Ten-year experience with pediatric splenic Hydatidosis: Clinical profiles, surgical outcomes, and prognostic indicators

Abstract

Hydatidosis, caused by Echinococcus granulosus, poses significant public health challenges, particularly in endemic regions. While hepatic and pulmonary involvement are common, splenic hydatidosis in pediatric populations is rare and frequently underreported. In this retrospective observational study, we present a decade-long single-center experience (2014–2024) in pediatric splenic hydatidosis, detailing demographic profiles, clinical presentations, imaging characteristics, and surgical outcomes. Ten patients (mean age: 11.3 years) were evaluated, with abdominal pain as the predominant symptom and cyst sizes ranging from 40 to 220 mm (WHO-IWGE classification: 1CE–CE3b). Total splenectomy was performed in nine cases, with one patient undergoing partial splenectomy. Preoperative albendazole was administered to eight patients, and postoperative albendazole to nine patients; long-term therapy (2–8 months) was provided in seven cases. Prophylactic measures, including pneumococcal vaccination and postoperative antibiotic prophylaxis, were implemented, resulting in no cases of overwhelming post-splenectomy infection or hydatid recurrence during a mean follow-up of approximately four years. These findings underscore the importance of early diagnosis, appropriate surgical intervention, and diligent long-term follow-up, as well as the need for strengthened public health initiatives to reduce the disease burden in endemic regions.

Highlights

• •All pediatric splenic hydatidosis cases presented with abdominal pain and imaging-confirmed cysts.
• •Multiorgan involvement (3/10) primarily affected liver and lungs alongside spleen.
• •Presurgical albendazole failed to prevent relapse (n = 3), including cyst rupture requiring emergency intervention.
• •Total splenectomy performed in 90 % (9/10); partial resection achieved in one case due to favorable anatomy.
• •No mortality, recurrence, or OPSI observed during mean 4-year follow-up, confirming management efficacy.

1. Introduction

Hydatidosis, caused by Echinococcus granulosus, is a zoonotic disease with significant public health implications, particularly in endemic regions such as the Mediterranean, Middle East, and parts of South America (Moro and Schantz, 2009). The chronic disease course, potential for severe complications, and socioeconomic burden, especially in low-resource settings, underscore the need for effective control measures (Bold et al., 2024; Craig et al., 2007). While hydatidosis affects all age groups, pediatric patients are particularly vulnerable due to increased exposure to contaminated environments and the often asymptomatic progression of cysts until they reach considerable size (Torgerson et al., 2010). Reported pediatric prevalence varies from 1 % to 10 % in endemic areas, highlighting the importance of early detection and targeted management strategies (Torgerson et al., 2010).

The liver is the most frequently affected organ (50–70 % of cases), followed by the lungs (20–30 %), whereas splenic involvement is rare, occurring in only 2–6 % of cases (Moro and Schantz, 2009). Splenic hydatidosis presents unique diagnostic and therapeutic challenges due to its nonspecific symptoms and potential complications, including cyst rupture, secondary infection, and hypersensitivity reactions (Boam et al., 2017; Luu et al., 2019; Prousalidis et al., 2012). The rarity of the condition often leads to delayed diagnosis, as its symptoms overlap with other abdominal pathologies, complicating clinical decision-making (Khalili et al., 2023; Rasheed et al., 2013).

Surgical intervention remains the primary treatment, with total splenectomy traditionally regarded as the standard approach due to the risk of recurrence and complications associated with cyst rupture (Belli et al., 2014; Brunetti et al., 2010; Prousalidis et al., 2012). However, there is increasing interest in spleen-preserving techniques, particularly in pediatric cases, to mitigate the long-term risks of asplenia, such as overwhelming post-splenectomy infection (OPSI) (Luu et al., 2019). Pharmacological therapy with albendazole is commonly used as an adjunct to surgery to reduce cyst viability and recurrence risk. However, optimal dosing regimens in children remain inadequately studied, and the long-term efficacy of antiparasitic therapy warrants further investigation (Arif et al., 2011; Zavoikin et al., 2021).

This study presents a decade-long single-center experience with pediatric splenic hydatidosis, offering comprehensive data on demographic characteristics, clinical presentation, radiologic findings, and surgical outcomes. By addressing existing knowledge gaps, our findings contribute to the evolving literature on pediatric hydatidosis and provide insights that may aid in refining diagnostic and therapeutic strategies for this rare but clinically significant condition.

