CASE REPORT

An 80-year-old male patient, with a recent diagnosis of chronic myeloid leuchemia and with no past medical history of cirrhosis, was admitted with sudden massive hematochezia and syncope. Early after admission, the patient developed hemorrhagic shock, requiring substantial transfusion support, and renal and respiratory failure.

Urgent upper endoscopy, ileocolonoscopy and CT angiography were inconclusive regarding the bleeding source. Push enteroscopy (anterograde approach), identified an intermittent spurting hemorrhage in the distal duodenum (D3), initially misinterpreted as being a Dieulafoy’s lesion (Figure 1A). Endoclips were placed in the bleeding lesion and the area was tattooed (Figure 1B).

Figure 1. Spurting bleeding in the distal duodenum (1A); endoclips placement and tattooing (1B).

Although initial hemostasis was achieved using endoclips, severe rebleeding occurred early after the procedure. A second enteroscopy identified an ectopic duodenal varix with active oozing underlying the previously applied endoclips. Cyanoacrylate injection was hampered by compromised visualization of the varix (endoclips and ongoing bleeding), but provided temporary hemostasis (Figure 2).

Figure 2. Cyanoacrylate injection.

Endoscopic ultrasound (EUS) showed patency (positive Doppler flow) of this large duodenal varix, even after these two attempts of endoscopic therapy (Figure 3).

Figure 3. Large varix in the distal duodenum (3A – upper figure), with positive Doppler flow (3B – lower figure).

In a multidisciplinary team meeting, it was decided to perform angiographic therapy. Percutaneous (transhepatic) portography allowed the identification and selective catheterization of the varix (a collateral of the superior mesenteric vein). A communicating collateral from the superior mesenteric vein, feeding the duodenal varix, was successfully embolized during angiography (Video 1).

Video 1. Percutaneous (transhepatic) portography and selective embolization of the duodenal varix-feeding vein (collateral from the superior mesenteric vein).

Postprocedure EUS confirmed absence of Doppler flow in the duodenal varix, documenting complete occlusion after angiographic therapy (Figure 4).

Figure 4. Occlusion of the ectopic varix, with absence of Doppler flow.

The patient recovered uneventfully and remained asymptomatic on 3-month follow-up. No signs of cirrhosis or portal hypertension were documented and the etiology of the ectopic varix remained unknown – possibly related to chronic myeloid leuchemia (1).

Commentary

Ectopic varices account for 1-5% of all bleeding varices in the setting of portal hypertension. Their presence should be considered in patients with portal hypertension and gastrointestinal bleeding when both upper and lower endoscopy are not conclusive (2,3). In the absence of cirrhosis or signs of portal hypertension, diagnosis and management of bleeding from ectopic varices is challenging.

Although EUS is not recommended in the guidelines for gastrointestinal bleeding, this case shows that it can be useful in the approach of some of these patients. EUS was essential for the differential diagnosis of this duodenal bleeding lesion, confirming the diagnosis made on the second enteroscopy: ectopic varix. As occurred in our case, Doppler US not only allows us to identify culpable lesions but also to monitor the efficacy of the therapy delivered, showing the disappearance of the blood flow after therapy (4).

According to recent data, this technique can also have a therapeutic role in the management of digestive tract’s varices and has shown excellent results. In fact, preliminary reports suggest that EUS may be used for imaging and for guiding angiotherapy in different sources of refractory gastrointestinal bleeding: esophageal, gastric and ectopic varices, peptic ulcers, Dieulafoy lesions and gastrointestinal stromal tumors (4). Two main techniques have evolved (and can be used together) in EUS-guided therapy of ectopic varices: EUS-FNA injection of glue or coil. Both techniques showed to be effective in a recent randomized controlled trial of EUS-guided therapy of gastric varices: obliteration rates >90%, with few endoscopic sessions required (range, 1-3) and coil application showed to be safer (5). Main limitations to the use of this therapeutic technique are the risk of damaging the expensive echoendoscopes, the interference of blood clots in US imaging, the risk of extraluminal bleeding that may require salvage angiographic or surgical intervention and the need of special expertise (4,6).

References

1. Toros AB, Gokay S, Cetin G, et al. Portal hypertension and myeloproliferative neoplasms: a relationship revealed. ISRN Hematol 2013;2013:673781.

2. Helmy H, Kahtani K, Fadda M. Updates in the pathogenesis, diagnosis and management of ectopic varices. Hepatol Int 2008;2:322-334.

3. Sarin SK, Kumar CKN. Ectopic varices. Clin liver dis 2012;1:167-172.

4. Vazquez-Sequeiros E, Olcina J. Endoscopic ultrasound guided vascular access and therapy: A promising indication. World J Gastrointest Endosc 2010 June 16; 2(6): 198-202.

5. Romero-Castro R, Ellrichmann M, Ortiz-Moyano C, et al. EUS-guided coil versus cyanoacrylate therapy for the treatment of gastric varices: a multicenter study (with videos). Gastrointest Endosc 2013;78:711-721.

6. Wong Kee Solng LM, Banerjee S, Barth BA, et al. Emerging technologies for endoscopic hemostasis. Gastrointest Endosc 2012;75:933-937.

Authors

Joana Carmo ¹, Susana Marques ¹, Miguel Bispo ^1,2, Pedro Barreiro ^1,2, Pedro Pinto Marques ^2,3, David Serra ².

1. Department of Gastroenterology, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal

2. Department of Gastroenterology, Hospital da Luz, Lisbon, Portugal

3. Department of Gastroenterology, Hospital Garcia de Orta, Lisbon, Portugal