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Splenic state in surviving patients with visceral heterotaxy

Published online by Cambridge University Press:  16 September 2005

Bert H. P. Nagel ,
Helen Williams ,
Louise Stewart ,
Joanne Paul  and
Oliver Stümper
Bert H. P. Nagel
Affiliation:
Department of Paediatric Cardiology, University Children's Hospital, Essen, Germany
Helen Williams
Affiliation:
Department of Paediatric Radiology, Birmingham Children's Hospital-NHS Trust, Birmingham, United Kingdom
Louise Stewart
Affiliation:
Department of Paediatric Radiology, Birmingham Children's Hospital-NHS Trust, Birmingham, United Kingdom
Joanne Paul
Affiliation:
The Heart Unit, Birmingham Children's Hospital-NHS Trust, Birmingham, United Kingdom
Oliver Stümper
Affiliation:
The Heart Unit, Birmingham Children's Hospital-NHS Trust, Birmingham, United Kingdom
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Abstract

Aim: To identify patients with visceral heterotaxy who are at risk from fulminant sepsis. Methods: We studied 38 patients, 37 having undergone abdominal ultrasound, all 38 having examination of blood films to establish presence of Howell-Jolly bodies, and all 38 documented to have had pneumococcal vaccination and prophylaxis with penicillin. We checked whether the parents were aware of the splenic state of their child, and when possible, we compared current results of blood films with those obtained postnatally. Results: Two of the 17 patients with multiple spleens, all 11 without a detectable spleen, and 1 of 9 patients with a normal spleen, showed Howell-Jolly bodies in their blood films. In 5 of 23 patients with serial blood films, Howell-Jolly bodies had not been seen postnatally, but could now be detected in current blood films. Of these patients, 2 had multiple spleens, 1 did not have a spleen, and 1 had a solitary spleen of normal size. In the other patient, ultrasound could not be performed. Only one of these patients was receiving penicillin prophylactically, and had received pneumococcal vaccination. Of the 15 patients in whom Howell-Jolly bodies were present in the blood, only 8 parents knew about the potential risk for infection. Another 7 parents were sure that their child was taking penicillin regularly, and had received pneumococcal vaccination. Conclusions: 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.

Keywords

Isomerismvisceral heterotaxyfunctional aspleniapolyspleniaHowell-Jolly bodies
Type
Original Article
Information
Cardiology in the Young , Volume 15 , Issue 5 , October 2005 , pp. 469 - 473
Copyright
© 2005 Cambridge University Press

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References

Anderson RH, Becker AE. Hearts with isomeric atrial appendages. In: Anderson RH, Becker AE (eds). Controversies in the Description of Congenitally Malformed Hearts. Imperial College Press, London, 1997, pp 67–112.
Uemura H, Yen Ho S, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.Google Scholar
Cheung YF, Cheng VYW, Chau AKT, Chiu CSW, Yung TC, Leung MP. Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage? Heart 2002; 87: 146–152.Google Scholar
Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000; 36: 908–916.Google Scholar
Hashmi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31: 1120–1126.Google Scholar
Waldmann JD, Rosenthal A, Smith AL, Shurin S, Nadas AS. Sepsis and congenital asplenia. J Pediatr 1977; 90: 555–559.Google Scholar
Wu M-H, Wang J-K, Lue H-C. Sudden death in patients with right isomerism (asplenism) after palliation. J Pediatr 2002; 140: 93–96.Google Scholar
Huhta JC, Smallhorn JF, Macartney FJ. Two dimensional echocardiographic diagnosis of situs. Br Heart J 1982; 48: 97–108.Google Scholar
Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD. Normal splenic size in infants and children: sonographic measurements. AJR 1991; 157: 119–121.Google Scholar
Siniluoto TMJ, Tikkakoski TA, Lähde ST, Päivänsalo MJ, Koivisto MJ. Ultrasound or CT in splenic diseases? Acta Radiologica 1994; 35: 597–605.Google Scholar
Oleszczuk-Raschke K, Set PAK, von Lengerke HJ, Tröger J. Abdominal sonography in the evaluation of heterotaxy in children. Pediatr Radiol 1995; 25: S150–156.Google Scholar
Pearson HA. The spleen and disturbances of splenic function. In: Nathan DG, Orkin SH (eds). Hematology of Infancy and Childhood. WB Saunders Company, Philadelphia, 1998, pp 1051–1068.
Corazza GR, Ginaldi L, Zoli G, et al. Howell-Jolly body counting as a measure of splenic function. A reassessment. Clin Lab Haematol 1990; 12: 269–275.Google Scholar
Rodin AE, Sloane JA, Nghiem QX. Polysplenia with severe congenital heart disease and Howell Jolly Bodies. Am J Clin Pathol 1972; 58: 127–134.Google Scholar
Pearson HA, Schiebler GL, Spencer RP. Functional hyposplenia in cyanotic congenital heart disease. Pediatrics 1971; 48: 277–280.Google Scholar
Nathan DG. Rubbish in the red cell. N Engl J Med 1969; 281: 558–559.Google Scholar
Padmanabhan J, Risemberg HM, Rowe RD. Howell Jolly Bodies in the peripheral blood of full-term and premature neonates. Hopkins Med J 1972; 132: 146–150.Google Scholar
Murdoch IA, Dos Anjos R, Mitchell A. Fatal pneumococcal septicaemia associated with asplenia and isomerism of the right atrial appendages. Br Heart J 1991; 65: 102–103.Google Scholar
American Academy of Pediatrics Policy statement: Recommendations for the prevention of pneumococcal infections, including the use of pneumococcal conjugate vaccine, pneumococcal polysaccharide vaccine and antibiotic prophylaxis. Pediatrics 2000; 106: 362–366.
American Academy of Pediatrics. Technical report: Prevention of pneumococcal infections, including the use of pneumococcal conjugate and polysaccharide vaccines and antibiotic prophylaxis. Pediatrics 2000; 106: 367–376.
Finn A, Booy R, Moxon R, Sharland M, Heath P. Should the new pneumococcal vaccine be used in high-risk children? Arch Dis Child 2002; 87: 18–21.Google Scholar
Rubin LG. Pneumococcal vaccine. Pediatr Clin North Am 2000; 47: 269–285.Google Scholar