2. Methods

This retrospective observational study was conducted at Namazi Educational Hospital in Shiraz, Iran, from 2014 to 2024. Pediatric patients (aged 0–18 years) diagnosed with splenic hydatidosis who underwent either total or partial splenectomy were included. Patients were excluded if they were older than 18 years, had incomplete follow-up data, or underwent surgery at a different institution. Written informed consent was obtained from all participants or their legal guardians. The study protocol was approved by the institutional ethics committee (Reference ID: IR.SUMS.MED.REC.1403.432). Patient confidentiality was rigorously maintained, and all data were anonymized prior to analysis.

Clinical data were extracted from patient medical records and included demographic characteristics, clinical presentation, imaging findings, surgical interventions, and postoperative outcomes. Data collection was supplemented with telephone-based follow-up assessments. Descriptive statistics were applied: continuous variables were reported as means with standard deviations (SD) or medians, and categorical variables were presented as absolute counts and frequencies. The study was conducted in accordance with the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines (von Elm et al., 2008).

2.1. Preoperative protocols

Preoperative management included albendazole therapy administered in accordance with pediatric dosing recommendations (10–15 mg/kg/day, not exceeding 800 mg/day, divided into two doses). Treatment duration ranged from one week to several months, based on clinical judgment and cyst characteristics, to reduce cyst viability and minimize intraoperative spillage.

The distinction between isolated and non-isolated splenic hydatidosis was based on imaging findings: isolated cases were defined as cystic lesions confined solely to the spleen, whereas non-isolated cases involved additional cysts in the liver, lungs, or other organs.

The American Society of Anesthesiologists (ASA) physical status classification was used to assess patients' preoperative health status (Foley et al., 2021; Hendrix and Garmon, 2025). Additionally, the Systemic Inflammation Index (SII) was calculated for each patient, given its potential relevance in evaluating inflammatory responses in parasitic infections (Ren et al., 2021; Xiaobin et al., 2022).

2.2. Imaging protocol

A standardized imaging protocol was employed for the diagnosis and management of pediatric splenic hydatidosis. Ultrasound served as the first-line imaging modality due to its radiation-free nature, high sensitivity (90–95 %), ability to delineate key cystic features, and cost-effectiveness, particularly in resource-limited settings (Brunetti et al., 2018; Garg et al., 2016; Rasheed et al., 2013). Computed tomography (CT) was reserved for surgical planning, clarification of indeterminate ultrasound findings, detection of complications, and assessment of extrasplenic involvement (Abbasi et al., 2021; Rasheed et al., 2013).

Cysts were classified according to the World Health Organization Informal Working Group on Echinococcosis (WHO-IWGE) classification system, categorizing lesions as active (1CE, 2CE), transitional (CE3A, CE3B), or inactive (4CE, 5CE), based on ultrasound features (Tamarozzi, 2024; Turgut et al., 2008).

2.3. Follow-up assessments

Postoperative follow-up included clinical evaluation, laboratory testing, and imaging surveillance. Asymptomatic patients underwent routine ultrasound at regular intervals (typically at 3, 6, and 12 months post-surgery) to monitor for recurrence, followed by annual assessments. CT scans were performed at extended intervals or when ultrasound findings were inconclusive (Sen et al., 2019). In symptomatic patients presenting with new-onset symptoms such as abdominal pain or allergic reactions, serological (ELISA, IHA) and inflammatory marker (CRP, ESR) testing was conducted, followed by imaging as needed (Prousalidis et al., 2012; Tercan et al., 2020). This structured approach facilitated early detection of recurrence and timely intervention.

Long-term albendazole therapy (ranging from 2 to 8 months) was continued in patients deemed at high risk of recurrence. In addition, surveillance for Overwhelming Post-Splenectomy Infection (OPSI) was carried out through regular clinical evaluations and implementation of prophylactic strategies, including pneumococcal vaccination and, in selected cases, penicillin prophylaxis, in line with established guidelines (Jones et al., 2010; Luu et al., 2019).

