Minimally invasive management of pediatric hereditary spherocytosis: severity-stratified biliary risk and indications for simultaneous cholecystectomy and biliary intervention at laparoscopic splenectomy – a single-center retrospective study

Abstract

Background

Hereditary spherocytosis (HS) is an inherited hemolytic anemia in which splenectomy may be indicated in moderate to severe disease. Chronic hemolysis increases the risk of cholelithiasis and biliary complications, but optimal timing of cholecystectomy remains uncertain.

Methods

A retrospective single-center analysis of pediatric patients with moderate to severe HS who underwent laparoscopic splenectomy between January 2017 and January 2024. Evaluated variables included disease severity, demographics, cholelithiasis, biliary manifestations, requirement for endoscopic retrograde cholangiopancreatography (ERCP), operative time, postoperative complications, and length of hospital stay.

Results

Thirteen patients were included (5 male, 8 female; mean age 11.9 ± 4.3 years): 6 with moderate HS and 7 with severe HS. Cholelithiasis was present in 9 patients (69.2%). Major biliary complications (biliary pancreatitis or obstructive jaundice) occurred in 4/7 patients with severe HS (57.1%) compared with 1/6 patients with moderate HS (16.7%); ERCP was required in the same proportions. Mean operative time was 124.7 ± 44.7 min and mean hospital stay was 6.1 ± 1.9 days. No major postoperative surgical complications occurred; one patient developed pleural effusion (Clavien–Dindo grade I). Hemoglobin, reticulocyte count, and bilirubin levels improved after surgery and remained improved during follow-up.

Conclusions

Laparoscopic splenectomy was safe and effective in this small cohort of children with hereditary spherocytosis. These preliminary findings suggest that children with severe HS and cholelithiasis may represent a subgroup in whom the potential role of concomitant or early cholecystectomy warrants prospective evaluation. Because of the small sample size, retrospective design and absence of a direct comparison between prophylactic and delayed cholecystectomy strategies, these findings should not be interpreted as a clinical recommendation. The data are insufficient to support a subgroup-specific conclusion for children with moderate HS.

Background

Hereditary spherocytosis (HS) is a hereditary red blood cell disease characterized by spherical-shaped erythrocytes. This disorder leads to increased splenic destruction (hemolysis) [1]. The prevalence of HS varies across different ethnic and geographic populations. The highest reported incidence is in individuals of Northern European ancestry, where up to 1 in 2,000 people may be affected [2–4]. HS is caused by a mutation in genes which encode erythrocyte membrane proteins: α-spectrin (SPTA1 gene), β-spectrin (SPTB gene), ankyrin (ANK1 gene), protein 4.2 (EPB42 gene) and band 3 (SLC4A1 gene) [5]. HS is typically inherited in an autosomal dominant pattern, although rarer autosomal recessive forms and de novo mutations have been reported [6, 7]. Clinical manifestations of HS vary widely. Some individuals may be asymptomatic, while others can experience severe hemolytic anemia requiring frequent blood transfusions and other medical support to manage their symptoms [1, 4]. Typical symptoms are fatigue, jaundice, and pain in the left hypochondrium [8]. According to the guidelines for the diagnosis and management of HS, splenectomy (SE) should be performed in children with a severe form of HS and considered in those with moderate disease based on spleen size and quality of life [9, 10]. Open SE has largely been replaced by the minimally invasive laparoscopic approach, which offers better postoperative outcomes, including reduced blood loss, shorter hospital stays and faster recovery times [11]. Multiple studies have confirmed the safety and efficacy of the laparoscopic approach in the management of HS [12, 13].

HS significantly increases the risk of gallstone disease due to chronic hemolysis [14]. For the early detection of cholelithiasis, regular abdominal ultrasound examinations are recommended [15]. Concurrent cholecystectomy (CE) is recommended for symptomatic cholelithiasis. If gallstones are an incidental finding without symptoms, the value of CE remains controversial [9]. According to a recent study, SE performed concurrently with CE does not significantly alter postoperative morbidity, length of hospital stay, or readmission rates when compared to SE alone [16]. The epidemiology of pediatric gallstone disease has also shifted in recent decades, from a predominance of hemolysis-related pigment stones toward an increasing proportion of obesity-related cholesterol stones; illustrating this trend, a 20-year single-center pediatric cholecystectomy series reported that hereditary spherocytosis as an indication for cholecystectomy declined from 63.6% to 11.8%, while cholesterol stones rose from 27.3% to 70.6%, alongside a rise in mean BMI [17]. This shift underscores the importance of interpreting hemolysis-driven cholelithiasis in HS separately from the broader contemporary pediatric gallstone population, in which obesity-related cholesterol stones are increasingly represented.

