Plata-Muñoz JJ, Hernández-Ramírez D, Anthón FJ, Podgaetz E, Avila-Flores F, Chan C

Language: English
References: 10
Page: 114-117
PDF size: 171.54 Kb.

Text Extraction

Aims: To report a case of polysplenia syndrome (PSS) in an adult patient. Background: The PSS is a form of situs ambiguos with multiple spleen, cardiac anomalies, abdominal heterotaxia, short pancreas, major venous system and bronquial malformations. It is a rare syndrome, more often found in childhood, and only the 10% of the patients that do not have cardiac anomalies can reach adulthood.
Results: A 56 y/o male with obstructive jaundice and intestinal obstruction who was submitted to an abdominal laparotomy suspecting cholangiocarcinoma. He had choledocolithiasis, duodenal kinking by a preduodenal portal vein, intestinal levorotation, hypoplasic vena cava with a prominent acigos vein, short pancreas and polysplenia. A cholecistectomy, biliodigestive and gastroyeyunal bypasses were performed without any complications and with a succesful evolution.
Conclusions: In conclusion, PSS is a rare hereditary syndrome that often occurs in childhood and its discovery in an adult is frequently fortuitus. Surgical treatment is an excellent therapeutic option, however is reserved just for complications. The outcome is good and the final evolution depends on the degree of the cardiac anomalies.

REFERENCES

1. Vaughan TI, Hawkins LP, Elliot LP. Diagnosis of polysplenia syndrome. Radiology 1971; 101: 511-518.

2. Gayer G, Apter SS, Jonas T, et al. Polyspenia syndrome detected in adulthood. Report of eight cases with review of the literature. Abdominal Imaging 1999; 24: 178-184.

3. Vossen PG, Van Hedent EF, Degryse HR ,et al. Computed tomography of the polysplenia syndrome in the adult. Gastrointestinal Radiology 1987; 12: 209-211.

4. Mclhenny. Biliary atresia and polysplenia syndrome. AJR 1996: 271-272.

5. Abramson SJ, Berdon WE, Altman RP, et al. Biliary atresia and non cardiac polisplenic syndrome: US and surgical considerations. Radiology 1987; 163: 377-379.

6. Ito K, Matsunaga N, Mitchell D, et al. Imaging of congenital abnormalities of the portal venous system. AJR 1997; 168: 233-237.

7. Li CS, Tu HY, Chen RC, Yang MT, Ting CCI. Polysplenia syndrome with preduodenal portal vein and short pancreas: incidental findings in a case of CBD adenocarcinoma. Chin J Radiol 2001; 26: 269-274.

8. Sener RN, Alper H. Polysplenia syndrome: A case associated with transhepatic portal vein, short pancreas anf left inferior vena cava with hemiazygous continuation. Abdominal Imaging 1994; 19: 64-66 .

9. Hadar H, Gadoth N, Herskovitz P, et al. Short pancreas in polysplenia syndrome. Acta Radiol 1991; 32: 299-301.

10. Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR 1991; 156: 799-800.