Polysplenia Syndrome:A Rare Case Report with Computerized Tomography Findings

Avcu, Serhat; Arslan, Harun; Ünal, Özkan

doi:10.5336/medsci.2008-7196

Cited by 1 publication

(1 citation statement)

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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.…”

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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