3. Results

3.1. Patient characteristics and initial clinical presentation

This study included ten pediatric patients diagnosed with hydatidosis, comprising six females and four males, aged between 6.25 and 18 years. Patient heights ranged from 100 to 173 cm, and body weights varied from 16.5 to 72 kg. Based on BMI classification, three patients were underweight (Patients 1, 8, and 9), four had normal weight (Patients 3, 4, 6, and 7), and three were overweight (Patients 2, 5, and 10). Six patients resided in urban areas, while four were from rural settings; all had access to potable water. The mean age was 11.27 ± 4.21 years, with a median of 10.5 years. The mean body weight was 36.09 ± 16.59 kg, and the mean height was 137.18 ± 26.80 cm. The average BMI was 17.0 ± 6.20, with a median of 18.8. Comprehensive demographic and BMI classification data are presented in Table 1.

Table 1.

Demographic, clinical, and epidemiological characteristics of 10 pediatric patients diagnosed with splenic hydatidosis. Data include age, weight, body mass index (BMI), sex, residence, access to sanitary water, symptom duration, and presenting clinical features. All patients resided in urban or rural settings with reported sanitary water access; abdominal pain was universal, with variable comorbidities such as nausea, vomiting, and constitutional symptoms. Symptom duration ranged from 4 to 210 days.

Patient No.	Age (Years)	Weight (kg)	BMI	Sex	BMI Category	Residence	Sanitary Water Access	Symptom Duration (Days)	Clinical Symptoms
1	6.25	16.5	8.0	M	Underweight	Urban	Yes	4	Abdominal pain
2	8.5	30	24.8	M	Overweight	Urban	Yes	5	Abdominal pain, nausea, vomiting, productive cough
3	15	53.1	18.8	F	Normal	Urban	Yes	90	Abdominal pain, amenorrhea
4	16	58.1	19.4	F	Normal	Urban	Yes	90	Abdominal pain, nausea, vomiting
5	10	48.3	25.2	M	Overweight	Urban	Yes	7	Abdominal pain, nausea, vomiting
6	7	24.6	15.0	F	Normal	Urban	Yes	16	Abdominal pain
7	14	50	19.5	F	Normal	Urban	Yes	180	Abdominal pain, nausea, vomiting, episodic fever, hepatosplenomegaly, anorexia
8	18	45	15.9	F	Underweight	Urban	Yes	18	Abdominal pain, episodic fever, anorexia
9	11	20	10.2	F	Underweight	Rural	Yes	210	Abdominal pain, nausea, anorexia
10	7	22.3	22.3	F	Overweight	Rural	Yes	180	Abdominal pain, constipation, anorexia

Mean Age: 11.27 years (SD ± 4.21); Median Age: 10.5 years; Mean BMI: 17.0 (SD ± 6.20); Median BMI: 18.8.

The time from symptom onset to diagnosis ranged from 4 to 210 days, with a median duration of 52 days. All patients presented with abdominal pain as the primary complaint. Additional symptoms included nausea and vomiting in five patients (Patients 2, 4, 5, 7, and 9), anorexia in four (Patients 7, 8, 9, and 10), and fever in two (Patients 7 and 8). Hepatosplenomegaly was observed in one patient (Patient 7), while another (Patient 2) presented with respiratory symptoms, specifically cough.

3.2. Radiologic findings

Radiologic assessments revealed considerable variability in cyst location, size, and morphology. A detailed summary of imaging characteristics, including cyst location, dimensions (by CT and ultrasound), WHO-IWGE classification, cyst morphology, and multiplicity, is provided in Table 2. According to the WHO-IWGE classification, cysts were categorized as active (1CE–2CE), transitional (CE3A–CE3B), or inactive (4CE–5CE).

Table 2.

Radiologic imaging findings and cyst characteristics in 10 pediatric patients with splenic hydatid disease. Data include cyst location, size (CT and ultrasonography), WHO-IWGE classification, morphological patterns, and multiplicity. Imaging revealed splenic involvement in all cases, with concurrent hepatic (n = 3) and pulmonary (n = 1) cysts. Cysts spanned active (1CE, 2CE) to transitional (3CE, CE3A/B) stages, displaying features such as septations, calcifications, and detached laminations.

