Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature
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
BMC Gastroenterology 2010, 10:122 doi:10.1186/1471-230X-10-122Published: 20 October 2010
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.
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.
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.