Cholelithiasis In Children Research Articles

Treatment of Cholelithiasis in Children with Sickle Cell Disease

Medical and surgical advances have improved the treatment of cholelithiasis (ie, gallstones) in children with sickle cell disease (SCD). Children with SCD have an increased risk of developing pigment gallstones that initially may be asymptomatic but that can lead to acute symptoms of cholelithiasis. An elective laparoscopic cholecystectomy procedure is recommended for pediatric patients with SCD to prevent the risk of requiring an emergency cholecystectomy procedure. The primary benefits of this approach include a shorter hospital stay after surgery, decreased postoperative discomfort, decreased risk of complications, and a quicker return to normal activities.

Examination: Treatment of Cholelithiasis in Children with Sickle Cell Disease

Examination: Treatment of Cholelithiasis in Children with Sickle Cell Disease

Influence of bilirubin uridine diphosphate-glucuronosyltransferase 1A promoter polymorphisms on serum bilirubin levels and cholelithiasis in children with sickle cell anemia.

Genetic mutations in the uridine diphosphate (UDP)-glucuronosyltransferase 1A (UGT1A) enzyme promoter have been associated with unconjugated hyperbilirubinemia and Gilbert syndrome. The effects of UGT1A promoter polymorphisms on serum bilirubin levels and symptomatic gallstone formation were studied in a cohort of children with sickle cell anemia (SCA). The UGT1A promoter genotype was deterrmined for 115 consecutive children with SCA. Steady-state laboratory parameters and previous cholecystectomy for symptomatic gallstones were recorded retrospectively, then analyzed according to UGT1A genotype. Children with SCA had a lower frequency of the normal (TA)6 UGT1A promoter allele (0.413) than the abnormal (TA)7 allele (0.461). A previously described shorter (TA)5 allele (frequency 0.074) and longer (TA)8 allele (frequency 0.052) were also observed. Children with the 7/7 UGT1A genotype had a significantly higher mean bilirubin level (5.8 +/- 3.1 mg/dL) than those with the 6/6 (2.4 +/- 0.8 mg/dL) or 6/7 genotype (3.0 +/- 1.1 mg/dL; P < 0.001 by analysis of variance). Patients with the 7/7 genotype were more likely to have previous cholecystectomy (87.5%) than those with the 6/6 (35.7%) or the 6/7 genotype (36.1%; P = 0.002 by chi2). Genetic variation in the UGT1A promoter significantly influences serum bilirubin levels and the development of symptomatic cholelithiasis in children with SCA. The UGT1A promoter polymorphisms represent an important nonglobin genetic modifier of clinical disease expression in SCA.

Lithiase biliaire chez l’enfant drépanocytaire : expérience d’un hôpital pédiatrique parisien

Background. – Gallstones are frequently encountered in sickle cell disease. Their complications are difficult to distinguish from vaso-occlusive abdominal pain and they can sometimes threaten the patient’s life. The aim of this study was to describe our local experience with cholelithiasis in children with sickle cell disease. Patients and methods. – We analyzed the follow-up records and abdominal sonography results of 185 children with sickle cell anemia, aged zero to 18 years, followed up in Trousseau Children’s Hospital (Paris) from 1982 to 1998. Results. – Cholelithiasis was detected in 26 patients. The youngest patient was five years old. Cholelithiasis was discovered because of clinical manifestations in 12 patients. Asymptomatic cholelithiasis patients developed clinical manifestations in 28% cases in a maximum delay of two and a half years after its diagnosis. Laparoscopic cholecystectomy was performed in nine cases and open cholecystectomy in 17 cases. The mean postoperative length of stay was significantly shorter in the group of patients with laparoscopy in comparison with the group with open cholecystectomy. Histologic analysis of the gallbladders noted 85 % of acute or chronic cholecystis. Conclusion. – We suggest that cholelithiasis should be carefully sought in the presence of abdominal manifestations in sickle cell patients. We recommend that annual abdominal sonography be performed in sickle cell patients as early as seven years of age and elective cholecystectomy be performed on patients with cholelithiasis.

Milk of calcium cholelithiasis in children

Milk of calcium bile is uncommon and occurs mainly in the adult population. The authors report on 2 children, each having a distinct clinical history and presentation, and each with milk of calcium bile/calculi possessing differing chemical composition and highly notable gross morphology. J Pediatr Surg 36:644-647. Copyright © 2001 by W.B. Saunders Company.

