Liver transplantation in children with biliary atresia and polysplenia syndrome

Falchetti, Diego; Carvalho, Filipa; Clapuyt, P.; Goyet, Jean de Ville de; Hemptinne, Bernard de; Claus, D.; Otté, Jean-Bernard

doi:10.1016/0022-3468(91)90698-s

Cited by 62 publications

(25 citation statements)

References 17 publications

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“…2,4 Other vascular anomalies which are rarely described in cases of PS are transhepatic portal vein, hepatic artery originating from the superior mesenteric artery, circumaortic renal vein, portal vein hypoplasia or the absence of portal vein. [8][9][10][11] The hepatic segment of the inferior vena cava was not observed in this case and the inferior vena cava demonstrated continuity with the dilated azygos vein. The dilated azygos vein results from the increased blood flow to the inferior vena cava.…”

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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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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“…EHBA is the most common indication for liver transplantation in children and is associated with a number of congenital anomalies including polysplenia, situs inversus, intestinal malrotation, portal vein anomalies, caval interruption and cardiac malformations. The most common of these is polysplenia syndrome (4–6). Cardiac anomalies were originally considered a contraindication to liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

Liver transplantation for syndromic biliary atresia with a pedunculated accessory hepatic lobe

Faraj

Dar

Marangoni

et al. 2010

Pediatric Transplantation

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“…Although PDPV is a rare anomaly, its presence should be kept in mind by pediatric surgeons undertaking a laparotomy in a patient with high intestinal obstruction, especially in those affected by malrotation or abnormal position of viscera. Its aberrant anatomical position puts it at risk for iatrogenic injury particularly during liver transplantation or surgical correction of biliary atresia [9]. From the pediatric surgical reports, it is difficult to assess a long-term follow-up of the corrected anomaly.…”

Section: E28mentioning

confidence: 99%