What precautions are necessary for children with hereditary spherocytosis undergoing splenectomy?

Precautions for Splenectomy in Hereditary Spherocytosis in Children

Children with hereditary spherocytosis undergoing splenectomy must receive preoperative immunization against encapsulated bacteria (pneumococcus, meningococcus, and Haemophilus influenzae), followed by lifelong antibiotic prophylaxis with phenoxymethylpenicillin to prevent overwhelming post-splenectomy sepsis. 1

Preoperative Immunization Requirements

Vaccination must be completed at least 2 weeks before elective splenectomy to ensure optimal antibody response. 1

• Pneumococcal vaccine: Administer the 23-valent polysaccharide vaccine, which is more than 90% effective in healthy children and covers the most prevalent serotypes 1
• Meningococcal vaccine: Can be given safely to children and is essential for preventing meningococcal sepsis 1
• Haemophilus influenzae type B vaccine: Should be administered as part of the encapsulated bacteria protection strategy 1
• Influenza vaccine: Recommended yearly to reduce the risk of secondary bacterial infection 1

Reimmunization Strategy

• Consider reimmunization after 2 years in children who remain at risk, as young children have inherently reduced ability to mount an antibody response 1
• Long-term reimmunization is recommended every 5-10 years for asplenic patients 1

Antibiotic Prophylaxis Protocol

Lifelong prophylactic antibiotics should be offered in all cases, with particular emphasis on the first two years after splenectomy when risk is highest. 1

• First-line prophylaxis: Phenoxymethylpenicillin (oral penicillin V) has been used effectively for years in children with functional asplenia 1
• Penicillin allergy alternative: Erythromycin should be offered to patients allergic to penicillin 1
• Home supply: Patients should keep a supply of amoxicillin at home to use immediately if symptoms of infection develop 1

Important Caveat

Antibiotic prophylaxis may not prevent all cases of sepsis, as phenoxymethylpenicillin does not cover H. influenzae, and amoxicillin does not reliably cover all organisms 1

Timing Considerations

Splenectomy should ideally be delayed until after age 5-6 years when possible, as children under 5 years have the highest risk of overwhelming post-splenectomy infection (1 death per 300-1,000 patient-years). 1

• For children requiring earlier intervention, consider partial splenectomy as an alternative that maintains residual splenic phagocytic function while decreasing hemolytic rate 2
• Partial splenectomy can increase hemoglobin by an average of 3 g/dL and substantially prolong RBC life span while maintaining normal splenic uptake on technetium scans 2

Preoperative Assessment Requirements

A multidisciplinary team including the surgeon, anesthetist, and pediatric hematologist must collaborate on preoperative planning. 1

• Laboratory testing: Obtain complete blood counts, reticulocyte count, renal function tests, liver function tests, and bilirubin to verify the patient is at optimal baseline 1
• Blood typing: Perform preoperative blood typing and immune-hematological screening for possible erythrocyte antibodies to expedite potential transfusions 1
• Clinical examination: Complete preoperative assessment with vital signs and review of personal health history focusing on cardiovascular, respiratory, renal complications, recent infectious events, and prior transfusions 1

Contraindications to Elective Surgery

Active infection or acute hemolytic crisis are absolute contraindications for any elective, non-urgent splenectomy. 1

Long-Term Infection Risk Management

The risk of overwhelming infection is lifelong, with cases of fulminating infection reported more than 20 years after splenectomy. 1

• Patients and families must be educated that while most infections occur within the first two years, up to one-third may manifest at least five years later 1
• Provide patients with an information leaflet and patient card about splenectomy for emergency situations 1
• Notify the general practitioner of the splenectomy and vaccinations given to avoid potential reactions from premature reimmunisation 1

Special Precautions for Specific Exposures

Animal bites require immediate antibiotic coverage, as asplenic patients are particularly susceptible to Capnocytophaga canimorsus infection. 1

• Administer a 5-day course of co-amoxiclav (or erythromycin in allergic patients) after dog or other animal bites 1
• For travel to endemic areas, consider additional prophylaxis for histoplasmosis, babesiosis, and malaria 1

Evidence Supporting Conservative Approach

Recent evidence suggests that the infectious burden in hereditary spherocytosis children is actually higher before splenectomy than after, with 22% requiring hospitalization for infection pre-splenectomy versus only 4.1% post-splenectomy (OR 6.6,95% CI 3.0-14.2). 3 However, this does not negate the need for rigorous infection prevention protocols, as the consequences of post-splenectomy sepsis can be catastrophic despite lower overall incidence. 1