Congenital asplenia and group B streptococcus sepsis in the adult: case report and review of the literature

Abstract

Asplenia is associated with an increased incidence of fatal and life-threatening sepsis caused by encapsulated pathogens. Isolated congenital asplenia is a very rare condition, with only 33 cases reported in the literature. The authors report another case of this condition complicated by overwhelming Group B streptococcus sepsis secondary to paronychia that was managed successfully.

Congenital Asplenia Revealed by Streptococcus oralis septicemia: Case Report

“…Congenital asplenia is also appears to be heterogeneous in its inheritance as, although the majority of families reported show autosomal dominant inheritance, there are at least two families in the literature now which demonstrate autosomal recessive inheritance [4]. Conversely of our patient, only seven patients reported in the literature were older than 20 years of age [1]. Streptococcus pneumoniae is responsible for up to 80% of asplenia followed by sepsis cases.…”

“…This case of streptococcal septicemia in a previously well man illustrates how infection can rapidly progress in patients who have a congenital asplenia. Congenital asplenia is most often found in association with other anomalies [1]. The most common of these anomalies is Ivemark syndrome.…”

“…Congenital asplenia is often occurs in the context of a recognised malformation syndrome, the Ivemark syndrome, also called asplenia syndrome [2,3]. Conversely, isolated congenital asplenia is rare, with only 33 cases reported in the literature [1]. Different modes of inheritance have been reported for these syndromes, mostly autosomal recessive, exceptionally autosomal dominant or X-linked [4].…”

“…Different modes of inheritance have been reported for these syndromes, mostly autosomal recessive, exceptionally autosomal dominant or X-linked [4]. Most of these presented in the early years of life and only seven cases were diagnosed in the adult [1]. Commonly, the infections strictly correlated to the absence of the spleen are due to encapsulated bacteria that are the most virulent pathogens in this set of patients [5].…”

“…Asplenia is an uncommon condition that may be acquired following surgery, functional or congenital [1]. Congenital asplenia is often occurs in the context of a recognised malformation syndrome, the Ivemark syndrome, also called asplenia syndrome [2,3].…”