The long-term prognosis of congenital portosystemic venous shunt

Uchino, Tetsuya; Matsuda, Ichiro; Endo, Fumio

doi:10.1016/s0022-3476(99)70031-4

Cited by 131 publications

(124 citation statements)

References 15 publications

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“…Various imaging modalities and blood tests indicated that the natural histories of intrahepatic and extrahepatic CPSSs differed. Intrahepatic CPSS without hepatic tumor has been reported to spontaneously close or regress, whereas extrahepatic CPSS does not spontaneously regress (3,7,8,(16)(17)(18). Ultrasonography and dynamic contrast-enhanced CT evaluation showed spontaneous reductions in the shunt size in all of our patients with intrahepatic CPSS but in none with extrahepatic CPSS, strengthening the previous findings.…”

Section: Discussionsupporting

confidence: 86%

“…Shiomi et al (19) reported the usefulness of PRPS in providing detailed information about changes in portal hemodynamics; they reported that SI in adults increases as liver cirrhosis progresses. Uchino et al (3) reported that the risk of hepatic encephalopathy increases with the degree of portosystemic shunting, as indicated by the PRPS SI. The SI has also been shown to be useful for evaluating the postoperative course in dogs with extrahepatic CPSS (22).…”

Section: Discussionmentioning

confidence: 99%

“…c ongenital portosystemic shunt (CPSS), which is a major cause of neonatal hypergalactosemia without galactosemetabolizing-enzyme deficiency (1), causes brain manganese deposition, pulmonary hypertension, and hyperammonemia leading to hepatic encephalopathy (2)(3)(4)(5)(6)(7)(8). CPSS is generally suspected if serum total bile acid (TBA) and ammonia levels are elevated, and it is diagnosed by using color Doppler ultrasonography, dynamic contrast-enhanced computed tomography (CT), and per-rectal portal scintigraphy (PRPS) (7,(9)(10)(11)(12).…”

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confidence: 99%

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Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt

et al. 2014

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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. conclusion: PRPS can track changes in the shunt severity of CPSS and is more reliable than ultrasonography and computed tomography in patients with extrahepatic CPSS.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt

et al. 2014

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“…On the other hand, in type 2 patients, there is a possibility that a patient can enjoy a favorable life without any consequences by a simple shunt occlusion. Uchino, et al identified 51 cases of congenital portosystemic shunt in Japan ; 34 of the intrahepatic type and 17 of the extrahepatic type, of which spontaneous closure was occurred only in the intrahepatic type, but not in the extrahepatic type (17). If patients with CEPS are left untreated, they will sustain various consequences such as repeated encephalopathy (7,18), neurocognitive dysfunction (14), pulmonary hypertension (19), hepatopulmonary syndrome (9), cataract (3), liver atrophy (16), liver tumors (10,14) and so on.…”

Section: Discussionmentioning

confidence: 99%

Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children

et al. 2009

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“…Moreover, another plausible one is that the thin anastomoses at birth slowly become large as the patient ages. The shunt ratio may play a certain role in the occurrence of symptoms [100] . Certain special unknown mechanisms underlying CAPV lead to the delayed PSE.…”

Section: Psementioning

confidence: 99%