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Treatment of cholelithiasis in children with sickle cell disease - Home Study Program

AORN Journal, June, 2003 by Verna Hendricks-Ferguson, Martha A. Nelson

Sickle cell disease (SCD) is one of the most 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 by 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 recurrent abdominal pain. (3) Evaluation criteria used to confirm the diagnosis of cholelithiasis include radiological evaluation using biliary scintigram and ultrasound and laboratory results that indicate elevations in serum

[FIGURE 1 OMITTED]

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* bilirubin,

* alkaline phosphatase,

* hepatic enzyme levels, and

* white blood cell count. (4)

An estimated 50% of patients in sickle cell crisis (ie, pain crisis) experience acute abdominal pain, which often is difficult to differentiate from acute cholecystitis. (5) One group of researchers noted that acute abdominal pain symptoms in children with SCD often 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. Currently, most physicians advocate elective cholecystectomy for children with SCD who display asymptomatic cholelithiasis to prevent symptoms of acute cholecystitis and the risk of requiring emergency cholecystectomy that may place them at risk of complications. (7) Typically, the indication for undergoing a cholecystectomy is the presence 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 observed less frequently in healthy children who do not have a chronic illness. Gallstones are observed with greater frequency among children with SCD. Age and chronic hemolysis are identified as the major factors that increase the risk of formation of black pigment type gallstones in children with SCD. (9) Additionally, development of pigment gallstones in children with SCD appears to be age dependent and occurs in approximately 15% of children younger than 10 years of age (10) and 50% of children by 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 group, (12)

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

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

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

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

GALLBLADDER ANATOMY

The gallbladder's shape has been compared to a pear (Figure 2). It is approximately 7 cm long 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 blood supply. The common and right hepatic arteries provide the common bile duct with its blood supply. During cholecystectomy, the triangle of Calot serves as a surgical landmark to identify important structures. (18) These structures include the cystic duct, the common hepatic duct, and the inferior border of the liver. The common hepatic duct, gallbladder, and common bile duct function as a storage site for bile and a conduit for bile flow from the liver to the duodenum.

[FIGURE 2 OMITTED]

PIGMENT GALLSTONES

Children with chronic hemolytic disorders generally have an increased risk of developing pigment gallstones. (19) Pigment gallstones have been observed in 15% of children with SCD who are younger than 10 years of age. (20) and 50% of children with SCD by 20 years of age. The frequency 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 referred to as pigment stones. These are composed 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 body with needle-like points) and are made of calcium bilirubinate, inorganic salts (eg, calcium phosphate), and calcium carbonate. (22) Black pigment stones typically develop in patients who have a history of excessive secretion of unconjugated bilirubin into bile. (23) Black pigment stones are observed 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 age 22. (24)

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Treatment of cholelithiasis in children with sickle cell disease - Home Study Program

AORN Journal, June, 2003 by Verna Hendricks-Ferguson, Martha A. Nelson

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