Abstract
BackgroundIdiopathic 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 PresentationWe 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.ConclusionsOur 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.
Highlights
Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia
ET-1 was detectable in only a few mononuclear leukocytes in the red pulp of IPH spleens [30]. These results suggest that NO liberated in spleen in the presence of low ET-1, is responsible for the dilatation of splenic sinuses, leading to splenomegaly and thereby contributing to portal hypertension in IPH [9]
We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and show that disease remission after splenectomy in early childhood points to the spleen as the main organ underlying pathogenesis and progression of IPH
Summary
The success of surgical interventions targeting hypersplenism suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. This view of the pathophysiology is supported by the fact that i) liver function abnormalities do not occur in IPH, ii) abnormal eNOS and VCAM-1 expression was only seen in splenic but not hepatic tissue, iii) the signs of portal hypertension resolved in our patient after splenectomy, and iv) both the adult patients reported before and our young patient have not developed recurrent signs of portal hypertension, liver pathologies or complications after splenectomy. Competing interests The authors declare that they have no competing interests Received: 20 January 2010 Accepted: 20 October 2010 Published: 20 October 2010
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