Precautions for Splenectomy in Hereditary Spherocytosis in Children

Preoperative Immunization Requirements

Children with hereditary spherocytosis undergoing splenectomy must receive preoperative immunization against encapsulated bacteria, followed by lifelong antibiotic prophylaxis with phenoxymethylpenicillin to prevent overwhelming post-splenectomy sepsis, as recommended by the American Journal of Hematology 1, 2
Vaccination must be completed at least 2 weeks before elective splenectomy to ensure optimal antibody response, according to the BMJ 2
The 23-valent polysaccharide pneumococcal vaccine is more than 90% effective in healthy children and covers the most prevalent serotypes, as stated in the BMJ 2
The meningococcal vaccine can be given safely to children and is essential for preventing meningococcal sepsis, as reported in the BMJ 2
The Haemophilus influenzae type B vaccine should be administered as part of the encapsulated bacteria protection strategy, according to the BMJ 2
The influenza vaccine is recommended yearly to reduce the risk of secondary bacterial infection, as stated in the BMJ 2

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

Lifelong prophylactic antibiotics should be offered in all cases, with particular emphasis on the first two years after splenectomy when risk is highest, as stated in the BMJ 2
Phenoxymethylpenicillin (oral penicillin V) has been used effectively for years in children with functional asplenia, as reported in the BMJ 2
Erythromycin should be offered to patients allergic to penicillin, according to the BMJ 2
Patients should keep a supply of amoxicillin at home to use immediately if symptoms of infection develop, as recommended in the BMJ 2

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, as stated in Blood 3

A multidisciplinary team including the surgeon, anesthetist, and pediatric hematologist must collaborate on preoperative planning, as recommended by the American Journal of Hematology 1, 4
Laboratory testing, including complete blood counts, reticulocyte count, renal function tests, liver function tests, and bilirubin, should be obtained to verify the patient is at optimal baseline, according to the American Journal of Hematology 1, 4
Preoperative blood typing and immune-hematological screening for possible erythrocyte antibodies should be performed to expedite potential transfusions, as stated in the American Journal of Hematology 1, 4
A complete preoperative assessment with vital signs and review of personal health history focusing on cardiovascular, respiratory, renal complications, recent infectious events, and prior transfusions should be completed, as recommended by the American Journal of Hematology 1, 4

Active infection or acute hemolytic crisis are absolute contraindications for any elective, non-urgent splenectomy, as stated in the American Journal of Hematology 1, 4

The risk of overwhelming infection is lifelong, with cases of fulminating infection reported more than 20 years after splenectomy, as reported in the BMJ 2
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, according to the BMJ 2
Patients should be provided with an information leaflet and patient card about splenectomy for emergency situations, as recommended in the BMJ 2
The general practitioner should be notified of the splenectomy and vaccinations given to avoid potential reactions from premature reimmunisation, as stated in the BMJ 2

Animal bites require immediate antibiotic coverage, as asplenic patients are particularly susceptible to Capnocytophaga canimorsus infection, as reported in the BMJ 2
A 5-day course of co-amoxiclav (or erythromycin in allergic patients) should be administered after dog or other animal bites, according to the BMJ 2
For travel to endemic areas, consider additional prophylaxis for histoplasmosis, babesiosis, and malaria, as recommended in the BMJ 2

The World Journal of Emergency Surgery recommends vaccinating as soon as the patient is stable postoperatively, ideally within 14 days of surgery, if vaccines were not administered at least 2 weeks preoperatively 5
The antibody response is suboptimal if given before 14 days post-splenectomy, but vaccination before discharge is preferable to missing it entirely if follow-up compliance is uncertain, as stated by the World Journal of Emergency Surgery 5

The American Journal of Hematology recommends maintaining aggressive hydration, oxygenation, thermoregulation, and pain management to prevent vaso-occlusive complications 6
The American Journal of Hematology suggests continuing intravenous hydration until oral intake is fully re-established, providing supplemental oxygen to maintain transcutaneous saturation >95%, avoiding hypothermia, implementing multimodal analgesia, and encouraging early mobilization and incentive spirometry to prevent pulmonary complications 6

The World Journal of Emergency Surgery states that the risk of OPSI is 0.5-2% with mortality rates of 30-70%, and most deaths occur within the first 24 hours of symptom onset 5
The World Journal of Emergency Surgery recommends providing additional prophylaxis for travel to malaria-endemic areas and educating about increased risk from encapsulated bacteria: Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis 5

The World Journal of Emergency Surgery advises against delaying vaccination beyond 14 days postoperatively, as the earlier after this window, the better the antibody response 5
The American Journal of Hematology warns against using hypertonic fluids for hydration, as these can worsen red blood cell dehydration 6
The American Journal of Hematology cautions against applying ice packs for pain management, as hypothermia must be avoided at all costs 6