Ertan SAL, Lokman ÜSTYOL, M. Selçuk BEKTAŞ, Hayrettin TEMEL, Avni KAYA, Abdurrahman ÜNER

Polysplenia with dextrocardia and absence of the vena cavaınferior: A case report

Polispleni sendromu, abdominal, kardiyak, vasküler ve toraks anomalilerinin eşlik ettiği nadir konjenital anomalidir. Tanı, genellikle çocukluk döneminde kardiyak şikayetler nedeniyle yapılan radyolojik incelemeler sonucunda konulur. Bu makalede triküspit kapak yetmezliği, dekstrokardi, polispleni, genişlemiş vena azigos, pankreas corpus ve kuyruk kısmı olmayan ve vena cava inferior yokluğu olan altı yaşındaki erkek olgusu sunuldu. Sonuçta bu sendromdaki anomalilerin bilinmesi kısa pankreasın ya da dalakların kitle olarak yorumlanmasını önleyecektir.

Polispleni, dekstrokardi ve vena cava inferior yokluğu: Olgu sunumu

Polysplenia Syndrome is a rare congenital anomaly accompanied by abdominal, cardiac, vascular and thorax abnormalities. This condition is commonly diagnosed after radiological studies due to childhood cardiac complaints. In this study, a 6-year-old boy patient with tricuspid valve failure, dextrocardia, polysplenia, widening vena azygos and absence of vena cava inferior, pancreas corpus and tail cut is presented. If the anomalies in thissyndrome are identified then it will be possible to avoid an understanding as a mass of short pancreas or spleens.

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1. Winer-Muram HT, Tonkin ILD. Spectrum of heterotaxic syndromes. Radiol Clin North Am 1989; 27: 1147-1170.
2. Peoples WM, Moller JH, Edward JE. Polysplenia: A review of 146 cases. Pediatr Cardiol 1983; 4: 129-137.
3. Roguin N, Hammerman H, Korman S, Riss E. Angiography of azygos continuation of inferior vena cava in situs ambiguus with left isomerism (polysplenia syndrome) Pediatr Radiol 1984; 14: 109–112.
4. Winer-Muram HT. Adult presentation of heterotaxic syndromes and related complexes. J Thorac Imaging 1995; 10: 43–57.
5. Ahmetoğlu A, Koşucu P, Sarı A, Gümele H.R. Polisipleni sendromunda radyolojik bulgular. Tanısal ve Girişimsel radyoloji 2002; 8: 510-512.
6. Kapa S, Gleeson F.C, Vege S.S. Dorsal pankreas agenezis and polysplenia/heterotaxy syndrome. JOP 2007; 8: 433-437.
7. Van de Pere S, Vanhoenacker F.M, Petre C, Van Doorn C, De Schepper A.M. Heterotaxy Syndrome JBR-BTR 2004; 87: 158-159.
8. Schulman M.H. Asplenia/polysplenia. Emecidine World Medical Library. (http://emedicine.medscape.com/article/406116-overview) (Updated: Jun 17, 2009)
9. Gayer G, Apter S, Jonas T, et al. Polysplenia syndrome detected in adulthood: Report of eight cases and review of the literature. Abdom Imaging 1999; 24: 178-184.
10. Soler R, Rodriquez E, Comesana ML, Pombo F, Marini M. Agenesis of dorsal pancreas with polysplenia syndrome: CT features. J Comput Assist Tomogr 1992; 15: 921-923.