Rare case of extrahepatic congenital porto-systemic venous shunt presented with lower gastrointestinal bleeding.

Nwachukwu I.; Matcovici M.; Patel J.; Alizai N.

Rare case of extrahepatic congenital porto-systemic venous shunt presented with lower gastrointestinal bleeding.

All Authors

Nwachukwu I.

Matcovici M.

Patel J.

Alizai N.

LTHT Author

Nwachukwu, Ijeoma
Matcovici, Melania
Patel, J.
Alizai, Naved

LTHT Department

Leeds Children's Hospital
Paediatric Surgery

Publication Date

2025

Item Type

Conference Abstract

Subject

ENDOSCOPY , RADIOLOGY , RADIOLOGY, INTERVENTIONAL , PAEDIATRICS

Abstract

Aims: Extrahepatic congenital porto-systemic venous shunts divert enteric venous return into systemic veins. It is a rare condition with 136 cases reported by 2010 and another 66 reviewed in 2020. Presentations include GI bleeding, systemic encephalopathy and liver tumors. In a reported series, GI bleeding was associated with shunts that drain into the iliac veins. Method(s): Patient's clinical notes were reviewed and a literature review was conducted. Result(s): We present the case of a six year old girl who presented acutely with severe bleeding per rectum (PR), requiring blood transfusion. Prior to the presentation she had a two year history of intermittent bright red PR bleeding. She had a normal OGD and ultrasound. Lower endoscopy showed a prominent rectal varix. Exploratory laparoscopy found a large vessel separate from the iliac veins behind the rectum. CT showed a markedly dilated 1.3 cm venous shunt from the superior mesenteric vein (SMV) to right internal iliac vein (RIIV) which was confirmed at venography. At occlusion test, no increased intrahepatic pressures were noted. Following discussion at the International Registry of Congenital Portosystemic Venous Shunts meeting, she underwent interventional radiology (IR) embolisation of the shunt with post procedure heparinisation. She developed a non-occlusive thrombus in the RIIV which resolved at 3 months. Oral anticoagulants were stopped and she is being followed up. Conclusion(s): This case highlights the importance of international MDT for rare cases and considerations for IR versus surgical treatment. Heparinisation and close follow up post procedures is paramount.