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Open Access Highly Accessed Case report

Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature

Jan Däbritz1*, Jennifer Worch2, Ulrike Materna3, Bernward Koch4, Gabriele Koehler5, Christina Duck1, Michael C Frühwald2 and Dirk Foell1

Author Affiliations

1 Department of General Pediatrics, University Children's Hospital Muenster, Münster, Germany

2 Department of Pediatric Hematology and Oncology, University Children's Hospital Muenster, Münster, Germany

3 Department of Radiology, University Hospital Muenster, Münster, Germany

4 Department of Pediatric Surgery, University Hospital Muenster, Münster, Germany

5 Department of Pathology, University Hospital Muenster, Münster, Germany

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BMC Gastroenterology 2010, 10:122  doi:10.1186/1471-230X-10-122

Published: 20 October 2010

Abstract

Background

Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings.

Case Presentation

We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy.

Conclusions

Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.