Compared to adults, cholelithiasis is rare in the pediatric population and idiopathic in 40% to 65% of cases1. While, several pathologies represent risk factors for pediatric cholelithiasis, in our study, idiopathic cases represented 31.25% of patients. However, we also found the presence of hematologic diseases (31.25%), CF (12.5%), and obesity (18.75%), which are similar to the results of a recent review2. Children with cholelithiasis tend to be asymptomatic in 33% to 40% of cases. The classic manifestation is biliary colic, but complications such as obstructive jaundice, choledocholithiasis, acute cholecystitis, acute pancreatitis, and cholangitis may also occur3. In our study, 18.75% of the patients were asymptomatic, and in the symptomatic patients, the most common manifestation was biliary colic. According to our analyses, patients with the most clinical manifestations and complications were idiopathic. This could be due to the prolonged and asymptomatic presence of the gallstones, which would have increased complications. These findings are aligned with a 2020 study, which concluded that symptomatic cholelithiasis is associated with a higher risk of complications (thus supporting early surgical treatment)6.
Cholelithiasis is clinical diagnosed and confirmed by ultrasound1,2. In our study, an ultrasound was performed on every patient at the time of the diagnosis and before surgery. Among the ultrasound findings, numerous alterations were consistent with the presentation of cholelithiasis in the hematologic and CF groups.
Pediatric cholelithiasis can be treated conservatively with UDCA, which is reserved primarily for asymptomatic patients but has shown limited results in stone dissolution4. For symptomatic patients, the gold standard is LC. Other indications for surgery include conservative treatment failure, choledocholithiasis, and the presence of one or more complications1. The principal issue in cholelithiasis management is the lack of standardized guidelines. Typically, symptomatic patients undergo surgery, but there is a notable gap in guidance regarding the treatment and optimal timing of surgery for asymptomatic patients. While recommendations for elective cholecystectomy are emerging for asymptomatic patients with risk factors, such as hemolytic diseases1 and the presence of cholelithiasis at a very young age3,7, standardized guidelines have not yet been defined, even for patients with risk factors. Moreover, there is no recognized consensus regarding the indications for LC in asymptomatic children without specific risk factors1.
In this study, 68.75% of patients had risk factors for cholelithiasis, and most progressed from being asymptomatic to symptomatic (72.7%). Hematologic disorders represented the largest group (31.25%). Despite the small number of cases, we found that most hematological patients were symptomatic with manifestations such as jaundice and acute cholecystitis. They also had ultrasound abnormalities consistent with cholelithiasis, and their intraoperative complications indicated chronic inflammation. Two asymptomatic hematologic patients in our study underwent elective LC and, aside from the absence of complications, we observed a reduction in the median LoS for these patients (5 days) compared to the hematologic group (6 days). These data are compatible with the idea that patients with hematologic diseases and asymptomatic cholelithiasis could benefit from prophylactic LC to reduce clinical symptoms, intraoperatively difficult situations, complications, and LoS.
Several studies have suggested using preventive cholecystectomy in patients with hematologic disorders. Goodwin et al. found a reduced LoS and fewer surgical complications in patients with sickle cell disease who underwent elective/asymptomatic cholecystectomy than those who underwent emergency cholecystectomy8. Liu and colleagues found that surgical interventions for asymptomatic gallstones in patients with hereditary spherocytosis necessitating splenectomy were linked to a reduced risk of adverse outcomes9. Premawardhena et al. strongly advocated that elective LC should be considered in patients with thalassemia to prevent the onset of serious complications associated with LC in symptomatic patients10.
The benefits of prophylactic LC in hematologic patients may also apply to the CF patients in our study. We observed the presence of ultrasound abnormalities and intraoperative difficulties due to chronic inflammatory conditions, even though LC was well tolerated by all CF patients in our study. Notably, one patient with CF and asymptomatic cholelithiasis who underwent elective surgery had a significant reduction in hospitalization duration (4 days) compared to the median of the CF group (8.5 days). Considering the clinical, ultrasound, and intraoperative findings, prophylactic LC in CF patients with asymptomatic cholelithiasis appears to be a feasible option for preventing complications and reducing hospital stays. Other reasons for considering prophylactic LC include the fact that CF patients may undergo lung transplantation and immunosuppressive therapy, which, in the case of chronic cholelithiasis, would pose an increased risk of cancer and cholecystitis11,12. Cholelithiasis alone also carries a risk of cancerous lesions, especially in association with a very young patient age3,7.Assis and colleagues found an increased risk of gallbladder and bile duct cancer in CF patients13. It has also been observed that lung transplant patients experience a significant incidence of cholecystitis with posttransplant gallstones. Hence, prophylactic treatment could be beneficial12. Similarly, Graham et al. observed that pretransplant cholecystectomy patients experience no morbidity or mortality. In contrast, those who underwent cholecystectomy after transplantation had significantly higher rates of morbidity, mortality, and graft loss14.Finally, several studies have shown patient improvement in lung function and quality of life following a cholecystectomy— after excluding patients with highly compromised lung functions from surgery13,15.
As guidelines are currently lacking for asymptomatic patients, we would like to emphasize that 18.75% of the operated patients in our study were asymptomatic (two hematologic, one CF, one autoimmune, and two obese cases). In these patients, we found ultrasound signs consistent with cholelithiasis. Moreover, during surgery, 33.3% of asymptomatic patients had adhesions, indicating a chronic inflammatory state that could make removal of the bladder more difficult. In addition, unlike symptomatic patients, asymptomatic ones did not experience postoperative complications. While the median LoS was similar between the two groups, we observed LoS reductions in asymptomatic patients with CF and hematologic disorders.
Study limitations
Our study has several limitations, including its retrospective nature and the small cohort of patients. Further prospective research with more patients is needed to establish evidence-based guidelines for treating cholelithiasis in pediatric populations.