In splenectomized patients, a significant problem is represented by overwhelming post-splenectomy infections (OPSI) that are characterized by a sudden and rapid general deterioration that occurs after about 24 h from the onset of the first infectious sign, such as flu, up to the framework of fulminating sepsis with CID and septic shock that can quickly lead to death
[5][6][9,10]. The incidence of sepsis in splenectomized children is currently between 1.8–4%, with a mortality that can reach about the 50% of cases. This incidence is at a maximum in the first three years after the splenectomy and then is progressively reduced, persisting throughout the life span and being able to occur even after 50 years. In the first five years of life, a risk between 60 and 100 times greater than non-splenectomized subjects is estimated
[7][11].
The age of the child (maximum under 2 years and however high up to 5 years), the time elapsed from the splenectomy (greater risk in the first 2–3 years that then decreases without ever zeroing and resurfacing after 60 years), the basic disease (drepanocytosis, thalassemia and some neoplastic pathologies and in traumas) are known as risk factors for OPSI
[8][9][10][12,13,14].
Splenectomy should therefore be avoided up to five years of life, when possible. OPSI are caused mainly by capsulated bacteria (Streptococcus pneumoniae in 50% of cases, to a lesser extent Haemophilus Influenzae type b, and Neisseria Meningitides)
[11][12][15,16]. In addition, splenectomized children have a higher risk of developing severe forms of Malaria, Bordetella, Babesiosis and secondary dog bite infections (such as Capnocytophaga canimorsum)
[13][14][17,18].
Vaccination is the most effective prevention tool for splenectomized patients. Children should be vaccinated two weeks before the splenectomy, when the surgery is planned electively, as in the case of hematologic disorders. In those cases when the splenectomy is performed in an emergency, vaccination should be carried out from 14 days after surgery, although some data suggest that they can be effective even in the immediate peri-operative time
[15][21].
Adherence to vaccination recommendations has proved very variable and unsatisfactory in most splenectomized subjects
[16][28]. Moreover, a vaccine failure or an infection caused by a pathogen other than those provided by the vaccination coverage (Pseudomonas Aeruginosa, Escherichia Coli) may occur even in those who appropriately follow the proposed vaccination schedule. Orally administered penicillin is the indicated antibiotic. Although for the recent outbreaks of resistant bacteria, amoxicillin-clavulanic acid and trimethoprim-sulfamethoxazole or, in allergic subjects, erythromycin, are now recommended. There is less consensus on the duration of the above-mentioned prophylaxis.
Splenectomy may generate a prothrombotic state, with consequent increased risk of venous thromboembolic disease, such as thromboembolic pulmonary hypertension. Thus, splenectomy is recognized as a potential risk factor for chronic thromboembolic pulmonary hypertension (CTEPH)
[13][17]. Recently,
Zhang et al. performed a systematic review and meta-analysis to explore the association between splenectomy and CTEPH, finding a prevalence of splenectomy in CTEPH of about 4%
[17][30].
From a surgical point of view, the laparoscopic approach is preferable as it reduces intraoperative complications, post-operative pain and hospitalization time. Currently, the improvement of surgical techniques has allowed to extend the laparoscopic approach to this procedure
[18][31]. Partial splenectomy consists of removing 70–80% of the spleen, as it has been shown in experimental models that the remaining 25–30% of the initial splenic tissue may ensure its immune action. The benefits of a partial splenectomy could be greater in patients with a high risk of infection, since the residual splenic tissue could ensure the desired hematologic effect, maintaining a good immunological function and reducing the risk of severe sepsis in children. Despite these assumptions, current data are limited, not allowing to clearly define the risk/benefit ratio of this procedure
[19][32].