The New Gastroenterologist

The Answer
From What’s Your Diagnosis? on page 24

This is sinistral portal hypertension and gastric variceal bleeding secondary to splenomegaly from increased splenic blood flow owing to myelofibro- sis-induced extramedullary hematopoiesis. CT of the abdomen and pelvis revealed marked splenomegaly and prominent gastric (Figure B, C, arrow), paragastric, splenic, and mesenteric varices (Figure B, C, arrowhead). The patient underwent a liver biopsy, which revealed extramedullary hematopoiesis without evidence of fibrosis or nodular regenerative hyperplasia. Measurement of the hepatic venous pressure gradient was 8 mm Hg, confirming the absence of right-sided portal hypertension.

In our patient, sinistral portal hypertension and gastric variceal bleeding occurred secondary to splenomegaly from increased splenic blood flow owing to myelofibrosis-induced extramedullary hematopoiesis. Isolated gastric varices are less prevalent than esophageal or gastroesophageal varices.

Our patient had type 1 varices (IGV1), which are confined to the fundus. A common cause of IGV1 is splenic vein thrombosis and this diagnosis should be excluded. Initial management of gastric variceal bleeding includes antibiotics, vasoactive drugs, and selective transfusion.1

Beyond this initial management, studies have documented the use of splenectomy, splenic embolization, endoscopic variceal obturation (EVO) using tissue adhesives such as cyanoacrylate, endoscopic injection sclerotherapy (EIS), variceal band ligation (EBL), transjugular intrahepatic portosystemic shunt and balloon-occluded retrograde transvenous obliteration. 2 Cyanoacrylate has demonstrated higher hemostasis and lower rebleeding rates compared with EBL and EIS. A recent study has shown that endoscopic ultrasonographic (EUS)-guided therapy for fundal varices with cyanoacrylate and coils may improve efficacy and decrease embolization of glue. 3 Cyanoacrylate is not currently available in the United States. However, many experts agree that, in patients with bleeding gastric fundal varices, the use of cyanoacrylate is preferred where available, with EBL as an alternative.1

Splenectomy can be definitive treatment in patients with gastric varices associated with sinistral portal hypertension.1 In patients with myelofibrosis and splenomegaly, splenectomy is reserved for those with drug-refractory, symptomatic splenomegaly associated with frequent transfusions, portal hypertension, or severe thrombocytopenia.

In our patient, splenectomy was chosen over EVO or trans-
jugular intrahepatic portosystemic shunt owing to lack of
available cyanoacrylate in the United States and the lack of
elevated right-sided portal pressures. Postoperatively, his
white blood cell count was 6. 7 × 103/microL and platelet
count was 279 × 103/microL. He received low-molecu-
lar-weight heparin for splenic and portal vein thrombosis,
as well as hydrea and interferon. Repeat endoscopy 1 year
after surgery showed no evidence of any varices. This case
highlights the need for larger scale, randomized, controlled
trials to guide management of gastric variceal bleeding. In
addition, not all effective endoscopic and interventional tech-
niques to treat gastric varices are widely available, making it
difficult for providers to follow current recommendations. n

References

1. Garcia-Pagan J.C., Barrufet M., Cardenas A., et al. Management of gastric varices. Clin Gastroenterol Hepatol. 2014;12:919-28.

2. Goh B.K., Chen J.J., Tan H.K., et al. Acute variceal bleed in a patient with idiopathic myelofibrosis successfully treated with endoscopic variceal band ligation. Dig Dis Sci. 2007;52:173-5.

3. Binmoeller K.F., Weilert F., Shah J.N., et al. EUS-guided transesophageal treatment of gastric fundal varices with combined coiling and cyanoacrylate glue injection (with videos). Gastrointest Endosc. 2011;74:1019-25.