Management of cholelithiasis in pediatric patients who undergo bone marrow transplantation

The aim of this study was to determine the incidence, risk factors, and proper management for asymptomatic cholelithiasis in children undergoing bone marrow transplantation (BMT). The authors reviewed retrospectively the records of 575 children who underwent bone marrow transplantation at a University bone marrow transplantation unit (BMT) unit between February 1991 and October 1999. Of these patients, 235 underwent abdominal ultrasonography for evaluation of jaundice, sepsis, abdominal pain, or metastasis. To identify risk factors for cholelithiasis, the authors stratified the patients based on their disease and treatment regimen. Finally, the authors analyzed the natural history and management of BMT children with cholelithiasis. The authors identified 20 cases of cholelithiasis (8.5%) in the 235 BMT patients who underwent ultrasonography. Children who underwent BMT to treat bone marrow failure showed a significantly increased risk of cholelithiasis compared with children treated for malignancy (27% v 7.4%; P<.01). Most children (85%) with gallstones did not require surgical intervention. Specifically, 9 (45%) died from their primary disease, 5 (25%) showed sonographic resolution of their gallstones, and 3 (15%) underwent follow-up nonoperatively with persistent cholelithiasis. Three of the 20 patients with gallstones (15%) had signs of acute cholecystitis and underwent surgery. There were no surgical complications or deaths in the operative group. Cholelithiasis occurs at a high incidence in pediatric bone marrow transplant patients. Children undergoing BMT for bone marrow failure are at higher risk of having gallstones than those being treated for malignancy. Finally, these data support a strategy of nonoperative management for asymptomatic cholelithiasis in this highly selected group of patients.

Elective laparoscopic cholecystectomy.

Gallbladder stones are very common in patients with sickle cell disease and are the cause of recurrent abdominal pain. Their management has been highly controversial, especially for children. Nonoperated patients and those treated on an emergency basis have a very high rate of morbidity (>50%). We performed a retrospective review of a series of 29 homozygous SS sickle cell children who underwent laparoscopic cholecystectomy between 1991 and April 1998. Only in one case a conversion was necessary (early in the series). Exploration of the common bile duct was done via intraoperative cholangiography. There were no mortalities. The morbidity rate was 17%; (however, of the five patients concerned, four suffered from hyperthermia for 2 days. All of the children were improved and enjoyed resolution of their abdominal pain. We believe that elective laparoscopic cholecystectomy at the earliest time possible, along with correct perioperative management, is the treatment of choice for cholelithiasis in children with sickle cell disease.

Symptomatic Cholelithiasis in Children: A Hospital-Based Review

Symptomatic Cholelithiasis in Children: A Hospital-Based Review

Laparoscopic Cholecystectomy

Historically, cholelithiasis in children was thought to develop secondary to hemolytic disease. Within the past 20 years, however, its incidence in children without hemolysis is being diagnosed more frequently. Laparoscopic cholecystectomy has become the procedure of choice for gallbladder removal in children. Many of the principles for this technique in children are similar to those in the adult population. Because of the patient's smaller size and more pliable abdominal wall, however, several improvements in technique have been developed. In addition, special precautions are necessary in children because of other unique characteristics. At Children's Hospital, Vanderbilt University Medical Center, 80 infants and children have undergone laparoscopic cholecystectomy since June 1990 without intraoperative or postoperative complications. Modifications in the operative technique used in these patients are detailed.

Sequential endoscopic/laparoscopic management of cholelithiasis and choledocholithiasis in children who have sickle cell disease

Background/Purpose: Cholelithiasis and choledocholithiasis are common complications of sickle cell disease (SCD). With the recent advances in laparoscopic cholecystectomy (LC), which has been used successfully for the management of cholelithiasis in children who have SCD, exclusion of choledocholithiasis before LC is of great importance. Methods: Eighteen children who had SCD, cholelithiasis, and choledocholithiasis were treated at our hospital. Seven were treated with open cholecystectomy (OC) and common bile duct (CBD) exploration, and two were treated with transduodenal sphincteroplasty. The remaining 11 patients underwent endoscopic retrograde cholangiopancreatography (ERCP), sphincterotomy, and stone extraction followed by laparoscopic cholecystectomy (LC). Results: A dilated CBD noted on ultrasound, elevated alkaline phosphatase, elevated total bilirubin of more than 5 mg/dL, history of pancreatitis, either singly or in combination, should raise suspicion of choledocholithiasis, and these patients together with those who have choledocholithiasis detected on ultrasound should undergo ERCP to confirm and extract the stones before LC. Conclusion: This sequential approach of endoscopic sphincterotomy and stone extraction followed by LC is a safe and effective approach for the management of cholelithiasis and choledocholithiasis in children who have SCD.

Immune deficiencies in chronic intestinal pseudo-obstruction.