Existing guidelines generally recommend CE for symptomatic gallstones in children with HS undergoing SE, whereas the management of asymptomatic cholelithiasis remains controversial. The natural history of gallstones in the setting of ongoing hemolysis may be more aggressive than is appreciated. This study aimed to determine whether the presence of cholelithiasis predisposes pediatric patients with HS to serious biliary complications requiring invasive biliary intervention (ERCP with CBD stone extraction with possible use of endoscopic sphincterotomy and/or papillary balloon dilatation and/or lithotripsy), and to generate hypotheses regarding the potential role of early or concomitant cholecystectomy in this population, to be tested in future prospective studies.

Methods

We conducted a retrospective review of medical records for children with HS who underwent laparoscopic splenectomy (LS) with or without laparoscopic cholecystectomy (LCE) between January 2017 and January 2024. The need for biliary tract interventions for complications related to cholelithiasis was evaluated. Diagnosis of HS was confirmed through clinical presentation, laboratory tests and genetic analysis.

Inclusion criteria were a confirmed diagnosis of HS, age 5–18 years, and treatment with LS with or without LCE at our institution during the study period. Demographic data, clinical characteristics, length of hospital stay, operative time, type of procedures (including LCE and biliary interventions), perioperative outcomes and long-term follow-up data were collected. To ensure a homogeneous cohort with primary HS, patients with concomitant systemic autoimmune/connective tissue disease (e.g., systemic lupus erythematosus) were excluded. The study cohort comprised all consecutive pediatric patients with hereditary spherocytosis who fulfilled the eligibility criteria at our institution during the study period. No patient meeting the eligibility criteria was excluded because of incomplete perioperative or follow-up data. To reduce information bias, data were extracted from institutional medical records using predefined clinical and perioperative variables. No formal sample size calculation was performed because of the design of the study.

Before surgery, patients underwent routine preoperative evaluation, including a complete blood count and liver function tests. All patients received vaccinations against pneumococcus, Haemophilus influenzae and meningococcus to reduce the potential risk of overwhelming post-splenectomy infection. Thromboprophylaxis was administered perioperatively. For patients with severe anemia, erythrocyte transfusions were administered to optimize their red blood cell parameters before the procedure.

All SE were performed laparoscopically by experienced pediatric surgeons using a standard four-port technique. The patients were positioned in a right lateral decubitus position with the left side elevated. A small infraumbilical incision was made to establish pneumoperitoneum. Three additional ports were placed in the upper abdomen for dissection and mobilization of the spleen. The splenic hilum was divided using an energy device, and the spleen was removed through an enlarged umbilical incision. If concurrent cholecystolithiasis was present, we first performed a conventional LCE, followed by LS. At the end of the procedure, a drain was placed under the left hemidiaphragm to prevent fluid accumulation. If the LCE was also performed at the same time, we placed a drain below the liver.

After the procedure, patients were monitored in the intensive care unit. Careful attention was dedicated to pain management and fluid intake. The drain was removed on postoperative day 2 or day 3. Patients were discharged once they were able to tolerate a regular diet and had satisfactory pain control. During the follow-up period, patients were monitored for any complications (bleeding, infection, bile leakage, thrombosis). Postoperative development and adequacy of the splenectomy were confirmed in collaboration with the hematology department.

Endoscopic biliary interventions, when indicated for choledocholithiasis, obstructive jaundice or biliary pancreatitis, were performed by experienced interventional gastroenterologists via ERCP using standard techniques (endoscopic sphincterotomy with stone extraction, biliary stenting and, where required, mechanical lithotripsy or endoscopic papillary balloon dilatation). A detailed technical evaluation of endoscopic modalities and of biliary-pancreatitis management was beyond the scope of this study; the choice of technique was left to the discretion of the interventional endoscopist.

