Overview
Definition:
Pediatric splenectomy for hereditary spherocytosis (HS) involves the surgical removal of the spleen in children diagnosed with this inherited red blood cell membrane disorder, aiming to alleviate chronic hemolytic anemia and its associated complications.
Epidemiology:
Hereditary spherocytosis is the most common inherited hemolytic anemia in Caucasian populations, with an incidence of approximately 1 in 2000 to 1 in 5000 births
Splenectomy is indicated in a significant proportion of affected children, typically after medical management fails.
Clinical Significance:
Untreated HS in children can lead to severe anemia, jaundice, splenomegaly, gallstones, aplastic crises, and extramedullary hematopoiesis, impacting growth and development
Splenectomy, when indicated, is a curative option for the hemolytic component of the disease, significantly improving quality of life and preventing long-term sequelae.
Indications
Surgical Indications:
Persistent, symptomatic anemia requiring frequent transfusions
Severe, symptomatic splenomegaly causing abdominal discomfort or early satiety
Recurrent episodes of aplastic crisis that are transfusion-dependent
Documented cholelithiasis in the presence of significant hemolysis
Failure of conservative medical management (e.g., folic acid supplementation, watchful waiting in milder cases).
Timing Of Surgery:
Generally recommended after the age of 5-6 years to allow the spleen to develop immunologically important lymphoid tissue and reduce the risk of overwhelming postsplenectomy infection (OPSI)
However, earlier intervention may be considered in severe, life-threatening cases.
Contraindications:
Absolute contraindications are rare
Relative contraindications include significant comorbid conditions, uncorrected coagulopathy, and very young age (<2-3 years) without compelling indications
Postponement may be advised during acute infection or aplastic crisis.
Preoperative Preparation
Hematological Assessment:
Complete blood count (CBC) with differential, reticulocyte count, peripheral blood smear for characteristic spherocytes, direct and indirect Coombs tests (usually negative in HS, but important to rule out autoimmune hemolytic anemia), hemoglobin electrophoresis if thalassemia is suspected
Assess for evidence of hemolysis (elevated bilirubin, LDH
decreased haptoglobin).
Immunizations:
Essential to administer pneumococcal (PCV13, PPSV23), meningococcal (MenACWY, MenB), and Haemophilus influenzae type b (Hib) vaccines at least 2 weeks before surgery
Booster doses will be required.
Prophylaxis:
Prophylactic antibiotics (e.g., Penicillin V) should be initiated, especially in younger children, and continued long-term post-splenectomy
Folic acid supplementation should be continued.
Patient And Family Education:
Thorough explanation of the procedure, risks, benefits, and long-term implications of splenectomy, particularly OPSI
Education on signs and symptoms of infection and the importance of prompt medical attention.
Surgical Procedure
Surgical Approaches:
Laparoscopic splenectomy is the preferred method due to minimally invasive nature, shorter hospital stay, and faster recovery
Open splenectomy may be necessary for very large spleens, extensive adhesions, or in resource-limited settings.
Technique Laparoscopic:
Typically performed under general anesthesia using 3-4 ports
The spleen is carefully dissected from surrounding structures, taking care to ligate and divide the splenic artery and vein
Preservation of accessory spleens is crucial to prevent recurrence of HS.
Technique Open:
Requires a subcostal or midline incision
Similar dissection principles apply, with meticulous attention to hemostasis
Intraoperative palpation for accessory spleens is important.
Accessory Spleen Management:
Accessory spleens (ranging from 1 to over 20) are common in HS and their presence can lead to continued hemolysis
Meticulous intraoperative palpation of the splenic hilum, mesentery, and omentum is critical
If identified, they should be removed
Failure to remove all accessory spleens is a major cause of post-splenectomy relapse.
Postoperative Care
Pain Management:
Adequate analgesia using multimodal approaches (e.g., IV paracetamol, NSAIDs, opioids if necessary) is essential
Epidural or regional blocks may be considered.
Monitoring:
Close monitoring of vital signs, fluid balance, wound healing, and signs of infection
Routine lab work to assess for anemia and hemolysis
Platelet count may transiently increase post-splenectomy (reactive thrombocytosis).
Antibiotic Prophylaxis:
Continued oral antibiotic prophylaxis (e.g., Penicillin V 125 mg twice daily for children <5 years, 250 mg twice daily for older children) is crucial lifelong
Frequency and dosage may be adjusted based on weight and age.
Dietary And Activity Recommendations:
Gradual return to normal diet
Avoid heavy lifting and strenuous physical activity for 4-6 weeks post-laparoscopic surgery, and longer for open surgery
Encourage hydration.
Complications
Overwhelming Postsplenectomy Infection Opsi:
The most serious complication, a rapidly progressing sepsis caused by encapsulated bacteria (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type b)
Incidence is highest in the first few years post-splenectomy but remains lifelong.
Thromboembolism:
Reactive thrombocytosis can increase the risk of venous thromboembolism, especially in the early postoperative period
Anticoagulation may be considered in select high-risk patients.
Bile Duct Injury:
Rare complication during dissection, especially with laparoscopic approaches
Careful surgical technique minimizes this risk.
Wound Infection And Hernia:
Standard surgical complications, more common with open procedures
Laparoscopic surgery generally has lower rates.
Relapse Of Hemolysis:
Due to the presence or growth of accessory spleens, leading to a return of spherocytosis and hemolytic anemia
Requires re-evaluation and potentially further intervention.
Prognosis
Long Term Outcomes:
Splenectomy is highly effective in correcting the hemolytic anemia of HS, leading to resolution of jaundice, normalization of hemoglobin, and reduced risk of gallstones and extramedullary hematopoiesis
Quality of life significantly improves.
Monitoring For Infection:
Lifelong vigilance for signs of infection is paramount
Patients and families must be educated to seek immediate medical attention for fever or any signs of illness
Prompt empiric antibiotic treatment is critical.
Accessory Spleen Surveillance:
Regular follow-up with hematology is essential to monitor for recurrence of hemolysis, which may indicate the presence or growth of accessory spleens
Further investigations and potentially re-exploration may be needed.
Key Points
Exam Focus:
Indications for splenectomy in pediatric HS
importance of age > 5-6 years
necessity of removing accessory spleens
lifelong vaccination and antibiotic prophylaxis protocols for OPSI prevention
management of reactive thrombocytosis
symptoms of OPSI.
Clinical Pearls:
Thorough palpation for accessory spleens is key to preventing relapse
Empiric antibiotics for any febrile illness in asplenic children
Educate parents extensively on OPSI risks and management
Consider laparoscopic approach for faster recovery.
Common Mistakes:
Inadequate removal of accessory spleens
neglecting or discontinuing lifelong antibiotic prophylaxis and immunizations
delayed recognition and treatment of OPSI
performing splenectomy too early without sufficient medical indication or pre-operative preparation.