble calcium salts, especially calcium bilirubin, and are dark in color. Pigment stones are categorized as black, brown or mixed pigment gallstones. Black pigment gallstones are small, hard, and spiculated (ie, sharp material substance with needle-like points) and are made of calcium bilirubinate, inorganic salts (eg calcium phosphate), and calcium carbonate. (22) Black pigment stones typically evolve in patients who have a history of excessive secretion of unconjugated bilirubin into bile. (23) Black pigment stones are observ in approximately 14% of children with SCD who are younger than 10 years of age. The percentage increases to 36% among school-age children and adolescents (ie, 10 to 20 years of age) and to 50% by means of age 22. (24)