No missing data were identified for the analyzed perioperative and biliary variables. Quantitative variables are reported as mean ± SD or median (IQR), as appropriate, after assessment of normality using the Shapiro–Wilk test. Between-group comparisons were performed using the Mann–Whitney U test for continuous variables and Fisher’s exact test for categorical variables. Owing to the small sample size, no multivariable adjustment for potential confounding was performed. A two-sided p value < 0.05 was considered statistically significant. This was a hypothesis-generating study; as the cohort comprised all consecutive eligible patients at a single center, no a priori sample-size calculation was performed. Given the small fixed sample, all between-group comparisons should be interpreted with caution and were not powered for confirmatory inference. The primary outcome was the occurrence of a major biliary complication, defined a priori as biliary pancreatitis or obstructive jaundice requiring ERCP. Surgical complications were graded by the Clavien–Dindo classification, with grades ≥ III considered major. Secondary outcomes were operative time, length of hospital stay, conversion to open surgery, postoperative complications, and postoperative hematological improvement.

For comparison with the existing literature, a targeted PubMed search was performed on 13 January 2026 using the keyword combination (“splenectomy”) AND (“cholecystectomy”) AND (“hereditary spherocytosis”) AND (“pediatric”). Studies published more than 10 years before the search date were excluded to ensure clinical relevance. Further exclusion criteria were ineligible age, single case reports, mixed hematological populations without extractable HS-specific surgical data, and incomplete clinical information on biliary comorbidities (cholecystolithiasis, biliary complications, cholecystectomy). Of 10 records identified and screened, 3 comparator studies met all eligibility criteria; these are presented alongside the present (index) study in Table 3. This was a targeted narrative comparison and not a systematic review, and searching a single database is acknowledged as a limitation.

Table 3.

Targeted comparison of recent pediatric hereditary spherocytosis surgical series

Study / Year	Patients (n, HS)	Splenectomy performed (n) / Procedure type	Approach	Cholelithiasis (n)	Biliary intervention	CE / gallbladder intervention performed (n)	Concomitant CE (n)	Operative time (min), mean ± SD	Mean hospital stay (days), mean ± SD	Surgical complications (n)
Ambrož et al., 2026	13	13 / TS	LS	9 (69.2%)	5 ERCP	9 (69.2%)	4 (30.8%)	124.7 ± 44.7	6.1 ± 1.9	1 (pleural effusion, CD-I)
Liu et al., 2023 [29]	52	52 / NR	NR	52 (100%)	NR	14 (26.9%)	NR	NR	NR	NR
Boaro et al., 2023 [30]	42	10 / TS 3 / PS	NR	16 (40.0%)	NR	10	8	NR	NR	NR
Huang et al., 2025 [31]	16	13 / TS	LS mainly; 1 open	16 (100%)	5 ERCP + 1 cholecystostomy	6 CE; 2 cholecystolithotomy	NR	NR	NR	2 (fistula/coagulopathy; pleural effusion)

Complications are reported as described in each source study; grading systems and thresholds for major complications were not uniformly applied across studies

TS Total splenectomy, PS Partial splenectomy, LS Laparoscopic splenectomy, CE Cholecystectomy, NR Not reported, ERCP Endoscopic retrograde cholangiopancreatography, SD Standard deviation, CD Clavien–Dindo classification. 

Results

A total of 13 patients with hereditary spherocytosis underwent LS during the study period (Table 1). The median follow-up duration was 34 months (IQR 24–68; range 2–120 months). No patient was lost to follow-up with respect to the assessed postoperative clinical outcomes. The mean age was 11.9 ± 4.3 years, and 61.5% were female. Among the participants, 6 (46.2%) had moderate HS and 7 (53.8%) had severe HS. Indications for laparoscopic splenectomy reflected HS severity and overall clinical burden, including clinically significant anemia, splenomegaly-related impairment of quality of life, and associated biliary manifestations. Coexisting cholelithiasis, biliary symptoms, or major biliary complications determined the indication and timing of cholecystectomy and biliary intervention.

Table 1.

