Splenectomy in Children

Topic Name Introduction Indications Preoperative Evaluation Surgical Techniques Postoperative Management Complications Long-Term Considerations Partial Splenectomy

Splenectomy in Children

Splenectomy, the surgical removal of the spleen, is a procedure performed in children for various hematological, oncological, and other medical conditions. While the spleen plays crucial roles in immune function and blood filtration, its removal may be necessary in certain circumstances. Splenectomy in children requires careful consideration due to the long-term implications for immune function and the risk of overwhelming post-splenectomy infection (OPSI).

Indications

The indications for splenectomy in children can be broadly categorized as follows:

1. Hematological disorders:
   • Hereditary spherocytosis
   • Sickle cell disease (for acute splenic sequestration or hypersplenism)
   • Thalassemia major (for hypersplenism)
   • Immune thrombocytopenic purpura (ITP) refractory to medical management
   • Autoimmune hemolytic anemia
2. Oncological conditions:
   • Hodgkin lymphoma (historically; now rarely indicated)
   • Non-Hodgkin lymphoma
   • Leukemia with symptomatic splenomegaly
3. Splenic cysts or tumors:
   • Large symptomatic cysts
   • Primary splenic tumors
4. Trauma:
   • Severe splenic injury not amenable to non-operative management or splenic preservation techniques
5. Other indications:
   • Gaucher disease with massive splenomegaly or cytopenias
   • Portal hypertension with hypersplenism

Preoperative Evaluation

Thorough preoperative evaluation is crucial for children undergoing splenectomy:

1. Medical history and physical examination:
   • Assessment of underlying condition and comorbidities
   • Evaluation of spleen size and any associated symptoms
2. Laboratory studies:
   • Complete blood count (CBC) with differential
   • Coagulation profile
   • Liver function tests
   • Blood type and cross-match
3. Imaging studies:
   • Ultrasound or CT scan to assess spleen size and anatomy
   • Evaluation for accessory spleens (present in 10-30% of patients)
4. Vaccination:
   • Administration of encapsulated organism vaccines at least 2 weeks prior to elective splenectomy:
     1. Pneumococcal vaccine (PCV13 and PPSV23)
     2. Haemophilus influenzae type b (Hib) vaccine
     3. Meningococcal vaccines (MenACWY and MenB)
5. Antibiotic prophylaxis:
   • Initiation of prophylactic antibiotics (usually penicillin) prior to surgery
6. Hematology consultation:
   • Optimization of hemoglobin levels in patients with hemolytic disorders
   • Management of platelet counts in ITP patients
7. Anesthesia evaluation:
   • Assessment of risks related to potential comorbidities

Surgical Techniques

The choice of surgical technique depends on various factors, including the child's age, size, underlying condition, and surgeon's expertise:

1. Open splenectomy:
   • Traditional approach, often used for massive splenomegaly or in trauma settings
   • Left subcostal or midline incision
   • Allows thorough exploration of the abdominal cavity
2. Laparoscopic splenectomy:
   • Increasingly preferred approach in children
   • Typically uses 3-4 ports
   • Benefits include reduced postoperative pain, shorter hospital stay, and improved cosmesis
   • May be challenging in cases of massive splenomegaly
3. Single-incision laparoscopic splenectomy (SILS):
   • Evolving technique with potential for even less invasive approach
   • May be suitable for select patients
4. Robotic-assisted splenectomy:
   • Emerging technique in pediatric surgery
   • Offers enhanced visualization and precision
   • Limited availability and higher cost

Key steps in splenectomy, regardless of approach:

• Careful ligation of splenic artery and vein
• Meticulous dissection of splenic attachments
• Thorough search for accessory spleens
• Retrieval of the spleen (may require morcellation in laparoscopic approach)

Postoperative Management

Proper postoperative care is essential for optimal outcomes in children undergoing splenectomy:

1. Pain management:
   • Multimodal analgesia, including opioids, NSAIDs, and regional anesthesia techniques
2. Monitoring:
   • Regular vital signs and abdominal examinations
   • Hemoglobin and platelet count checks
3. Thromboprophylaxis:
   • Early mobilization
   • Consideration of pharmacological prophylaxis in high-risk patients
4. Antibiotic prophylaxis:
   • Continuation of prophylactic antibiotics (duration varies based on institutional protocols)
5. Nutritional support:
   • Early initiation of oral intake as tolerated
6. Education:
   • Patient and family education on the risks of post-splenectomy infection
   • Importance of antibiotic prophylaxis and prompt medical attention for febrile illnesses
7. Follow-up:
   • Arranging follow-up appointments with surgery and hematology
   • Planning for long-term antibiotic prophylaxis and vaccination schedules

Complications

While generally safe, splenectomy in children can be associated with various complications:

1. Immediate postoperative complications:
   • Bleeding
   • Pancreatic injury or pancreatitis
   • Gastric injury
   • Wound infection
2. Early postoperative complications:
   • Subphrenic abscess
   • Thrombocytosis (usually transient)
   • Portal or splenic vein thrombosis
3. Long-term complications:
   • Overwhelming post-splenectomy infection (OPSI):
     • Life-threatening sepsis, typically caused by encapsulated organisms
     • Highest risk in the first 2 years post-splenectomy, but persists lifelong
   • Increased risk of thrombotic events
   • Pulmonary hypertension (particularly in patients with hemolytic disorders)