Chronic intestinal pseudo-obstruction has been associated with urinary disorders, myopathy, and ophthalmoplegia in adults and cholelithiasis in children. We observed a high percentage of total-parenteral-nutrition-dependent patients with pseudo-obstruction and recurrent infections requiring gammaglobulin infusions. All records for 23 children with chronic intestinal pseudo-obstruction (10 females and 13 males, mean age 9.8 y +/- 4.9 y, range 4-24 y) referred for a nutritional evaluation from 1992 to 1995 were reviewed. Chronic intestinal pseudo-obstruction was diagnosed by clinical, radiographic findings and antroduodenal manometry. Intestinal full-thickness biopsies were performed in seven children. Hypogammaglobulinemia was diagnosed in 18 patients (78%): 16 patients had various immunoglobulin deficiencies and 2 had selective antibody deficiency. Intravenous gammaglobulin was administered in 14 patients. Other medical conditions affecting the children are summarized as follows: autonomic dysfunction in 10 patients (43%), recurrent hypoglycemia in 9 (39%), asthma in 9 (39%), cholecystitis in 7 (30%), low serum carnitine level in 6 (26%), urinary dysfunction in 6 (26%), pancreatitis in 5 (22%), behavioral problems in 5 (22%), myopathy in 2 (9%), idiopathic thrombocytopenia in 2 (8%), velopharyngeal insufficiency in 1 (4%), oculocutaneous albinism in 1 (4%), Pierre-Robin syndrome in 1 (4%), and protein C deficiency in 1 (4%). Munchausen syndrome was suspected in two patients. Chronic intestinal pseudo-obstruction appears to be associated with immune deficiencies. It is unclear if the immune deficiencies, intestinal pseudo-obstruction, and the other medical conditions have a common underlying etiology. Repeated infections may be due to impaired immune function in children with chronic intestinal pseudo-obstruction. We recommend screening for immune deficiencies in children with chronic intestinal pseudo-obstruction.

Laparoscopic Cholecystectomy: The Treatment of Choice for Cholelithiasis in Infancy and Childhood

Twelve consecutive laparoscopic cholecystectomies (LC) were performed between January 1994 and October 1996 at King Khalid University Hospital. In all patients the indication for cholecystectomy was symptomatic gallstones. Among the 12 children, six had sickle cell disease. The operating time ranged between 65 and 135 minutes (mean=897plusmn;21.06). There was no major morbidity or mortality. The average duration of postoperative parenteral analgesia (pethidine hydrochloride) required was 0.47+/-0.19 day (ranged between 0.3 and one day). The average postoperative stay was 1.67+/-0.44 days (ranged between 1 and 2.5 days). In conclusion, LC is safe, effective and the preferred approach for cholelithiasis in children, with the advantages of short postoperative analgesia requirement, shorter hospitalization, and therefore, an early return to normal daily activities.

Cholelithiasis in children with sickle cell disease

Abdominal ultrasonography was performed on 305 children with sickle cell disease (SCD) (285 SS and 20 S-beta-thalassemia) to establish the prevalence of cholelithiasis in Saudi children with SCD. Their ages ranged from 1 to 18 years (mean 10.45 years). Gallstones were demonstrated in 60 children, giving a prevalence of 19.7%. An additional 50 patients (16.4%) had only biliary sludge. The youngest patient with gallstones was 3 years old. There was a correlation between the presence of gallstones and increasing age. Patients with gallstones were also found to have higher serum bilirubin levels, but their hemoglobin, hematocrit, reticulocyte count, hemoglobin S, and hemoglobin F levels were not significantly different from those of patients without gallstones.

Laparoscopic cholecystectomy versus open cholecystectomy in children: Which is better?

Twenty-nine consecutive laparoscopic cholecystectomies (LC) performed between April 1992 and December 1993 were compared with 23 consecutive open cholecystectomies (OC) performed between January 1991 and March 1992 with regard to clinical, surgical, and economic factors. Most patients were Caucasian (>70%), and symptomatic nonhemolytic cholelithiasis was the most common indication for cholecystectomy. The introduction of LC did not significantly increase the number of cholecystectomies performed per annum. There is a learning curve to LC: the average length of operative time required during the first year was significantly longer than that of OC and the average time for LC during the second year ( P < .01). By the second year, the average operative time of LC was not significantly different from OC. There was no conversion from LC to OC, and the complication rate was minor in both groups. The postoperative parenteral analgesic requirement for LC was significantly less than OC ( P < .01). The mean length of hospitalization of LC was about three times shorter than that of OC ( P < .01). Although the average operating cost per case of LC was significantly more expensive than OC, LC was significantly cheaper because the period of hospitalization was significantly shortened ( P < .01). In conclusion, LC is the procedure of choice in the treatment of symptomatic cholelithiasis in children.

