Rectal Bleed in a Child With Abernathy Malformation- Endovascular Management

Abstract

Abernathy malformations are congenital extrahepatic porto-systemic shunts which allow splanchnic circulation to bypass the metabolic screen of the liver and drain directly into the systemic circulation. The resulting metabolic abnormalities have a multitude of implications ranging from hyperammonaemia, hepatic encephalopathy, to pulmonary hypoxemia. The shunt also causes anatomical implications in the form of varices. Interventional radiology plays the central role in this era of minimal invasive surgeries from establishing diagnosis to therapeutic interventional management. The holistic approach provided through interventional radiology reduces intraprocedural time as well as hospital stay. We describe a very rare case of peripheral congenital porto-systemic shunt communicating Inferior mesenteric vein and internal iliac vein with rectal bleed with complete management at the department of interventional radiology.

Keywords: abernathy malformation; embolization; haematochezia; vascular plug.

Figure 1

a to f: US shows normal sized PV at the hepatic hilum with hepatopetal flow (a); Spleen appears normal in size (b); Dilated perisplenic vascular channel measuring upto 18.2 mm (c); High vascularity seen on Doppler (d); Retrovesical and perirectal dilated vascular channels seen with suspected fistulous communications (e & f). US: Ultrasound, PV: Portal vein.

Figure 2

a to f: CECT abdomen shows normal sized PV (a) with markedly dilated IMV (b), Near normal sized SV proximal to Confluence with IMV (c), Direct fistulous communication between IMV and Right Internal iliac vein (d and e), Thickened enhancing rectal mucosa (f). CECT: contrast enhanced computed tomography, PV: portal vein, IMV: inferior mesenteric vein, SV: splenic vein.

Figure 3

a to g: Right IJV access check venogram showing communication between internal iliac vein and IMV (a), Left common femoral access showing same shunt between the portal and systemic veins (b), Inflated occluding balloon in situ (c), Amplatzer Vascular plug II being placed at the shunt, Coils were placed with 1 ml of glue in-between (d–f), NCCT pelvis axial section post-procedure (g). IJV: internal jugular vein, IMV: inferior mesenteric vein, NCCT: Non-contrast computed tomography.