Review ArticleThe classification based on intrahepatic portal system for congenital portosystemic shunts☆,☆☆
Section snippets
Patients and methods
The medical records of the patients included in this study were reviewed, and data were collected retrospectively. Between November 2002 and October 2013, 18 of the total of 19 patients with CPS could undergo angiography with the shunt occlusion test at the National Center for Child Health and Development (Tokyo, Japan). Twelve patients had been diagnosed with CPS type I, five with CPS type II and two patients were diagnosed with a patent ductus venosus (PDV) by abdominal sonography and CECT
Patients and clinical findings
The profiles of the 19 total patients (nine males and 10 females) are summarized in Table 1. All cases diagnosed were to have CPS, including PDV, by CECT and abdominal sonography before undergoing the angiographic examination. The median age of the patients was 6.8 years (range, 1.0–17.0 years) at the time of the angiographic study performed to provide more detailed information for determining the treatment strategy. This study found that all patients had some clinical and subclinical symptoms.
Discussion
The radiological balloon occlusion test found that a hypoplastic portal system existed in CPS with Morgan's classification type I, which had previously been considered to require liver transplantation. The classification of the severity of the hypoplasia of IHPS had not been described in the previously published reports, but appears to be useful for stratifying patients. We herein distinguished three types of CPS according to the severity of hypoplasia of the IHPS, and evaluated their features
Conclusion
The intrahepatic and extrahepatic portal systems could be visualized by the shunt occlusion test even in patients who had been diagnosed as CPS type I by means of abdominal sonography or CECT. We proposed our classification based on the severity of the hypoplasia of IHPS. The IHPS classification reflects the clinicopathological characteristics of CPS, and was useful for selecting the therapeutic approach and management.
Acknowledgment
We would like to thank Dr. Hiroshi Terashima and Dr. Masaya Kubota from the Department of Neurology, and Dr. Masatoshi Matsunami, Dr. Kengo Sasaki, and Dr. Hajime Uchida from Transplant Center, National Center for Child Health & Development, for consultation for neurological assessments.
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2022, Clinical ImagingCitation Excerpt :These shunts are divided into two categories: Type I (complete) which are complete shunts with no perfusion to liver parenchyma and type II (partial) with some perfusion to the liver.1 Other classifications are based on location of the shunt in relation to the liver as well as presence or absence of the portal vein,2 origin of the shunt,3 severity of the shunt,4 systemic drainage site5 or combination of number and pattern of communications, shunt origin, and systemic communication.6 Current recommendations for treatment of extrahepatic shunts is early closure before one year of age to avoid complications.7
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Conflict of interest: None of the authors have any conflict of interest to declare concerning the present manuscript. This work was supported in part by a Grant from the National Center for Child Health and Development, Japan (24-08, 25-06).
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Authors' contribution: H. Kanazawa (research design, writing of the paper, performance of the research, provision of reagents and analytic tools, data analysis), S. Nosaka (performance of the research, provision of reagents and analytic tools, data analysis), O. Miyazaki (performance of the research), S. Sakamoto (research design, writing of the paper, performance of the research), A. Fukuda (research design, performance of the research), T. Shigeta (performance of the research), A. Nakazawa (performance of the research, provision of reagents and analytic tools, data analysis), M. Kasahara (research design, performance of the research, writing of the paper).