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A 17-year-old girl with cholelithiasis associated with mitral valve replacement four years earlier is presented. The potential for the development of cholelithiasis after cardiac valve replacement in children and adolescents is emphasized.
The incidence of cholelithiasis and surgical risk of cholecystectomy in patients with sickle cell anemia is reviewed. Records of all patients with sickle cell disease currently attending the Medical University of South Carolina Hematology Clinic were reviewed. An oral cholecystogram has been part of the initial evaluation for the past year. The records of 16 patients having cholecystectomy from 1968 to 1977 were reviewed with regard to diagnostic studies, preoperative preparation, morbidity, and mortality. When the status of the gallbladder is known, the incidence of cholelithiasis is 67%. The morbidity of elective cholecystectomy was high (37%); there was one death. Cholecystectomy is not recommended for the discovery of asymptomatic gallstones because of the relatively poor surgical risk and short life expectancy of these patients.
Sixty-nine patients were evaluated prospectively by sonography and history to determine the presence of cholelithiasis. Sixteen patients (23%) had a positive diagnosis. A control group was also prospectively evaluated. We have determined that patients above age 50 with a permanent ileostomy are at statistically significant risk of having cholelithiasis, and their risk is greater than that of a control group matched for age and sex. Radiologists should recognize this association and carefully evaluate the gallbladder of any patient with a permanent ileostomy who has abdominal pain.
OBJECTIVES: Symptomatic cholelithiasis is a common disease in the general population with an increased prevalence in patients with cirrhosis. While cholecystectomy is the procedure of choice for the treatment of symptomatic cholelithiasis, cirrhotics have an increased risk of complications associated with this therapy. We have found that placement of an endoscopic gallbladder stent is an alternative, less invasive treatment for cirrhotic patients with symptomatic gallbladder disease and describe our experience here. METHODS: A retrospective medical record review of 23 patients with cirrhosis who underwent endoscopic retrograde cholangiography with gallbladder stent placement for symptomatic gallbladder disease from July 1994 to August 2004. RESULTS: Indications for stent placement included recurrent biliary colic (56.5%), acute calculous cholecystitis (39%), acalculous cholecystitis (8.6%), and gallstone pancreatitis (8.6%). All patients experienced resolution of their symptoms following stent placement. Twenty patients (87%) were asymptomatic from 5 days to 3 years post-procedure until transplantation, death, or end of study period. Nine patients (39%) underwent liver transplantation, 5 days to 34 months after the procedure. Eleven patients are well, with ten patients awaiting liver transplantation. Three patients developed late complications and were treated successfully with antibiotics. CONCLUSION: Endoscopic stenting of the gallbladder may be a potential treatment for symptomatic gallbladder disease in patients with cirrhosis awaiting liver transplantation, considered to be high-risk for cholecystectomy.
An unusual case of cholelithiasis in an 18-month-old boy with cervico-oculo-acoustic (Wildervanck's) syndrome is presented. Our patient had Duane's retraction syndrome, Klippel-Feil anomaly and congenital deafness. To our knowledge this is the first case in which a probable association between cholelithiasis and Wildervanck's syndrome has been recorded. On the other hand, the presence of mutual malformations and anomalies such as scoliosis, ventricular septal defect, ectopic kidney, hydrocephalus, hypoplastic thumb and growth retardation seems to suggest that Wildervanck's syndrome is a clinical variant of Klippel-Feil sequence.
Down syndrome is a chromosomal disorder most often observed in the newborn period. Various facial, limb and internal abnormalities are found in this disorder but cholelithiasis in infancy has been described in only one report. We report two infants with Down syndrome associated with cholelithiasis. Except for polycythemia and indirect hyperbilirubinemia, no hemolytic process or biochemical abnormalities were evident in both patients. We believe that the cause of gallstones in our cases may have been polycythemia in the newborn period. To our knowledge this is only the second report of gallstones in infancy in Down syndrome.
A four-year-old female Japanese akita was admitted with icterus, ascites and chronically elevated serum bilirubin and liver enzymes. Abdominal ultrasonography revealed a diffusely thickened, hyperechoic gallbladder wall with a focal defect, hepatic lymphadenopathy and a large volume of anechoic fluid within the peritoneal space. Diagnosis of biliary tract rupture with bile peritonitis was based on the findings of bile and suppurative exudate in peritoneal aspirates. A perforated gallbladder and cholelithiasis were found on exploratory celiotomy, while histopathology revealed chronic suppurative cholecystitis. The dog recovered uneventfully after cholecystectomy. Although rare, the triad of cholelithiasis, cholecystitis and gallbladder perforation should be considered after detection of one of these conditions.