Patient No.	Cyst Location	CT-Scan Size (mm)	Ultrasound Size (mm)	WHO-IWGE Classification	Cyst Pattern	Solitary /Multiple
1	Liver (VII) / Spleen	60 × 53 mm in the VII hepatic lobe and 55 × 60 mm in the VII hepatic lobe	54 × 54 and 56 × 56 in right hepatic lobe and 55 × 99 in the spleen	1CE (Active)	Simple cystic lesions without septation or calcification	Multiple
2	Lung (RLL) / Spleen	50 × 70 mm in the right lower lobe of the lung	48 × 64 (Spleen)	2CE (Active)	Multilocular cysts with internal septations	Multiple
3	Spleen	80 (Spleen)	60 × 60, 80 (Spleen)	2CE (Active)	Simple cyst with internal septations without solid matrix	Solitary
4	Spleen	112 × 88 × 92	102 × 82 in the splenic hilum	3CE	Low-level internal echo with daughter cysts and wall calcifications	Solitary
5	Spleen	N/A	107 × 5	CE3A (Transitional)	Cyst with detached, irregular lamination (“water lily sign”)	Solitary
6	Spleen	58 × 60 × 65	53 × 57 × 60	3CE (Transitional)	Large, thin-walled cyst with faint wall calcifications	Solitary
7	Spleen	205 × 180 × 150	220 × 150 × 160	CE3B	Large irregular cyst with septations	Solitary
8	Liver & Spleen	65 (VI and IVb segments of the liver), 115 (Spleen)	70 × 65 (Liver), 100 × 90 (Spleen)	2CE	Multiple cystic hyperechoic lesions with well-defined borders	Multiple
9	Spleen	53 × 52	N/A	1CE	Simple cystic lesion without solid component or calcification	Solitary
10	Spleen	N/A	45 × 46	1CE	Simple cystic lesion without septation or calcification	Solitary

Footnotes for Table 2: Overview of the WHO-IWGE Classification

Splenic involvement was identified in all patients, with cyst sizes ranging from 45 mm to 220 mm. Hepatic involvement was present in two patients (Patients 1 and 8), each exhibiting multiple cysts. One patient (Patient 2) had pulmonary involvement, with multilocular cysts located in the right lower lobe of the lung. The largest cyst (220 × 150 × 160 mm), classified as CE3B, was found in Patient 7, while the smallest (45 × 46 mm, 1CE) was observed in Patient 10. Five patients (Patients 1, 2, 3, 8, and 9) had active-stage cysts (1CE or 2CE), and four (Patients 4, 5, 6, and 7) were in the transitional stage (CE3A or CE3B). No inactive cysts (4CE or 5CE) were identified. The cyst characteristics and classifications are summarized in Table 2.

Cyst size variability is further illustrated in Fig. 1, with examples ranging from 40 mm (A) to 220 mm (B), 150 mm (C), and 160 mm (D).

Fig. 1.

Axial CT scans (A–D) of splenic hydatid cysts in pediatric patients (ages 6–18 years). (A) Small unilocular cyst (40 mm); (B) Large multilocular cyst (220 mm) with septations; (C) Transitional cyst (150 mm) displaying detached laminations; (D) Complex cyst (160 mm) with calcifications. All images confirm hydatid cyst morphology and staging (WHO-IWGE classification).

3.3. Hospitalization, clinical course, and complications

The average hospitalization duration was 7.1 days (range: 3–12 days). Three patients had multi-organ involvement: Patient 1 had a cyst in the right hepatic lobe, Patient 2 in the right lower lung lobe, and Patient 8 in both the liver (segments IVB and VI) and the right lower lung lobe.

Preoperative complications were observed in several cases. Two patients had a history of relapsing hydatidosis prior to this study, and one presented with a ruptured splenic cyst. Patient 5 had undergone hepatic hydatid cyst surgery two years earlier and was diagnosed with a splenic CE3A cyst. Despite preoperative albendazole therapy, Patient 7 exhibited cyst enlargement and progression to CE3B. Patient 2 presented with a ruptured splenic cyst and acute respiratory distress, requiring emergency intervention and partial splenectomy. Postoperative recovery in this case was complicated by pleural effusion, necessitating drainage due to a preexisting pleurocutaneous fistula resulting from prior lung hydatidosis surgery.

In total, two patients had relapsing hydatidosis prior to splenectomy, and one experienced cyst rupture. Nine patients underwent total splenectomy, while one (Patient 6) underwent partial splenectomy due to a favorable cyst location allowing for organ preservation. Clinical manifestations, hospitalization duration, symptom chronicity, organ involvement, cyst rupture status, surgical procedures, and recurrence are summarized in Table 3.

