A 75-year-old woman presented to an outside hospital with hematochezia and hematemesis. She had a surgical history of two small bowel resections four and six years prior to presentation. Following these resections, she was left with 35 centimeters of small bowel and developed short-gut syndrome reliant on total parenteral nutrition (TPN). She had also developed ascites within the preceding four years, presumed to be secondary to parenteral nutrition-associated liver disease.
The patient was resuscitated with seven units of packed red blood cells and four units of fresh frozen plasma. Upper endoscopy at this time visualized grade 3 esophageal and grade 2 gastric varices. Balloon tamponade was performed with a Linton tube and the patient was transferred to a tertiary care hospital on octreotide and a proton pump inhibitor. VIR was consulted to evaluate the patient for a TIPS procedure; however, several findings were inconsistent with a working diagnosis of portal hypertension due to cirrhosis.
The patient did not have typical cirrhotic risk factors (no hepatitis, alcohol use disorder, or obesity) and fibrosis was not seen on a liver biopsy 4 years prior, so long-term use of TPN was the primary factor that would predispose her to cirrhosis. These considerations, along with a lack of cirrhotic features on an abdominal computed tomography angiography (CTA) scan, indicated that cirrhotic liver disease was unlikely. Closer inspection of the abdominal CTA scan revealed abnormal hyperenhancement and dilation of both the superior mesenteric vein (SMV) and portal vein (PV) in the arterial phase. Further review confirmed the presence of a SMAVF distal to the intestinal branches of the superior mesenteric artery (SMA) (Fig. 1).
We proceeded with embolization of the SMAVF to reduce portal pressure. A 6-F 25 cm sheath was placed in the right common femoral artery. A 5-F Cobra 1 catheter (Merit Medical, South Jordan, Utah) and 2.4-F Progreat microcatheter (Terumo, Tokyo, Japan) were used to cannulate the SMV across the fistula from the SMA (Fig. 2a). Then a 9 mm Eclipse dual-lumen balloon occlusion catheter (Cobalt Medical, Wayne, New Jersey) was used to measure pressure in the distal SMA (9.3 kPa) and the wedge pressure of the SMV (7.1 mmHg). The balloon catheter was then exchanged for a 4-F angled Glidecath (Terumo, Tokyo, Japan) to accommodate an 0.035” Rosen wire (Cook Medical, Bloomington, Indiana). Then, a 4-F 90 cm Flexor sheath (Cook Medical, Bloomington, Indiana) was advanced into the peripheral aspect of the SMV outflow and an 8-mm AMPLATZER Vascular Plug II (AGA Medical, Golden Valley) was deployed, extending from the SMV outflow into the distal SMA inflow. Post-embolization angiogram ensured that the plug was distal to major arterial intestinal branches and also demonstrated an absence of portal venous filling (Fig. 2b). The patient was transferred to the intensive care unit and subsequently discharged in stable condition on postprocedural day 8. Although her ascites persisted, she had no further bleeding during the remainder of her hospitalization.
An upper endoscopy at 1 month post-procedurally demonstrated complete resolution of both esophageal and gastric varices. Liver elastography performed shortly after this visit revealed minimal scarring with a kPa of 9.1 (METAVIR stage F2). At her 2- and 3-month postprocedural follow-up visits, the patient’s ascites had resolved, and she denied any recurrence of bleeding. This patient will continue to be followed in the VIR clinic indefinitely at 6-month intervals for surveillance of the SMAVF and liver disease management.