Baseline, biliary and perioperative characteristics of children undergoing laparoscopic splenectomy for hereditary spherocytosis

Characteristic	Total cohort (n = 13)
Demographics and follow-up
Male sex	5 (38.5%)
Female sex	8 (61.5%)
Age at surgery, years, mean ± SD	11.9 ± 4.3
Follow-up, months, median (IQR; range)	34 (24–68; 2–120)
HS severity
Moderate HS	6 (46.2%)
Severe HS	7 (53.8%)
Biliary manifestations
Symptomatic cholecystolithiasis	4 (30.8%)
Obstructive jaundice	2 (15.4%)
Biliary pancreatitis	3 (23.1%)
ERCP before definitive surgical management	5 (38.5%)
Cholecystectomy overall	9 (69.2%)
Concomitant LCE at the time of LS	4 (30.8%)
Cholecystectomy before planned LS	5 (38.5%)
LS alone without cholecystectomy	4 (30.8%)
Spleen size
Spleen length (cm), mean ± SD	17.5 ± 5.8
Spleen width (cm), mean ± SD	10.5 ± 1.1
Perioperative outcomes
Operative time (min), mean ± SD	124.7 ± 44.7
Drain removal, postoperative day	2.4 ± 0.7
Length of hospital stay, days	6.1 ± 1.9
Conversion to open surgery	0
Major postoperative surgical complications	0

Data are presented as n (%) or mean ± SD, unless otherwise stated. For the purpose of this table, biliary manifestations are reported as mutually exclusive categories; each patient was classified according to the single most severe manifestation in the following hierarchy: biliary pancreatitis > obstructive jaundice > symptomatic cholecystolithiasis. The subcategories therefore sum to the total number of patients with biliary manifestations

ERCP Endoscopic retrograde cholangiopancreatography, HS Hereditary spherocytosis, IQR Interquartile range, LCE Laparoscopic cholecystectomy, LS Laparoscopic splenectomy, SD Standard deviation

Biliary manifestations were present in 9/13 (69.2%) (symptomatic cholecystolithiasis 30.8%, obstructive jaundice 15.4%, and biliary pancreatitis 23.1%). Cholecystectomy was performed in 9/13 (69.2%) patients. 4/13 (30.8%) underwent concomitant laparoscopic cholecystectomy at the time of LS, while 5/13 (38.5%) had cholecystectomy performed before splenectomy. ERCP was performed in 4 patients (57.1%) from the severe HS group and in 1 patient (16.7%) from the moderate HS group. The primary indications for ERCP were biliary pancreatitis (23.1%) and obstructive jaundice (15.4%). Patients with severe HS had a numerically higher incidence of preoperative ERCP (57.1% vs. 16.7%) and biliary pancreatitis (42.9% vs. 0%) compared with moderate HS. Length of stay was numerically longer in severe HS (6.9 ± 1.9 vs. 5.2 ± 1.6 days; p = 0.111), but the difference was not statistically significant, likely due to the small sample size (Table 2). Among the 9 patients with cholelithiasis, all were symptomatic by the time of surgery, and none with gallstones was managed conservatively. Using the clinically relevant denominator of patients with cholelithiasis, major biliary complications requiring ERCP were observed in 4/5 stone-bearing patients with severe HS (80.0%) and in 1/4 stone-bearing patients with moderate HS (25.0%). The 5 cholecystectomies performed before splenectomy were prompted by an acute major biliary complication requiring ERCP (biliary pancreatitis, n = 3; obstructive jaundice, n = 2); the remaining 4 underwent concomitant LCE at splenectomy for symptomatic cholelithiasis without common-bile-duct complication.

Table 2.

Moderate vs severe HS: perioperative characteristics and biliary complications

	Moderate (n = 6)	Severe (n = 7)	p-value
Cholecystolithiasis	4/6 (66.7%)	5/7 (71.4%)	1.000
ERCP	1/6 (16.7%)	4/7 (57.1%)	0.266
Biliary pancreatitis	0/6 (0.0%)	3/7 (42.9%)	0.192
Obstructive jaundice	1/6 (16.7%)	1/7 (14.3%)	1.000
Pancreatitis and/or jaundice	1/6 (16.7%)	4/7 (57.1%)	0.266
LOS (days), mean ± SD	5.2 ± 1.6	6.9 ± 1.9	0.111
Op. time (min), mean ± SD	123.2 ± 59.7	126.0 ± 31.9	1.000

Values are n/N (%) or mean ± SD. Fisher’s exact test was used for categorical variables and the Mann–Whitney U test for continuous variables (two-sided). Given the very small sample size, all between-group comparisons are underpowered; p-values are reported for descriptive purposes only and should be interpreted with caution. 