Table 3.

Clinical management and hospitalization outcomes in 10 pediatric splenic hydatidosis cases, including preoperative regimens, surgical interventions, and postoperative care. Total splenectomy was performed in 9/10 patients, with partial splenectomy reserved for one case. All received combination antiparasitic (albendazole) and antibiotic therapy; two cases experienced relapsing hydatidosis. Complications included preoperative cyst rupture (n = 1). Postoperative protocols included extended antibiotic prophylaxis and antiparasitic therapy, with vaccination (e.g., Pneumovax) administered post-splenectomy.

Patient No.	Hospitalization Duration (Days)	Extra-Splenic Involvement (Location & Cyst Size)	Preoperative Complication (Cyst Rupture)	Relapsing hydatidosis	In-Hospital Preoperative Regimen (Antiparasitic & Antibiotic)	Surgical Procedure (Total/Partial Splenectomy)	In-Hospital Postoperative Regimen (Antibiotic & Antiparasitic)
1	12	Right hepatic lobe (55 × 49 × 52 × 51 mm)	No	Yes	Albendazole, Ceftriaxone, Metronidazole	Total	Cephalexin syrup q6hrs for 10 days + Penicillin-V syrup q12hrs for 10 days + Albendazole
2	11	Lung (RLL) (50 × 20 mm)	Yes	Yes	Ceftriaxone, Clindamycin, Metronidazole, Albendazole	Partial	Cefixime 200 mg q12hrs for 10 days, Clindamycin 300 mg q8hrs for 10 days, Albendazole 20 mg q12hrs
3	10	None	No	No	Cefazolin, Penicillin-V	Total	Cefazolin 1-g q8hrs + Penicillin-V 250 mg q12hrs; no albendazole due to poor compliance
4	3	None	No	No	Albendazole, Cefazolin	Total	Cephalexin 500 mg q6hrs for one month, Albendazole 400 mg for one week, Penicillin Amp stat
5	8	None	No	No	Ceftriaxone, Metronidazole, Albendazole, Cefazolin, Penicillin-V	Total	Penicillin-V 500 mg QD until age 15, Albendazole 300 mg q12hrs for one month
6	3	None	No	No	Cefazolin, Metronidazole, Penicillin-V	Total	Cephalexin 300 mg q6hrs, Penicillin-V 250 mg QD, Albendazole tablet BID for 1 month
7	6	None	No	No	Cefazolin, Penicillin-V, Tinidazole	Total	Albendazole 400 mg q12hrs, Penicillin-V 500 mg q12hrs, Cephalexin 500 mg q6hrs; Pneumovax administered 2 weeks post-surgery, followed by PPSV 23 8 weeks later; second dose recommended in 5 years
8	8	Liver IVB, V1 segments (65 mm) and right lower lobe (7 mm)	No	No	Albendazole, Ceftriaxone, Metronidazole, Penicillin-V	Total	Cefixime 400 mg QD, Metronidazole 500 mg q8hrs, Albendazole 400 mg q12hrs, Penicillin-V 250 mg q12hrs
9	3	None	No	No	Pneumovax, Albendazole, Metronidazole, Ceftriaxone	Total	Penicillin-V 500 mg q8hrs for two months, Cephalexin 500 mg q6hrs, Albendazole 300 mg q12hrs
10	7	None	No	No	Albendazole, Ceftriaxone, Metronidazole, Penicillin-V	Total	Cefixime 200 mg q8hrs, Metronidazole 250 mg q8hrs, Penicillin-V 500 mg QD, Albendazole 300 mg BID (200 mg morning, 100 mg evening)

3.4. Surgical interventions

Surgical treatment primarily involved total splenectomy (n = 9). Partial splenectomy was performed in one case (Patient 6) where cyst location favored organ preservation.

3.5. Medical therapies

Pharmacological management included a combination of anthelmintic and antibiotic therapies. All patients received preoperative antibiotic prophylaxis, predominantly cephalosporins (ceftriaxone or cefazolin). Postoperative antibiotic regimens varied depending on the clinical course. Patients without complications were treated with ceftriaxone monotherapy for 5–7 days. Patient 2, who had a ruptured cyst, was treated with a combination of ceftriaxone and metronidazole for 10 days.