Impact of laparoscopic cholecystectomy on the management of cholelithiasis in children with sickle cell disease

Children with sickle cell disease, well known to have a high incidence of cholelithiasis, are frequently admitted to the hospital for episodes of abdominal pain. Before the advent of laparoscopy, few children with sickle cell and cholelithiasis underwent cholecystectomy unless absolutely necessary, because of the high morbidity of an open cholecystectomy (OC). We reviewed our records of all children with sickle cell disease and cholelithiasis treated from 1985 to 1992 to investigate the impact of laparoscopic cholecystectomy (LC). During that period, 32 children underwent cholecystectomy: 10 OC and 22 LC (all since December 1990). Before December 1990, all children had either classic biliary tract symptoms or abdominal pain of unknown etiology. However, of the 22 LC children, five had asymptomatic cholelithiasis. Only three of the 32 patients had choledocholithiasis, although 30 of 32 had elevated total bilirubins. Two LC children presented with choledocholithiasis and were initially treated with endoscopic sphincterotomy and stent placement. A standard intraoperative cholangiogram (IOC) through the cystic duct was performed in all OC cases. In 19 of 22 LC cases, an IOC through the gallbladder was performed before any dissection; unsuspected choledocholithiasis was not found, but the IOC did allow visualization of the course of the cystic duct, facilitating its subsequent dissection. Total operative length was comparable between the two groups, but the LC patients' postoperative length of stay was half that of the OC patients (2.1 v 4.6 days). Postoperative complications in the OC group included three children who had severe pain, atelectasis, fever, and hypoxemia (30%). In the LC group, one child experienced ventricular ectopy requiring ICU monitoring, and another had atelectasis and hypoxemia (9%). To date there have been no bile duct complications in the LC group. Thus, LC has proven to be a safe and efficacious technique in this high-risk patient population. Realizing this, our pediatric hematologists now aggressively investigate the biliary tracts of their patients who have sickle cell, and have begun to refer them for surgery before the development of biliary tract symptoms.

Laparoscopic cholecystectomy: treatment of choice for cholelithiasis in children.

Laparoscopic cholecystectomy is rapidly replacing traditional cholecystectomy as the standard treatment for cholelithiasis and cholecystitis in adults. Over a period of 16 months, 14 children with a clinical diagnosis of cholelithiasis, ranging in age from 4 to 15 years (mean 12.2), were treated. All had symptoms of abdominal pain or vomiting; one had jaundice and recurrent cholecystitis. Five children (35%) had associated metabolic or hemolytic diseases. The 14 children were operated on using the laparoscopic cholecystectomy technique. No operation was converted to open cholecystectomy, but two patients required laparotomy: one because of suspected injury to the common duct, and the other because of nonvisualization of the gallbladder during laparoscopy. The mean post-operative hospital stay for the 11 children who underwent only laparoscopic cholecystectomy (one patient also had a simple mastectomy) was 50 hours (range 48-72 hours). All children resumed their normal activities almost immediately after discharge from hospital. No long-term biliary or other complications were seen in any patient throughout an average follow-up period of 6.2 months (range 3-16 months). The benefits of this operation in children are obvious: It is safe, effective, and well tolerated.

Alterations in Bile Following Urinary Intestinal Diversion

In order to determine whether alterations in bile occur which make it more lithogenic when intestinal segments are interposed in the urinary tract, 42 female Sprague-Dawley rats had intestinal and urointestinal manipulations followed by analysis of bile constituents. Times collections were obtained from the common bile duct which were analyzed for bile acids, phospholipids, cholesterol, bilirubin, volume, osmolality, pH, calcium, chloride, sodium, and potassium. Changes in bile constituents were identified following urinary diversion which were specific for the type of diversion and the segment of bowel used, but they were not of sufficient degree to cause significant changes in the lithogenicity of bile. This correlates well with the lack of clinical findings of cholelithiasis in children with urinary intestinal diversion.

Importance of Family History in Adolescent Gallbladder Disease-Reply

In Reply. —Dr Goldenring has raised a legitimate point. We were aware of the association of family history in gallbladder disease; however, due to the fact that our work was based mainly on reviews of medical records, we could not draw any definitive conclusion. Although family history was not mentioned in many of the records, that does not mean that it did not exist. In only one case was there a positive family history for cholelithiasis, and that case was part of the idiopathic group. Further studies are needed to determine whether family history is more associated with cholelithiasis in children than in adults, particularly in the idiopathic group.

AN EXPERIENCE WITH CHOLELITHIASIS IN CHILDREN (2 CASES)

We describe two cases of cholelithiasis in children in our department, together with a review of 56 cases which have been documented for the recent one decade. The diagnosis and ethiology of cholelithiasis in children are also discussed in these 58 cases.

Clinical Study on Cholelithiasis in Children : Report of 7 Cases and Review of 122 Cases Reported Previously in Japan

Clinical Study on Cholelithiasis in Children : Report of 7 Cases and Review of 122 Cases Reported Previously in Japan