HS Hereditary spherocytosis, ERCP Endoscopic retrograde cholangiopancreatography, LOS Length of hospital stay, SD Standard deviation

The mean operative time was 124.7 ± 44.7 min. The mean length of hospital stay was 6.1 ± 1.9 days. No patients required conversion to open surgery. No major postoperative surgical complications (Clavien–Dindo ≥ III) occurred; one patient developed a pleural effusion (Clavien–Dindo grade I), a minor complication managed conservatively without sequelae. Hemoglobin, reticulocyte count and bilirubin levels improved after surgery and remained improved during follow-up.

The shortest follow-up in the cohort (2 months) occurred in a patient from the cholecystectomy group whose continued care had been transferred to another institution; no patient was lost to follow-up. Among the patients who underwent LS alone, no new cholecystolithiasis was detected during the available follow-up; follow-up in this subgroup was heterogeneous, and later gallstone formation cannot be excluded.

A targeted PubMed search identified three comparator studies meeting all eligibility criteria (Liu et al. [29], Boaro et al. [30], Huang et al. [31]); these three studies are presented in Table 3 alongside the present cohort, for a total of four rows, to allow direct comparison of results.

Discussion

The diagnosis of HS is based on a combination of clinical history, physical examination and laboratory investigations: presence of spherocytes and reticulocytosis. Over 70% of cases of HS have a positive family history [18]. Most individuals have an enlarged spleen. Bilirubin gallstones are present in approximately half of patients with HS. Jaundice is usually absent in mild disease [19]. Many individuals have normal hemoglobin concentration due to compensation of hemolysis. Tests for confirmation of HS are used to exclude other red blood cell diseases. Confirmatory tests include EMA binding test, osmotic fragility test, acidified glycerol lysis test, cryohemolysis test, ectacytometry, and membrane protein electrophoresis [9]. Genetic testing is useful to estimate the disease development and to find optimal treatment options, including a decision regarding splenectomy [2]. Particular pathogenic variants of red blood cell membrane proteins are associated with the severity of HS [20, 21].

Imaging modalities, such as abdominal ultrasound, CT scan and MRI, play a fundamental role in the management of HS. These imaging techniques can provide valuable insights into the presence and extent of associated complications, including the detection of gallstones and the evaluation of the size and characteristics of the spleen. Abdominal imaging surveillance is recommended for early detection of cholelithiasis in these patients, to allow for further planning of CE.

SE is considered to be a standard treatment for HS with severe anemia, recurrent hemolytic crises, or aplastic crises. In the last few decades, the surgical management of HS has evolved toward adopting minimally invasive laparoscopic techniques. A minimally invasive approach offers several advantages over open splenectomy, including reduced blood loss, shorter hospital stays and faster recovery times [13]. 8–27% of patients with HS also develop cholelithiasis, and concurrent CE is often performed [22, 23]. In our study cohort, cholelithiasis was observed in 9 patients (69.2%). This high incidence of cholelithiasis correlates with the severity of chronic hemolysis [14]. This prevalence substantially exceeds the 8–27% reported in unselected HS cohorts; the difference is attributable to selection, as our surgical cohort was restricted to moderate-to-severe HS, the subgroup with the greatest hemolytic burden, and should therefore not be generalized to all HS.

The laparoscopic approach provides adequate visualization, precise dissection of the splenic hilum and the reduction of hospitalization time and the risk of intraoperative complications. This approach also offers the possibility of simultaneous LCE. When biliary clearance is required for proven choledocholithiasis, laparoscopic common bile duct exploration (LCBDE) offers a single-stage alternative to ERCP, allowing gallbladder and common bile duct stones to be managed during the same operation without papillotomy or radiation exposure; a recent pediatric series reported LCBDE to be feasible and safe, with a shorter hospital stay compared with LCE plus ERCP and a low rate of procedure-related complications [24]. Although LCBDE was not used in our cohort, in which biliary clearance was achieved by ERCP, it has been incorporated as an alternative in the proposed algorithm (Fig. 1). Our findings are consistent with the benefit of LS, with few intraoperative and postoperative complications and improvement of the hematological profile during follow-up.

Fig. 1.

