9 Acute Splenic Sequestration Crisis in an Infant With Sickle Cell Trait

Abstract

Only a few case reports in the pediatric population describe sickle cell trait (SCT)-associated deaths. We report a fatal case of acute splenic sequestration crisis in an infant with SCT and discuss postmortem diagnostic challenges associated with this condition. The deceased is a 4-month-old black boy with a history of prematurity (delivered at 34 weeks) and low birth weight (1.93 kg). The family was observed by child protective services. According to the caretaker, the child was placed to sleep in the same bed with his parents and another child. Several hours later, he was found unresponsive and pronounced dead on arrival in the ER. Illicit drug paraphernalia was present at the scene. Witness stories were inconsistent regarding child supervision throughout the day. At autopsy, external examination showed a well-developed, well-nourished infant. Gross findings included enlarged congested spleen (35 g, expected 16 +/- 6), and a small focus of leptomeningeal subarachnoid hemorrhage. Histology showed expansion of the red pulp with expanded sinusoids packed with sickled red blood cells in the spleen, and scattered fibrin thrombi with focal necrosis in lungs. No evidence of injury was found. Toxicology and blood cultures were negative. Vitreous fluid analysis was consistent with dehydration. Postmortem beta globin gene sequencing confirmed heterozygous hemoglobin S. Cause of death was attributed to acute splenic sequestration crisis due to SCT. Because unsafe sleep environment could not be excluded as a contributing factor, the manner of death could not be determined. Hypoxia, acidosis, dehydration, and fever can induce sickling in SCT, cumulating in fatal outcome. In a forensic setting, complete medical history is often unavailable, and diagnosis depends on meticulous examination of histologic sections and postmortem confirmatory testing. In suspicious cases, hemoglobin electrophoresis should be ordered on blood at the time of autopsy, as there is a time limitation for postmortem testing.