257:. If the anastomosis occurs outside of the liver, the shunt is considered to be extrahepatic. On the other hand, if the anastomosis is located within the liver, it is considered to be intrahepatic. The clinical manifestations of intra- and extra- hepatic portal systemic shunts can be similar; however the pathophysiology and treatment of the two types are distinct.
277:
as the cause of the shunt, either of which would require a specific treatment. Once the congenital, and isolated, nature of the shunt has been ascertained, closure by surgical intervention is usually recommended. Shunt closure prevents the development of complications in pre-symptomatic subjects and
168:
A CPSS results in a direct communication between the portal circulation and the systemic circulation. This breaks down the separation between these two systems which is crucial in ensuring normal physiological function. A reduction in the proportion of blood flowing from the digestive system to the
218:. In children, CPSS may present as neonatal cholestasis. These complications are generally induced by long term portosystemic derivations and are more commonly observed in children than in adults. Unexplained neurocognitive dysfunction and other behavioural issues linked to
265:
Spontaneous closure of CPSS can occur in some anatomic forms during the first two year of life. However, in instances where spontaneous closure does not occur, radiologic or surgical closure of the CPSS is recommended to prevent, resolve and/or stabilise complications.
293:) has been elaborated in order to better understand the underlying causes, as well as the signs and symptoms of CPSS. The primary aim of the registry is to "better identify patients who are at risk of developing complications and to offer them standardized care."
70:
Blood leaving the digestive tract is rich in nutrients, as well as in toxins, which under normal conditions undergo processing and detoxification in the liver. The liver's position downstream to the intestines in the body's circulatory system - the hepatic
632:[Interview with Amaria Remil, Project Manager of the AVB-CG Reference Center, and Prof. Stéphanie Franchi-Abella, Head of the pediatric interventional radiology sector (Paris-Saclay University)].
371:
Alonso-Gamarra E, ParrĂłn M, PĂ©rez A, Prieto C, Hierro L, LĂłpez-SantamarĂa M (2011-05-01). "Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review".
630:"Interview d'Amaria Remil, Cheffe de projet du Centre de Référence AVB-CG, et du Pr Stéphanie Franchi-Abella, Responsable du secteur de radiologie interventionnelle pédiatrique (Université Paris-Saclay)"
205:
are common clinical manifestations of CPSS. In adults, the discovery of a CPSS is often fortuitous but can also occur in response to the detection of one or several characteristic complications such as
804:
McLin V, Franchi-Abella S, Debray D, Korff S, Casotti V, Colledan M, et al. (April 2019). "FRI-436-Congenital porto-systemic shunts in children: Preliminary results from the IRCPSS".
181:
CPSS are thought to affect 1 in 30,000–50,000 live births. While most patients present with a single shunt, complex shunts, which include multiple abnormal vessels, are also reported.
173:
results. The coefficient of filtration is therefore reduced, less blood rich in nutrients and toxins is filtered, and an accumulation of toxins in the blood circulatory system occurs.
225:
Gastrointestinal bleeding is another common complication of PSS and has been observed in 8.1% of patients with extrahepatic portosystemic shunts. Other complications of CPSS are
58:. Improvements in imaging and awareness have contributed to an increase in cases.Thus a large part of medical and scientific literature on the subject is grounded in
54:(present at birth) or acquired condition and occurs in humans as well as in other species of animals. Congenital PSS are extremely rare in humans but are relatively
710:
Gong Y, Zhu H, Chen J, Chen Q, Ji M, Pa M, et al. (December 2015). "Congenital portosystemic shunts with and without gastrointestinal bleeding - case series".
950:
896:
Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guérin F (August 2018). "Congenital portosystemic shunts: diagnosis and treatment".
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117:
in the uterus and are present at birth. In contrast, acquired porto-systemic shunts occur after birth and typically develop secondary to
75:
conveys blood from the intestines to the liver - allows it to filter this nutrient rich blood before it passes to the rest of the body.
82:, a bypass of the liver, causes blood to flow directly to the heart. This blood is no longer filtered by the liver and reaches the
241:(presence of blood in the urine). It is generally agreed amongst specialists that the majority of CPSSs should be closed by
189:
The size of the liver in patients affected by PSS is typically 45% to 65% of the standard volume for a given age. Neonatal
17:
145:. Two distinct systems provide the liver with blood. Oxygen rich blood is sent to the liver from the heart via the
302:
55:
629:
753:
Bahadori A, Kuhlmann B, Debray D, Franchi-Abella S, Wacker J, Beghetti M, et al. (February 2022).
211:
194:
149:, while the portal vein brings nutrient rich (but depleted in oxygen) blood to the liver from the
537:
Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (November 2012).
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Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (November 2012).
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95:
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McLin VA, Franchi Abella S, Debray D, Guérin F, Beghetti M, Savale L, et al. (May 2019).
407:
DiPaola F, Trout AT, Walther AE, Gupta A, Sheridan R, Campbell KM, et al. (April 2020).
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DiPaola F, Trout AT, Walther AE, Gupta A, Sheridan R, Campbell KM, et al. (April 2020).
146:
87:
583:
Stringer MD (March 2008). "The clinical anatomy of congenital portosystemic venous shunts".
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234:
8:
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118:
59:
51:
409:"Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes"
324:"Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes"
253:
Congenital porto-systemic shunts (CPSS) are classified occurring to the position of the
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165:. The division between these two systems helps assure the liver's physiological roles.
