Fatal pneumococcal septicemia in a girl with visceral heterotaxy and polysplenia: a case report
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CASE REPORT
Fatal pneumococcal septicemia in a girl with visceral heterotaxy and polysplenia: a case report Ingo von Both 1,2 & Michael S. Pollanen 2,3 Accepted: 13 April 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract We report an unusual case of a 15-month old previously healthy girl who died of pneumococcal septicemia in the background of visceral heterotaxy with polysplenia. Heterotaxy can also present with asplenia whereas polysplenia cases usually present with functional asplenia. Of particular note, this girl received the 13-valent pneumococcal conjugate vaccine as recommended by the Centers for Disease Control and Prevention in the routine pediatric immunization schedule used in the USA and Canada. Unfortunately, although the strain causing death (serotype 22F) is not contained in Prevnar 13®, it is in the 23-valent pneumococcal polysaccharide vaccine (e.g. Pneumovax 23®), currently suggested only for immunocompromised children age 2 with either functional or anatomic asplenia. This syndrome has the potential of being diagnosed prenatally. The intent of our case report is to raise awareness of the syndrome, highlight that heterotaxy patients with polysplenia are at danger for infections with encapsulated organism, such as pneumococcus, meningococcus, and Haemophilus influenza amongst others due to functional asplenia, recommend the 23-valent pneumococcal polysaccharide vaccine for these children before age two for the outlined reasons, and illustrate that with early diagnosis of the heterotaxy syndrome, and early diagnosis and treatment of septic complications, the morbidity or death of young children with heterotaxy syndrome can likely be reduced or prevented. Keywords Pneumococcus . Pneumococcal septicemia . Visceral heterotaxy . Polysplenia . Functional asplenia
Introduction We report a case of a 15-month old child who died suddenly and unexpectedly of Streptococcus pneumoniae sepsis. Autopsy revealed the incidental finding of visceral heterotaxy with polysplenia. Pneumococcus serotype (22F) was isolated from lung and spleen tissue. This girl had received a vaccine currently suggested for infants in the USA and Canada to prevent pneumococcal infections (PCV-13; e.g. Prevnar 13®), but serotype 22F is only part of the PPSV23 vaccine (e.g. Pneumovax 23®) recommended for older children and
* Michael S. Pollanen [email protected] 1
Provincial Forensic Pathology Unit, Ontario Forensic Pathology Service, 25 Morton Shulman Avenue, Toronto, Ontario M3M 0B1, Canada
2
Department of Laboratory Medicine & Pathobiology, University of Toronto, Toronto, Canada
3
Ontario Forensic Pathology Service, 25 Morton Shulman Avenue, Toronto, Ontario M3M 0B1, Canada
adults with immune compromise. Awareness of the syndrome and associated risks as well as right treatment with possible early diagnosis in utero will aid in preventing infections from possibly fatal encapsulated organisms in this susceptible patient group.
Case report History The decedent was a 15-month old female
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