We prospectively studied the efficacy of near total splenectomy (NTS) for managing hereditary spherocytosis (HS) based on haemoglobin (Hb), total bilirubin and splenic remnant regrowth in 30 children receiving NTS for HS between November 1996 and December 2004 (mean followup 3.6 years). Patients were classified into three severity groups. At followup, mean Hb had increased by 2.9-5.0 g/dl (P = 0.110) and bilirubin dropped by 15.4-56.4 mumol/l. Remnant regrowth was moderate, within the weight-specific norm. Amongst spleen-preserving techniques, NTS shows markedly lower rates of recurrent haemolysis, remnant regrowth and secondary operations, thus potentially benefiting all clinical forms of HS.
Near-total splenectomy provides a favourable immunological basis for natural and vaccine-induced protection against meningococcal serogroup A and C infections. Sequential meningococcal vaccination is immunogenic in patients splenectomized for hereditary spherocytosis.
ZusammenfassungDie chirurgische Standardtherapie der hereditären Sphärozytose ist die komplette Splenektomie. Die Asplenie führt aber zu einem lebenslang bestehenden Immundefizit. Bisher praktizierte Milzteilentfernungen, bei denen 10-40 % einer vergrößerten Milz erhalten wurden, induzierten oft ein überproportionales Wachstum des Milzrestes, Rezidivhämolysen und Folgeoperationen. Unsere Hypothese war, ob ein wesentlich kleinerer Milzrest überhaupt überleben und Rezidive effektiv verhindern kann. Dazu entwickelten wir das standardisierte Operationsverfahren der near-total Splenektomie, bei dem ein Milzrest von 10 cm 3 erhalten wird, was einem durchschnittlichen Resektionsausmaß von 98 % entspricht. Innerhalb eines 7-Jahres-Zeitraums wurden 38 Patienten mit hereditärer Sphärozytose nach diesem Verfahren operiert. Wir berichten über die Operationsmethode und vergleichen diese mit anderen milzerhaltenden chirurgischen Verfahren. SchlüsselwörterSplenektomie · hereditäre Sphärozytose · Hypersplenismus · Hämolyse · OPSI-Syndrom Abstract Total splenectomy is the therapy of choice for moderate and severe forms of hereditary spherocytosis, but asplenia can lead to a lifelong risk for severe infection and critical sepsis. Partial parenchymal resections with removal of 60-90 % of enlarged spleen have been performed, but in several cases substantial regrowth of the remnant and recurrency of hemolysis were demonstrated. We hypothesized that a more radical approach would achieve a longer lasting reduction of hemolysis and remnant regrowth. Therefore we conducted a new standardized surgical technique, we call near-total splenectomy, with preservation of a remnant of 10 cm 3 size. This procedure for the management of hereditary spherocytosis was performed in 38 patients within a 7-year-period. We describe our technique, report our experience and discuss other kinds of spleen preserving procedures.
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