Hereditary spherocytosis surgical management algorithm. The proposed management algorithm is hypothesis-generating, based on a single-center retrospective series of 13 patients, and should not be interpreted as a validated clinical pathway. It does not constitute a treatment recommendation. Color coding distinguishes standard management pathways (blue), complicated biliary disease (red), and individualized decision points requiring further evaluation (yellow). LS = laparoscopic splenectomy; LCE = laparoscopic cholecystectomy; ERCP = endoscopic retrograde cholangiopancreatography; LCBDE = laparoscopic common bile duct exploration

In our study, an association between HS severity and the frequency of biliary complications was observed; given the small sample, this association was not statistically significant and should be interpreted with caution. Patients with severe HS showed a numerically higher requirement for ERCP than those with moderate disease (57.1% vs. 16.7%; p = 0.266). These findings raise the question of whether children with severe HS and established cholelithiasis represent a subgroup in whom early or concomitant cholecystectomy should be evaluated prospectively; our data cannot determine whether truly asymptomatic gallstones warrant a prophylactic approach.

Our cohort comprised symptomatic, surgically referred patients and therefore cannot be directly compared with natural-history cohorts of asymptomatic gallstones. As contextual background only, and not as a direct comparator, serious biliary events in unselected adults with asymptomatic gallstones have been reported at approximately 1–2% per year [25], with a cumulative 10-year complication probability of approximately 3% [26]; however, these estimates are derived from a different population and should not be extrapolated directly to children with HS. In our cohort, major biliary complications requiring ERCP occurred in 4/7 patients with severe HS and 1/6 patients with moderate HS. Among stone-bearing patients, these complications occurred in 4/5 children with severe HS and 1/4 children with moderate HS. Importantly, all patients with cholelithiasis were symptomatic by the time of surgery, and no patient with asymptomatic gallstones was managed conservatively. Our data therefore cannot directly assess the rate or natural history of progression from asymptomatic to symptomatic biliary disease in HS. External data from other chronic hemolytic disorders suggest that a conservative, watch-and-wait approach to gallstones may be associated with clinically relevant biliary events, although this evidence is indirect and cannot be extrapolated directly to pediatric HS: in children with chronic hemolytic anemia, emergency admission occurred in 28% over 32 months [27], and in adults with sickle cell disease, prophylactic laparoscopic cholecystectomy was associated with lower biliary morbidity than delayed surgery [28]. Considered together with the biliary complication burden observed in our surgically referred cohort, these findings support prospective evaluation of whether severe HS with established cholelithiasis identifies a subgroup that may benefit from concomitant or early cholecystectomy. The proposed management algorithm in Fig. 1 is presented as a hypothesis-generating framework rather than a validated clinical pathway.

Reporting across the comparator studies was heterogeneous. Liu et al. [29] studied children with asymptomatic gallstones and an indication for splenectomy; most were managed with stones left in situ, whereas 14/52 underwent gallbladder intervention, without a separately reported delayed-cholecystectomy rate. Boaro et al. [30] performed cholecystectomy in 10/16 stone-bearing patients, usually with splenectomy, but did not report later cholecystectomy rates for retained stones. Huang et al. [31] studied a biliary-obstruction cohort in which all 16 patients were, by definition, symptomatic; biliary obstruction was defined by biliary colic with aggravated jaundice, elevated conjugated bilirubin and imaging evidence of common-bile-duct stones with ductal dilatation. Conservative treatment resolved biliary obstruction in 10/16 patients, whereas ERCP or cholecystostomy was required in 6/16. The main interval-treatment complications followed biliary intervention and were linked to a prolonged interval to definitive surgery. Notably, six children in Huang et al.‘s series underwent splenectomy alone without cholecystectomy after clinical resolution of the obstructing episode and remained free of further biliary colic during a mean follow-up of 3.4 years. This suggests that, in selected post-splenectomy patients, residual gallstones may remain clinically quiescent after an obstructing episode has resolved; however, this should not be equated with the natural history of truly asymptomatic, never-obstructed stones, which neither Huang et al.‘s cohort nor our cohort can address. Huang et al.‘s findings provide an important counterpoint to our interpretation. In their obstruction-only cohort, splenectomy alone appeared sufficient in selected patients once biliary obstruction had resolved, without routine concomitant cholecystectomy. In addition, biliary obstruction was observed in both non-severe and severe HS, suggesting that obstruction is not confined to severe disease. These findings caution against a simple severity-based model of biliary risk and represent an important counterpoint to our hypothesis that HS severity may track with biliary complications. However, the two cohorts differ substantially: our series was a surgically referred laparoscopic-splenectomy cohort of children with moderate-to-severe HS, in which 9/13 had established cholelithiasis and varying biliary manifestations, whereas Huang et al. focused specifically on children presenting with biliary obstruction. In addition, the management strategies differed among stone-bearing patients, with prior or concomitant cholecystectomy in our cohort versus splenectomy alone after obstruction resolution in selected Huang et al. patients. Our interpretation should therefore be regarded as one hypothesis among several plausible management strategies, and only prospective comparative data can determine which strategy, if any, improves clinical outcomes. Overall, complication severity, symptomatic status and delayed-cholecystectomy rates are inconsistently reported across these series, underscoring the value of complete biliary datasets such as ours.

