Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt.
BACKGROUND
Congenital portosystemic shunt (CPSS) has the potential to cause hepatic encephalopathy and thus needs long-term follow-up, but an effective follow-up method has not yet been established. We aimed to evaluate the importance of per-rectal portal scintigraphy (PRPS) for long-term follow-up of CPSS.


METHODS
We retrospectively examined shunt severity time course in patients (median: 9.6 y, range: 5.2-16.6 y) with intrahepatic (n = 3) or extrahepatic (n = 3) CPSS by using blood tests, ultrasonography or computed tomography, and PRPS. Per-rectal portal shunt index (cutoff: 10%) was calculated by PRPS.


RESULTS
PRPS demonstrated that the initial shunt index was reduced in all intrahepatic cases (from 39.7 ± 9.8% (mean ± SD) to 14.6 ± 4.7%) and all extrahepatic cases (from 46.2 ± 10.9 to 27.5 ± 12.6%) during the follow-up period. However, ultrasonography and computed tomography disclosed different shunt diameter time courses between intrahepatic and extrahepatic CPSSs. Initial shunt diameter (5.8 ± 3.5 mm) reduced to 2.0 ± 0.3 mm in intrahepatic cases, but the initial diameter (6.3 ± 0.7 mm) increased to 10.6 ± 1.0 mm in extrahepatic cases. All patients had elevated serum total bile acid or ammonia levels at initial screening, but these blood parameters were insufficient to assess shunt severity because the values fluctuate.


CONCLUSIONS
PRPS can track changes in the shunt severity of CPSS and is more reliable than ultrasonography and computed tomography in patients with extrahepatic CPSS.
Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan.
Yuki
Y
Cho
Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan.
Daisuke
D
Tokuhara
Department of Radiology, Osaka City University Graduate School of Medicine, Osaka, Japan.
Taro
T
Shimono
Department of Radiology, Osaka City University Graduate School of Medicine, Osaka, Japan.
Akira
A
Yamamoto
Department of Nuclear Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.
Shigeaki
S
Higashiyama
Department of Nuclear Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.
Kohei
K
Kotani
Department of Nuclear Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.
Joji
J
Kawabe
Department of Genetics, Hyogo College of Medicine, Hyogo, Japan.
Yoshiyuki
Y
Okano
Department of Nuclear Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.
Susumu
S
Shiomi
Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan.
Haruo
H
Shintaku
D000293:Adolescent; D002648:Child; D005500:Follow-Up Studies; D006501:Hepatic Encephalopathy / Q000097:blood / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006801:Humans; D008297:Male; D011169:Portal Vein / Q000000981:diagnostic imaging* / Q000473:pathology; D011877:Radionuclide Imaging / Q000379:methods*; D012007:Rectum / Q000473:pathology; D012189:Retrospective Studies; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D054079:Vascular Malformations / Q000000981:diagnostic imaging* / Q000503:physiopathology