Albendazole was the principal antiparasitic agent. Preoperative albendazole was administered in eight patients, while nine received postoperative albendazole, with treatment durations ranging from one week to one month. Metronidazole was prescribed in six patients, with durations ranging from 1 to 4 weeks in three cases and 2–8 months in seven cases.

3.6. Hematological findings

Hematologic evaluations demonstrated substantial variability in blood parameters. The mean red blood cell (RBC) count was 4.66 × 10⁶/μL (SD ± 0.37), and the mean hemoglobin concentration was 11.71 g/dL (range: 9.8–13.6 g/dL). The mean hematocrit was 36.17 % (SD ± 2.92), and the mean platelet count was 289.8 × 10³/μL (SD ± 89.13). Neutrophils and lymphocytes constituted mean percentages of 61.42 % (SD ± 27.11) and 29.82 % (SD ± 16.36), respectively. The systemic inflammation index (SII) varied widely (range: 120.83–5095.51; mean: 788.03), although its prognostic relevance remains uncertain. These findings are summarized in Supplementary Table 1.

3.7. Follow-up and postoperative management

Table 4 presents detailed information regarding pneumococcal vaccination, long-term prophylaxis, and incidence of Overwhelming Post-Splenectomy Infection (OPSI). Vaccination schedules and dosage guidelines are provided in the Methods section and table legend.

Table 4.

Long-term pharmacological management and postoperative outcomes in pediatric splenic hydatidosis cases, including pneumococcal vaccination compliance, antibiotic/antiparasitic prophylaxis regimens, and follow-up data. All patients received albendazole-based therapy with variable durations (2 months to 6 years); pneumococcal vaccination compliance was partial (n = 4). No cases of overwhelming post-splenectomy infection (OPSI), recurrence, or mortality were observed during follow-up (2–7 years).

Patient No.	Follow-Up Duration	Pneumococcal Vaccination	Long-Term Prophylaxis	OPSI Development	Postoperative Hydatid Recurrence	Follow-Up Death
1	2 years, 4 months	Yes	Albendazole tablet BID for 6 months + Penicillin Amp IM monthly until age 18	No	No	No
2	3 years, 2 months	No (Poor compliance)	Albendazole tablet for 8 months	No	No	No
3	7 years	Yes	Albendazole tablet for 6 months + Penicillin-V daily for one year	No	No	No
4	5 years, 8 months	No (Poor compliance)	Third-generation cephalosporins; Anti-helminthic; Penicillin Amp monthly	No	No	No
5	5 years, 2 months	Yes	Penicillin Amp monthly until age 16 + Albendazole tablet BID for one month	No	No	No
6	2 years, 7 months	Yes	Albendazole tablet BID for 2 months, Penicillin-V QD for one year	No	No	No
7	3 years, 5 months	Yes	Penicillin-V tablet QD until age 19, Pneumococcal polysaccharide vaccine	No	No	No
8	3 years, 10 months	No (Poor compliance)	Albendazole tablet BID for two months	No	No	No
9	2 years, 8 months	Yes	Albendazole tablet BID for 3 months, Penicillin Amp IM q6months for 2 consecutive years	No	No	No
10	3 years, 3 months	No (Poor compliance)	Penicillin-V QD until age 12, Albendazole tablet BID for two months	No	No	No

Also, for children aged 6 years and older, the recommended dosage of albendazole is 7.5 mg/kg per administration, given twice daily, with a maximum dose of 400 mg per administration. In pediatric patients, albendazole may be administered at 10–15 mg/kg per day (maximum 800 mg per day), divided into two doses for a duration of 1 to 6 months, depending on clinical circumstances [Moroni et al., 2016]. OPSI (Overwhelming Post-Splenectomy Infection) is defined as septicemia and/or meningitis following splenectomy, with a high associated mortality rate [Jones et al., 2010].

3.8. Prophylactic measures

Pneumococcal vaccination was administered to six patients prior to discharge. Postoperative antibiotic prophylaxis included cephalosporins and penicillin, with six patients receiving long-term penicillin prophylaxis following total splenectomy. Four patients did not receive the pneumococcal vaccine due to compliance issues.