Cholelithiasis is considered uncommon in infancy, childhood, and adolescence. We performed a prospective, controlled study showing that children with Down's syndrome have a significantly higher prevalence of cholelithiasis (4.7%) compared with controls (0.2%). Clinicians should be aware of the risk of gallstones in children with Down's syndrome.
The present necropsy study shows an oriental pattern of gallstone disease in Singapore, namely, a relatively low overall frequency, an equal involvement of both sexes, a high proportion of pigment stones, and the common occurrence of choledocholithiasis associated with pyogenic cholangitis. There is a close association between opium addiction and cholelithiasis in the adult male Chinese in Singapore, and the long-term abuse of opium may be an important aetiological factor in the pathogenesis of oriental cholelithiasis.
Although, in suitable patients, oral chenodeoxycholic acid (CDCA) dissolves gallstones, the results of recent animal studies suggest that it might be hepatotoxic. Liver function was therefore studied in patients with gallstones before and during treatment with CDCA and liver biopsies were carried out both in patients with cholelithiasis given bile acid therapy and in those who had been given no medical treatment. In 25 patients treated with 0.5-1.5 g CDCA/day (7-20 mg kg body weight(-1) day(-1)) there was no significant change in serum bilirubin, albumin, globulin, transaminase, isocitric dehydrogenase, alkaline phosphatase, and gamma glutamyl transpeptidase levels before and at monthly intervals during six months' treatment. The kinetics of bromsulphthalein (BSP) clearance and its apparent transport maximum were not significantly changed during CDCA therapy. The mean fasting serum bile acid concentrations of 18.0 +/- SEM 1.2 mumoles/litre before and 20.0 +/- 3.5 mumoles/litre during treatment were both significantly greater than control values. Liver histology was not appreciably different in 11 patients treated with CDCA from that in eight patients with untreated cholelithiasis and in three patients who had received CDCA three to four months before biopsy. These results suggest that in doses of 0.5 to 1.5 g/day CDCA is not hepatotoxic in man.
Proliferation of the epithelial cells of the gall bladder during the initial stages of experimental cholelithiasis has been studied by autoradiography. Rabbits taking a diet with 1% dihydrocholesterol added showed a rise in labelling index from 1.5%-19% by three days. Mice taking a 1% cholesterol 0.5% cholic acid diet showed a similar rise from less than 1% to 15-21% at two and five days. Guinea-pigs receiving lincomycin showed a rise from less than 1% to 6% after 24 hours. These changes, which appear well before the appearance of gall stones, together with previous reports of enhanced mucous secretion during lithogenesis, make it clear that in these laboratory models of cholelithiasis the gall bladder is abnormal well before stones form.
The role of biliary deoxycholate as an endogenous colon carcinogen and the possible association between cholelithiasis and/or cholecystectomy and the subsequent development of large bowel cancer is unclear. This paper describes biliary bile acids analysis performed on 13 patients undergoing cholecystectomy for gall stones, 10 patients undergoing colonic resection for colon cancer, and eight control patients. For all 31 patients the total bile acids concentration was highly variable (8.3 mg/ml-106.5 mg/ml). The median ratio of primary to secondary bile acids was 2.7:1. The biliary bile acid ratios were similar in both control patients (3.7:1) and those with colon cancer (3.1:1), whereas patients with gall stones had significantly higher secondary bile acid levels in their biliary bile (ratio 1.9:1, p = less than 0.05). This result indicates that raised biliary deoxycholate concentrations are not present in patients with colon cancer and are therefore unlikely to be a major predisposing factor in the aetiology of this disease. It is unlikely that cholelithiasis and/or cholecystectomy predispose to the subsequent development of colon tumours.
