Hereditary spherocytosis surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
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Overview
Surgery
Splenectomy:
- Generally, the treatment of HS involves presplenectomy care, splenectomy, and management of postsplenectomy complications.
- In pediatric cases, splenectomy ideally should not be performed until a child is older than 6 years because of the increased incidence of postsplenectomy infections with encapsulated organisms such as S pneumoniae and H influenzae in young children.
- Partial splenectomies are increasingly used in pediatric patients, as this approach appears to both control hemolysis and preserve splenic function.
- European guidelines on splenectomy for HS note that a laparoscopic approach is currently considered the gold standard for removal of a normal-sized or slightly enlarged spleen and is preferred to open splenectomy, but it should be performed only by experienced surgeons.
- In children undergoing splenectomy, the gallbladder should be removed concomitantly if the patient has symptomatic gallstones.
Complications — Splenectomy has a number of known risks of which patients (or parents) should be aware:
- ●Operative risks (eg, infection, bleeding, or injury to adjacent organs such as the stomach or tail of the pancreas); these are relatively infrequent. ●Infections, including overwhelming sepsis, from encapsulated organisms (eg, Streptococcus pneumoniae, Neisseria meningitidis, H. influenzae) that can no longer be removed by normal splenic clearance mechanisms, as well as certain other microorganisms including plasmodia, Babesia, Bordetella, and Capnocytophaga species (from animal bites) [115]. These risks are thought to be highest in the first year following splenectomy and in individuals undergoing splenectomy before five to six years of age. However, risks of sepsis are likely to have declined with improved options for preoperative vaccinations and postoperative prophylactic penicillin. This was illustrated in a 1991 study from the Danish National Patient Registry that demonstrated a dramatic reduction in serious S. pneumoniae infections following pneumococcal vaccination [148]. Individuals who did not receive appropriate pre-splenectomy vaccinations should have a thorough review of their immunization history and should receive vaccinations as discussed separately. (See "Prevention of sepsis in the asplenic patient".) ●Venous thromboembolic (VTE) complications including thromboses of the deep veins, pulmonary emboli, splenic or portal vein thrombosis, as well as thrombosis in other unusual sites [149,150]. VTE events appear to be more common in individuals with HS who undergo splenectomy than in those who do not, but the individuals who undergo splenectomy may have had more severe underlying disease, making direct comparisons difficult [151]. Thromboprophylaxis at the time of surgery should be based on standard practices; there is no indication for extended thromboprophylaxis beyond the usual duration [80]. ●Arterial thrombotic events may also be increased relative to individuals with HS who do not undergo splenectomy, with the same caveat that applies to VTE (patients who undergo splenectomy may have more severe underlying disease) [151,152]. ●It is not clear whether pulmonary artery hypertension (PAH) is a complication of splenectomy in HS. [153].