Splenic state in surviving patients with visceral heterotaxy

Abstract

Howell-Jolly bodies can be found in the blood of patients with visceral heterotaxy independent of the anatomical state of the spleen. As Howell-Jolly bodies can be encountered in the blood of such patients with increasing age, those with multiple and solitary spleens should be monitored regularly to identify those at risk. Parental knowledge of the splenic state, and compliance for prophylaxis using penicillin, and pneumococcal vaccination, were unsatisfactory in our cohort.

Practice variability in management of infectious issues in heterotaxy: A survey of pediatric cardiologists

“…In this instance only 2% would assume normal function and perform no additional testing despite evidence that functional asplenia may be present in this setting. 16 While definition of splenic anatomy via ultrasound may add valuable clinical information, this does not accurately predict those at risk for bacteremia and subsequent serious bacterial illness. Therefore, functional splenic testing may be necessary to predict those requiring antibiotic prophylaxis and more aggressive work up of fever.…”

Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis

“…Accordingly, when taken alongside the extracardiac abnormalities, such as splenic dysfunctions, prognosis tends to be poor compared with those with left isomerism although even those with multiple spleens can exhibit splenic dysfunction. [171819] Double inlet to a functionally univentricular heart through a common atrioventricular valve is much more frequent in association with the right than the left isomerism. [1020] In our previous case series of patients with dominant right ventricles and common inlet,[20] all of the incomplete left ventricles were lying posterior to the dominant chamber, irrespective of whether they were right or left sided.…”

Development of quality metrics for ambulatory pediatric cardiology: Infection prevention

“…Importantly, the presence of splenic tissue does not rule out hyposplenism and the accompanying risk of infectious complications, and tests of splenic function are thus indicated for patients with identified splenic tissue. 25,26 Tests of splenic function may include assessment of the blood smear for Howell-Jolly bodies, quantification of pitted RBCs by interference contrast microscopy, and heat-damaged 99m Tc-labelled RBC scan. 27,28 Importantly, both Howell-Jolly bodies and pitted red blood cells can be seen in normal newborns up to 2 months of age.…”