Long-Term Considerations

Children who have undergone splenectomy require lifelong management and follow-up:

1. Antibiotic prophylaxis:
   • Daily prophylactic antibiotics (usually penicillin) until at least 5 years of age
   • Some guidelines recommend lifelong prophylaxis
2. Vaccination:
   • Regular booster vaccinations against encapsulated organisms
   • Annual influenza vaccination
3. Education:
   • Ongoing education about the risks of post-splenectomy infection
   • Importance of seeking immediate medical attention for febrile illnesses
4. Medical alert:
   • Wearing a medical alert bracelet indicating asplenic status
5. Travel considerations:
   • Additional vaccinations and precautions for international travel
6. Hematological monitoring:
   • Regular follow-up to monitor for disease-specific outcomes and potential complications

Partial Splenectomy

Partial splenectomy is an evolving approach aimed at preserving splenic function while addressing specific conditions:

1. Indications:
   • Hereditary spherocytosis
   • Sickle cell disease
   • Splenic cysts
2. Advantages:
   • Preservation of some splenic immune function
   • Reduced risk of overwhelming post-splenectomy infection
   • Potential for normal growth of residual splenic tissue
3. Techniques:
   • Open or laparoscopic approach
   • Typically aims to preserve 10-30% of splenic tissue
4. Challenges:
   • Technical difficulty in achieving adequate hemostasis
   • Potential for regrowth of diseased splenic tissue
   • Long-term efficacy still under investigation for some conditions
5. Follow-up:
   • Regular monitoring of splenic function and growth
   • May still require some antibiotic prophylaxis and vaccination, depending on the amount of residual splenic tissue

Splenectomy in Children

1. What is splenectomy?
   Answer: Surgical removal of the spleen
2. What are the most common indications for splenectomy in children?
   Answer: Hereditary spherocytosis, immune thrombocytopenia (ITP), and sickle cell disease
3. What is the primary function of the spleen?
   Answer: Filtering blood and removing old or damaged red blood cells
4. What is the main immunological function of the spleen?
   Answer: Production of antibodies and removal of encapsulated bacteria
5. What is the most serious long-term risk following splenectomy in children?
   Answer: Overwhelming post-splenectomy infection (OPSI)
6. Which bacteria are children most susceptible to after splenectomy?
   Answer: Encapsulated bacteria such as Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis
7. When should vaccinations be given in relation to elective splenectomy?
   Answer: At least two weeks before surgery, if possible
8. What is the recommended antibiotic prophylaxis regimen for post-splenectomy children?
   Answer: Daily oral penicillin or amoxicillin
9. Until what age is antibiotic prophylaxis typically recommended after splenectomy?
   Answer: At least 5 years post-splenectomy or until 18 years of age, whichever is longer
10. What surgical approaches can be used for splenectomy in children?
    Answer: Open surgery or laparoscopic splenectomy
11. What is the advantage of laparoscopic splenectomy over open surgery?
    Answer: Less postoperative pain, shorter hospital stay, and better cosmetic results
12. What is a potential complication of laparoscopic splenectomy?
    Answer: Bleeding requiring conversion to open surgery
13. What is the most common early postoperative complication of splenectomy?
    Answer: Left pleural effusion
14. What is thrombocytosis and why does it occur after splenectomy?
    Answer: Increased platelet count due to loss of the spleen's role in platelet sequestration
15. How long does post-splenectomy thrombocytosis typically last?
    Answer: Several weeks to months
16. What is the risk of thrombosis following splenectomy?
    Answer: Slightly increased, especially in the portal venous system
17. What is a splenunculus?
    Answer: Accessory splenic tissue, which may continue to function after splenectomy
18. How common are splenunculi?
    Answer: Present in about 10-30% of people
19. What imaging modality is best for detecting splenunculi?
    Answer: Nuclear medicine scan (technetium-99m sulfur colloid scan)
20. What is partial splenectomy and when might it be considered?
    Answer: Removal of part of the spleen, considered in some cases to preserve some splenic function
21. What percentage of splenic tissue needs to be preserved for adequate function?
    Answer: At least 25-30%
22. What is the typical length of hospital stay for uncomplicated laparoscopic splenectomy in children?
    Answer: 2-3 days
23. What preoperative imaging is typically performed before splenectomy?
    Answer: Ultrasound to assess spleen size and look for accessory spleens
24. What is the role of preoperative blood transfusion in children undergoing splenectomy?
    Answer: May be needed in cases of severe anemia or thrombocytopenia
25. What is the recommended fasting period before elective splenectomy in children?
    Answer: Usually 6-8 hours for solids, 2-3 hours for clear liquids
26. What type of incision is typically used for open splenectomy in children?
    Answer: Left subcostal incision
27. What is the role of intraoperative cell salvage in pediatric splenectomy?
    Answer: Can be used to reduce the need for allogeneic blood transfusion
28. What is the typical positioning of the patient for laparoscopic splenectomy?
    Answer: Right lateral decubitus position
29. What is the role of preoperative splenic artery embolization in pediatric splenectomy?
    Answer: Rarely used in children, may be considered for massive splenomegaly
30. What is the recommended follow-up schedule for children after splenectomy?
    Answer: Regular check-ups, at least annually, with special attention to immunization status and antibiotic prophylaxis

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