This study has several limitations. Its small sample size and retrospective design limit statistical power, so all subgroup comparisons should be interpreted with appropriate caution, and the higher incidence of biliary manifestations than in some other pediatric series may partly reflect referral-based selection. Because no patient with asymptomatic cholelithiasis was followed conservatively, this study cannot evaluate the natural history or rate of progression of asymptomatic gallstones in children with HS. The findings should therefore not be used to infer that asymptomatic gallstones in HS necessarily progress to symptomatic or complicated biliary disease. The potential role of concomitant or early cholecystectomy requires prospective validation before it can inform clinical practice. Follow-up was also heterogeneous in duration. Nonetheless, the cohort provides a complete biliary dataset with no patient lost to follow-up, and the findings warrant confirmation in larger multicenter studies. As the cohort comprised only children already referred for and undergoing splenectomy, selection occurred upstream at the point of hematological referral, so the resulting surgical referral population may not represent the full spectrum of pediatric HS.

Conclusions

Laparoscopic splenectomy was safe and effective in this cohort of children with hereditary spherocytosis and was associated with sustained hematological improvement. Cholelithiasis and major biliary complications requiring ERCP were frequent among surgically referred children, particularly those with severe HS. These observations raise the possibility that severe HS with established cholelithiasis identifies a subgroup for whom the potential benefit of concomitant or early cholecystectomy could be evaluated prospectively. Given the small sample size and retrospective, non-comparative design, the potential benefit of such a strategy remains untested and should not be interpreted as a clinical recommendation. The data are insufficient to support any subgroup-specific conclusion for children with moderate HS. Larger multicenter studies are needed to evaluate biliary outcomes across HS severity subgroups and to determine whether either a prophylactic strategy in truly asymptomatic gallstone carriers, or an early/concomitant strategy in symptomatic patients, improves clinical outcomes.

Abbreviations

CBD

common bile duct

CD

Clavien–Dindo classification

CE

cholecystectomy

ERCP

endoscopic retrograde cholangiopancreatography

HS

hereditary spherocytosis

IQR

interquartile range

LCBDE

laparoscopic common bile duct exploration

LCE

laparoscopic cholecystectomy

LOS

length of hospital stay

LS

laparoscopic splenectomy

NR

not reported

PS

partial splenectomy

SD

standard deviation

SE

splenectomy

TS

total splenectomy

Authors’ contributions

R.A. served as an attending physician, reviewed the literature, and contributed to the manuscript. P.B. and M.S. reviewed the literature and contributed to the interpretation of the findings. J.K. performed the surgical procedures and, together with D.K., M.L., and D.P., supervised the study. B.L. and Z.N. served as attending physicians. All authors read and approved the final manuscript.

Funding

Supported by the Ministry of Health, Czech Republic – conceptual development of research organization (FNOl, 00098892).

Data availability

The datasets generated and/or analysed during the current study are not publicly available due to patient privacy restrictions but are available from the corresponding author on reasonable request.

Declarations

Ethics approval and consent to participate

This study was approved by the Ethics Committee of the University Hospital and Faculty of Medicine and Dentistry of Palacký University Olomouc (Zdravotníků 248/7, 779 00 Olomouc, Czech Republic; reference 51/26; approved 9 March 2026). The study was conducted in accordance with the principles of the Declaration of Helsinki. The requirement for informed consent was waived by the same Ethics Committee owing to the retrospective design of the study.

Consent for publication

Not applicable.

Competing interests

The authors declare no competing interests.