Congenital extrahepatic portocaval shunts—The Abernethy Malformation

Howard, E. R.; Davenport, Mark

doi:10.1016/s0022-3468(97)90614-x

Cited by 242 publications

(232 citation statements)

References 15 publications

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“…However, there are situations in which such a procedure is very hazardous. Portocaval hemi-transposition is another reported technique for overcoming portal thrombosis and is also helpful in cases of congenital absence of the portal vein [7]. In such cases, impairment of renal function and perfusion of the lower limbs would be very important.…”

Section: Resultsmentioning

confidence: 99%

Portal vein thrombosis: an emergency solution for blood flow in liver transplantation

Bernardos¹,

Serrano²,

Gómez³

et al. 2003

Transplant International

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Section: Resultsmentioning

confidence: 99%

Portal vein thrombosis: an emergency solution for blood flow in liver transplantation

Bernardos¹,

Serrano²,

Gómez³

et al. 2003

Transplant International

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“…Congenital absence of the portal vein and its association with polysplenia is very rarely encountered in the literature. [11][12][13] Congenital absence of the portal vein leads to a complete interruption of mesentericoportal flow and development of a large portosystemic shunt, but not portal cavernoma. 14 In the cases with a large portosystemic shunt, there is no portal hypertension or ascites.…”

Section: Discussionmentioning

confidence: 99%

A Case of Polysplenia Syndrome Presenting with Ascites

Fidan¹,

Erkut²,

Demirel³

et al. 2012

Turkiye Klinikleri J Med Sci

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A AB BS ST TR RA AC CT T Polysplenia syndrome is a rare congenital anomaly frequently associated with various visceral anomalies. The most frequent manifestations of this syndrome, in addition to polysplenia are malrotation, congenital heart diseases and gastrointestinal, genitourinary and vascular abnormalities. In this report, we present an adult case of ascites due to prehepatic portal hypertension associated with portal cavernous transformation. The patient had a left-sided liver, right-sided multiple splenules and stomach, interrupted inferior vena cava with azygos continuation and extensive ascites. The portal vein was not observed and collateral vascular structures of cavernous transformation were identified in the hepatic hilus. Portal cavernous transformation with various congenital abnormalities has been reported in the literature; however, there are no cases of polysplenia syndrome presenting with ascites associated with portal cavernous transformation.K Ke ey y W Wo or rd ds s: : Ascites; situs inversus; spleen Ö ÖZ ZE ET T Polispleni sendromu sıklıkla çeşitli organ anomalileri ile ilişkili nadir bir konjenital anomalidir. Polispleniye ek olarak bu sendromun en sık görülen belirtileri malrotasyon, konjenital kalp hastalıkları, genitoüriner, gastrointestinal ve vasküler anomalilerdir. Bu yazıda, portal kavernöz transformasyon ile ilişkili prehepatik portal hipertansiyona bağlı asit gelişmiş olan erişkin bir olgu sunulmuştur. Hastada sol taraflı karaciğer, sağ taraflı çok sayıda aksesuar dalak ve mide, azygos devamlılığı ile kesintiye uğramış inferior vena kava ve yaygın asit vardı. Portal ven gözlenmedi ve karaciğer hilusunda kavernöz transformasyonlu kollateral vasküler yapılar tespit edildi. Literatürde portal kavernöz transformasyon ile beraber çeşitli konjenital anomaliler mevcuttur, ancak asit ile gelen portal kavernöz transformasyon ile ilişkili polispleni sendromu olgusu henüz bildirilmemiştir.A An na ah ht ta ar r K Ke el li im me el le er r: : Asit; situs inversus; dalak T Tu ur rk ki iy ye e K Kl li in ni ik kl le er ri i J J M Me ed d S Sc ci i 2 20 01 12 2; ;3 32 2(

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“…In such patients, liver transplantation is the only potentially curative treatment (6,15,16). 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.…”

Section: Discussionmentioning

confidence: 99%

“…Eroglu, et al reported damage incurred during development in childhood could reduce the functional capacity of the adult (14). These shunts should be preemptively occluded by either surgical or radiological intervention after ruling out severe associated anomalies such as cardiac defects or maldevelopment of the intrahepatic portal as well as hepatic veins (4,6,7).…”

Section: Discussionmentioning

confidence: 99%