The triglyceride, cholesterol, and phospholipid contents of heart papillary muscle were measured in groups of obviously healthy and diseased females and males on whom either routine or forensic necropsies were performed. In healthy men the triglyceride content was 1.77 +/- 1.30 mg/g of wet weight and in women 1.25 +/- 0.48 mg/g wet weight. The corresponding values for cholesterol were 1.07 +/- 0.24 mg/g and 1.21 +/- 0.22 mg/g and those for phospholipids 17.70 +/- 5.15 mg/g and 19.65 +/- 10.21 mg/g. The differences between the sexes were not significant. The hypertensive or cardiac hypertrophy group had about the same or slightly lower means for lipid content. In the cholelithiasis group, women had significantly high triglyceride values (3.38 +/- 2.36 mg/g). The cholesterol values were not significantly elevated in either men or women. In the diabetic group, triglycerides were significantly increased both in men (mean 8.12 +/- 0.54 mg/g) and in women (6.85 +/- 5.66 mg/g). The cholesterol mean values were also high in both sexes, but the rise was not significant because of the great variation. In the coronary atheroma group, both male and female hospital cases had high triglyceride contents (mean 4.48 +/- 4.25 mg/g and 3.65 +/- 3.94 mg/g) whereas the forensic cases had only slightly elevated or normal values. Cholesterol assays paralleled the triglyceride ones, but phospholipids showed an inverse trend. The results showed that the lipid content of papillary muscle was increased in diseases where disturbances of lipid metabolism are evident, as in diabetes and cholelithiasis. In coronary atheroma only those cases with advanced obstruction of the arteries were associated with abnormal values of papillary lipids. No increase of the lipid content with age alone was found, nor was there any correlation with obesity.
Review of 367 cases of cholelithiasis in children from the literature and our experience showed that 81% of these patients has no evidence of hemolytic anemia. Oral cholecystograms were diagnostic of cholelithiasis in approximately 70% of these cases. Plain film studies were found to be of diagnostic value as a high percentage of gallstones were visible on plain radiographs of the abdomen.
Cholecystitis and cholelithiasis should be considered in the differential diagnosis of abdominal pain in children. Ultrasonography should be a primary screening test because it allows rapid evaluation of the gallbladder and identifies other possible causes of the symptomatology. The presence of a thickened gallbladder wall, cholelithiasis, or a nonvisualized gallbladder indicates gallbladder disease. Since acalculous cholecystitis is more common in children than it is in adults, it is particularly important to examine the gallbladder wall carefully for evidence of thickening.
Five cases of carcinoma of the gallbladder associated with nonsurgical treatment of cholelithiasis are presented in view of the recent interest in gallbladder-preserving procedures. Three patients developed gallbladder cancer that was diagnosed 4, 11, and 72 years, respectively, after cholecystostomy. One other patient had gallbladder carcinoma diagnosed 7 months after extracorporeal shock wave lithotripsy for common bile duct stones. The tumor was probably present but not identified at the time of lithotripsy. The last patient had undiagnosed gallbladder malignancy at the time of cholecystostomy for treatment of acute calculous cholecystitis. Gallbladder carcinoma has been reported in 1% of patients undergoing cholecystectomy, and the risk of carcinoma developing in patients with asymptomatic cholelithiasis has been estimated at less than 1%. These figures should not diminish the role of gallbladder-preserving treatments in elderly and selected high-risk patients for whom cholecystectomy may be hazardous. However, such patients must undergo a thorough imaging assessment both before and after treatment to ensure that a gallbladder carcinoma is not overlooked.
The glucose-6-phosphate dehydrogenase (G6PD) enzyme activity was determined in 299 Northern Sardinian patients with cholelithiasis. Sixteen (12.80%) of the 125 male patients studied were G6PD deficient; 33 (18.96%) of the 174 females were heterozygous and 1 (0.57%) homozygous. Thus, the prevalence of G6PD deficiency in male subjects with cholelithiasis is about 35% higher (p less than 0.02) than that of a normal male control group (7.29%). As regards female patients, the incidence of the GdMed allele was also significantly different (p less than 0.05). Thus, G6PD-deficient subjects may have a predisposition to develop gallstones, even in the absence of clinical signs of chronic hemolysis.
A case of an infant with very rare concurrence of pseudohypoaldosteronism and cholelithiasis is described. Aldosterone concentration was extremely high (64.6 nmol/l; normal 0.03-4.4 nmol/l) and fludrocortisone did not have favorable effects on hyperkalemia (7.4 mEq/l) and hyponatremia (124 mEq/l). A gallstone was found using ultrasonography at age 6 months, and it was extirpated surgically. The combination has not been reported previously. The study supports the previous hypothesis that cholelithiasis is found more often in infants with dehydration or electrolyte derangement.