Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-28T00:19:08.106Z Has data issue: false hasContentIssue false
  • English
  • Français

Ectopic accessory liver masquerading as spleen in a case with isomerism of the right atrial appendages

Published online by Cambridge University Press:  19 August 2008

Siew Yen Ho ,
Simon E. Trotter  and
Andrew N. Redington
Siew Yen Ho*
Affiliation:
From the Department of Paediatrics, Royal Brompton and National Heart ' Lung Hospital, London.
Simon E. Trotter
Affiliation:
National Heart & Lung Institute, London and the Departments of Pathology, Royal Brompton and National Heart ' Lung Hospital, London
Andrew N. Redington
Affiliation:
and Paediatrics, Royal Brompton and National Heart ' Lung Hospital, London
*
Dr. Siew Yen Ho, Department of Paediatrics, National Heart & Lung Institute, Dovehouse Street, London SW3 6LY, United Kingdom. Tel. 71-351-8894; Fax. 71-352-0032.
Get access Rights & Permissions [Opens in a new window]

Summary

We report a case of an infant with isomerism of the right atrial appendages in whom a spleen-like nodule was found at post-mortem. The nodule, on histology, was found to be a fragment of liver. The cardiac lesions were typical of those associated with right isomerism, a complex generally described as the “asplenia syndrome.” The presence of either a spleen, or a nodule masquerading as a spleen, does not preclude an isomeric arrangement of the atrial appendages nor its commonly associated constellation of cardiovascular anomalies.

Keywords

Right isomerismectopic liverright atrial appendages
Type
Brief Reports
Information
Cardiology in the Young , Volume 3 , Issue 1 , January 1993 , pp. 43 - 46
Copyright
Copyright © Cambridge University Press 1993

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Ivemark, BI. Implications of agenesis of the spleen on the pathogenesis of cono-truncal anomalies in childhood. An analysis of the heart malformations in the splenic agenesis syndrome, with fourteen new cases. Acta Pediatr 1955; 44(Suppl 104): 1–110.Google Scholar
Van Mierop, LHS, Wiglesworth, FW.Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest 1962; 11: 1303–1315.Google Scholar
Ruttenberg, HD, Neufeld, HN, Lucas, RV.Syndrome of congenital cardiac disease with asplenia. Distinction from other forms of congenital cyanotic cardiac disease. Am J Cardiol 1964; 13: 387–406.CrossRefGoogle ScholarPubMed
Layman, TE, Levine, MA, Amplatz, K, Edwards, JE. “Asplenic syndrome” in association with rudimentary spleen. Am J Cardiol 1967; 20: 136–140.Google Scholar
Diehl, EJ, Landing, BH.Syndrome of pulmonary isomerism of right lung type, congenital heart disease, pulmonary and systemic venous abnormalities and mairotation of the intestine, with a spleen or multiple spleens (m-anisosplenia): Comparison with the Ivemark asplenia syndrome. Pediatr Path 1984; 2: 133–138.Google ScholarPubMed
Devine, WA.What if Ivemark had suggested the term “Syndrome ofvisceral symmetry with asplenia” instead of “Asplenia, a teratologic syndrome of visceral heterotaxy”? Cardiol Young 1992; 2: 108–113. [Editorial]CrossRefGoogle Scholar
Fock, G.Ectopic liver in omphalocele. Acta Paediatr 1963; 52: 288–292.CrossRefGoogle ScholarPubMed
Chiu, IS, How, SW, Wang, JK, Wu, MH, Chu, SH, Lue, HC, Hung, CR.Clinical implications of atrial isomerism. Br Heart J 1990; 63: 122–128.Google Scholar
Anderson, C, Devine, WA, Anderson, RH, Debich, DE, Zuberbuhler, JR.Abnormalities of the spleen in relation to congenital malformation of the heart: A survey of necropsy findings in children. Br Heart J 1990; 63: 122–128.CrossRefGoogle Scholar
Freedom, RM.Aortic valve and arch anomalies in the congenital aplasia syndrome. Case report, literature review and reexamination of the embryology of the congenital aplasia syndrome. Hopkins Med J 1974; 135: 124–135.Google Scholar