3.9. Postoperative outcomes and follow-up

The average follow-up duration was 3 years, 11 months (SD ± 18.44 months). No cases of OPSI or hydatid recurrence were observed post-splenectomy, underscoring the efficacy of prophylactic measures. Long-term albendazole therapy (2–8 months) was administered in seven patients. All patients survived, and no mortality occurred.

4. Discussion

Our study provides valuable insights into the clinical characteristics, radiological features, and management of pediatric splenic hydatidosis. The demographic profile of our cohort, with a mean age of 11.27 years and a slight female predominance, aligns with existing literature, which reports a higher prevalence of hydatid disease in school-aged children, particularly in endemic regions (Moro and Schantz, 2009). The prolonged interval between symptom onset and diagnosis (median: 52 days) underscores the diagnostic challenges, often exacerbated by limited healthcare access and the nonspecific nature of initial symptoms (Bold et al., 2024).

Abdominal pain was the predominant presenting symptom in all patients, consistent with previous reports on pediatric splenic hydatidosis (Alharbi et al., 2023; Khalili et al., 2023). Particularly when accompanied by systemic manifestations such as nausea, vomiting, and anorexia, this symptom underscores the necessity of thorough clinical evaluation and a high index of suspicion in endemic areas. Notably, cyst rupture occurred in one patient, and two cases exhibited relapsing hydatidosis before splenic involvement (out of 10 cases), findings that align with previously reported complications (Acharya et al., 2019; Oussama et al., 2014; Belli et al., 2014). These complications highlight the need for vigilant follow-up to prevent disease progression and optimize long-term outcomes (Oussama et al., 2014).

Radiological assessment revealed cysts with varying size, location, and stage, as classified by the WHO-IWGE system. The predominance of active and transitional stage cysts (1CE–CE3B) emphasizes the necessity of early intervention to prevent progression to more complex lesions (WHO Informal Working Group, 2003). These findings suggest that timely diagnosis and treatment are feasible in pediatric populations.

Surgical intervention remains the cornerstone of hydatid disease management. In our study, total splenectomy was performed in nine out of ten patients, while one underwent partial splenectomy due to favorable cyst location. Partial splenectomy offers a potential organ-preserving alternative when technically feasible (Hoteit et al., 2020). The choice of surgical approach remains a topic of debate. Some studies advocate spleen-preserving techniques to maintain immunological function and reduce the risk of overwhelming post-splenectomy infection (OPSI) (Al-Hakkak et al., 2022; Skattum et al., 2012). A cohort study comparing spleen-preserving surgery with total splenectomy found no significant difference in recurrence rates, suggesting that organ-preserving approaches can be effective while maintaining splenic function (Aljaiuossi et al., 2024). Conversely, other research supports total splenectomy as the preferred option, as it eliminates the potential for recurrence associated with residual splenic tissue, particularly in cases with extensive cyst involvement (Meimarakis et al., 2009). In our cohort, the decision to perform total splenectomy in most cases was influenced by factors such as cyst size, location, and the presence of complications such as rupture.

Pre- and postoperative albendazole therapy was administered in all patients in accordance with standard guidelines to reduce recurrence risk and manage residual disease. Our findings are consistent with previous reports on the efficacy of albendazole, particularly as an adjunct to surgical management (Arif et al., 2011). However, the optimal duration of antiparasitic therapy remains a subject of discussion. Some studies advocate for extended treatment courses to ensure complete eradication of viable protoscolices (Alvela-Suárez et al., 2014), while others caution against prolonged therapy due to concerns regarding hepatotoxicity and patient tolerance (Zavoikin et al., 2021). Further research is needed to establish standardized protocols for antiparasitic therapy duration in pediatric hydatidosis.

Postoperative antiparasitic therapy combined with antibiotic prophylaxis proved effective in preventing severe infections, with no cases of OPSI or post-splenectomy recurrence reported during follow-up. This favorable outcome may be attributed to prophylactic measures, including pneumococcal vaccination, antibiotic therapy, and antiparasitic prophylaxis. The importance of vaccination in preventing OPSI is well-documented, and strict adherence to vaccination protocols is crucial in splenectomized patients (Boam et al., 2017; Luu et al., 2019). Our findings support the efficacy of these prophylactic strategies in reducing infectious risks following splenectomy (Aljaiuossi et al., 2024). .

