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


METHODS
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.
Department of General Pediatrics, University Children's Hospital Muenster, Münster, Germany. jan.daebritz@ukmuenster.de
Jan
J
Däbritz
Jennifer
J
Worch
Ulrike
U
Materna
Bernward
B
Koch
Gabriele
G
Koehler
Christina
C
Duck
Michael C
MC
Frühwald
Dirk
D
Foell
D001706:Biopsy; D002496:Central Venous Pressure; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D006971:Hypersplenism / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006975:Hypertension, Portal / Q000150:complications* / Q000503:physiopathology; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013156:Splenectomy