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539:"Congenital portosystemic shunts in children: recognition, evaluation, and management"
458:"Congenital portosystemic shunts in children: recognition, evaluation, and management"
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Filfoie: tout savoir sur les maladies rares du foie, recherche, enseignement
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86:, resulting in a number of symptoms and complications with effects on the
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150:
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509:"International Registry of Congenital Portosystemic Shunts (IRCPSS)"
849:"Congenital Portosystemic Shunts: Current Diagnosis and Management"
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110:
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Upon discovery of a CPSS in a child, it is important to rule out
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370:
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before being evacuated by the hepatic veins into the inferior
755:"Presentation of Congenital Portosystemic Shunts in Children"
162:
43:
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Filfoie: all about rare liver diseases, research, teaching
233:, vaginal bleeding, and lower urinary tract symptoms like
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may reverse or stabilize signs and symptoms in patients.
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There are no major and direct communications between the
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321:
508:
853:
Journal of
Pediatric Gastroenterology and Nutrition
42:), also known as a liver shunt, is a bypass of the
661:Papamichail M, Pizanias M, Heaton N (March 2018).
287:An International Registry of Porto-Systemic Shunts
937:
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951:Syndromes affecting the hepatobiliary system
946:Congenital disorders of circulatory system
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780:
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141:and the portal, superieur mesenteric or
663:"Congenital portosystemic venous shunt"
14:
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153:. This blood passes by the network of
27:Liver bypass by the circulatory system
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113:malformations which occur during the
109:Congenital porto-systemic shunts are
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137:within the liver, nor between the
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413:Digestive Diseases and Sciences
328:Digestive Diseases and Sciences
303:Portosystemic shunts in animals
235:nephrolithiasis (kidney stones)
176:
38:(medical subject heading term;
667:European Journal of Pediatrics
449:
400:
364:
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222:occur in 17% to 30% of cases.
13:
1:
818:10.1016/s0618-8278(19)31173-9
640:] (in French). 2020-12-17
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866:10.1097/MPG.0000000000002263
260:
7:
296:
10:
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425:10.1007/s10620-019-05834-w
340:10.1007/s10620-019-05834-w
245:or surgical intervention.
115:development of an organism
910:10.1007/s00261-018-1619-8
724:10.1007/s00247-015-3417-6
679:10.1007/s00431-017-3058-x
543:Seminars in Liver Disease
462:Seminars in Liver Disease
212:hepatopulmonary syndrome
195:hepatopulmonary syndrome
772:10.3390/children9020243
556:10.1055/s-0032-1329896
475:10.1055/s-0032-1329896
220:hepatic encephalopathy
216:pulmonary hypertension
208:hepatic encephalopathy
199:pulmonary hypertension
185:Clinical manifestation
806:Journal of Hepatology
161:and subsequently the
50:. It can be either a
386:10.1148/rg.313105070
84:systemic circulation
898:Abdominal Radiology
712:Pediatric Radiology
271:portal hypertension
119:portal hypertension
60:veterinary medicine
36:portasystemic shunt
32:portosystemic shunt
18:Portosystemic shunt
104:endocrinal systems
92:neurophysiological
78:The presence of a
48:circulatory system
718:(13): 1964–1971.
282:On-going research
193:, liver tumours,
169:liver during the
96:gastro-intestinal
16:(Redirected from
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904:(8): 2023–2036.
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419:(4): 1239–1251.
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334:(4): 1239–1251.
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227:hyperandrogenism
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125:Physiopathology
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859:(5): 615–622.
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673:(3): 285–294.
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591:(2): 147–157.
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549:(4): 273–287.
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468:(4): 273–287.
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380:(3): 707–722.
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249:Classification
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203:encephalopathy
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147:hepatic artery
139:systemic veins
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88:cardiovascular
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56:common in dogs
46:by the body's
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642:. Retrieved
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512:. Retrieved
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243:radiological
231:pancreatitis
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177:Epidemiology
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128:
108:
77:
69:
39:
35:
31:
29:
812:(1): e586.
273:or hepatic
191:cholestasis
155:capillaries
73:portal vein
940:Categories
765:(2): 243.
644:2021-07-28
514:2021-07-28
309:References
275:hemangioma
255:anastomose
239:haematuria
171:first pass
151:intestines
66:Background
52:congenital
834:145951554
826:0168-8278
261:Treatment
159:vena cava
926:19116849
918:29730740
883:58578546
875:30628988
791:35204963
759:Children
740:19515262
732:26209117
697:29243189
613:42632424
605:18161055
565:23397528
484:23397528
443:31549332
394:21571652
358:31549332
297:See also
111:vascular
782:8870378
688:5816775
492:6584627
434:8180198
349:8180198
100:urinary
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291:IRCPSS
131:portal
922:S2CID
879:S2CID
830:S2CID
736:S2CID
636:[
609:S2CID
488:S2CID
163:heart
80:shunt
44:liver
914:PMID
871:PMID
822:ISSN
787:PMID
728:PMID
693:PMID
601:PMID
561:PMID
480:PMID
439:PMID
390:PMID
354:PMID
237:and
214:and
201:and
133:and
102:and
906:doi
861:doi
814:doi
777:PMC
767:doi
720:doi
683:PMC
675:doi
671:177
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551:doi
470:doi
429:PMC
421:doi
382:doi
344:PMC
336:doi
40:PSS
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