The role of the Systemic Inflammation Index (SII) as a prognostic marker in hydatid disease is an emerging area of interest. Previous research has demonstrated its prognostic value in hepatic alveolar echinococcosis, where it correlates with disease severity, postoperative outcomes, and survival rates (Ren et al., 2021; Xiaobin et al., 2022). In our study, SII values varied widely among patients. However, due to the limited sample size and observational nature of our study, we refrained from drawing definitive conclusions regarding its prognostic significance. Further large-scale studies are warranted to determine the potential utility of SII in predicting outcomes in pediatric hydatidosis.

Our study has several limitations. The small sample size limits the generalizability of our findings and precludes robust statistical analyses. Given the rarity of splenic infection, most studies on this topic have a retrospective design and include sample sizes ranging from 5 to 20 patients. Additionally, the retrospective nature of our study introduces inherent biases in data collection and interpretation. The variability in treatment protocols, particularly regarding the duration of albendazole therapy, reflects the lack of standardized guidelines and highlights the need for consensus in this area. Future prospective studies with larger cohorts are essential to validate our findings and inform evidence-based management strategies for pediatric splenic hydatidosis.

5. Conclusion

Our study contributes to the understanding of pediatric splenic hydatidosis by detailing clinical presentations, diagnostic challenges, surgical management, and postoperative outcomes. While total splenectomy remains the most frequently performed procedure, the potential benefits of spleen-preserving surgery warrant further investigation. Standardization of antiparasitic therapy duration and prophylactic measures is crucial to optimize patient outcomes. Further research is needed to refine management strategies and improve prognostic assessments for this rare but clinically significant disease in pediatric populations.

Authors contribution

Mehdi Forooghi, Ali Tadayon, and Danial Ebrahimi conceptualized the study. Sedighe Hooshmandi and Mehdi Ghaderian Gahromi were responsible for the investigations. Zahra Sharifi, Rojan Abdollahzade, Simin Sharifi, and Asal Ebrahimi undertook visualizations and provided resources. Raha Shahrokhi, shayan Yousufzai, and Hossein Hosseini contributed to the original draft; and Writing - review & editing.

• •Active Group (1CE and 2CE):

  1CE: This category includes unilocular anechoic cysts exhibiting a double line sign, which indicates the presence of a viable cyst.

  2CE: This category encompasses multilocular cysts characterized by multiple internal septations, often described as having a “honeycomb” appearance, also signifying viability.

• •Transitional Group (3CE):

  3CEa: Cysts in this category display detached membranes, commonly referred to as the “water-lily sign,” suggesting that they are in a transitional phase while still potentially viable.

  3CEb: This category consists of predominantly solid cysts that contain daughter vesicles, indicating a more complex transitional state.

• •Inactive Group (4CE and 5CE):

  4CE: Cysts classified here exhibit mixed echogenicity (both hypoechoic and hyperechoic) and lack daughter vesicles, indicating a degenerative process.

  5CE: This category includes cysts that are either partially or completely calcified, signifying non-viable cysts.

CRediT authorship contribution statement

M. Forooghi: Conceptualization. H. Hosseini: Writing – review & editing, Writing – original draft, Investigation, Data curation. Sh. Yousufzai: Writing – review & editing, Writing – original draft, Investigation, Data curation. D. Ebrahimi: Data curation. R. Shahrokhi: Writing – original draft. A. Ebrahimi: Data curation. R. Abdollahzade: Data curation. S. Hooshmandi: Investigation. M.G. Gahromi: Investigation. S. Sharifi: Data curation. Z. Sharifi: Data curation. A. Tadayon: Supervision, Project administration, Methodology, Investigation, Conceptualization.

Consent for publication

Informed consent was obtained from all patients and their legal guardians. A copy of the informed consent form and any accompanying images are available upon reasonable request from the corresponding author.

Ethics approval and consent to participate

Informed written consent was secured from the legal guardians of all participants involved in the study prior to publication. Subsequently, the research was conducted in compliance with ethical standards and received approval from the Institutional Review Board (IRB) of Shiraz University of Medical Sciences, under reference ID “IR.SUMS.MED.REC.1403.432.”

Funding

None.

Declaration of competing interest

The authors declare no conflict of interests.

Appendix A. Supplementary data

Supplementary materials are provided for further information.

Data availability

Patients' dataset is available upon reasonable request.