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Aberrant collaterals in cirrhosis and challenges in its management

Published:November 22, 2022DOI:https://doi.org/10.1016/j.jceh.2022.11.009

      Abstract

      Portosystemic collateral formation in cirrhotic patients plays an essential role in the natural history of patients. Thorough understanding of collateral anatomy and hemodynamics in cirrhotics is important to envisage diagnosis and outcomes of portal hypertension. The understanding of the patterns of aberrant portosystemic collateral channels has important implications both for the clinician and the interventionist. In this case report, our patient presented to us with formation of aberrant collaterals at the site of subcostal hernia for which he underwent a mesh repair 8 years back. The technical challenges in the management of shunt closure of these aberrant collaterals were discussed.

      Graphical abstract

      Keywords

      Abbreviations:

      dL:
      Deciliter
      PT:
      Prothrombin time
      INR:
      International normalized ratio
      HIV:
      Human Immunodeficiency Virus
      HBsAg:
      Hepatitis- B surface Antigen
      Anti-HCV:
      ntibody against Hepatitis C Virus
      Hb:
      Hemoglobin
      TLC:
      Total Leucocyte Count
      AST:
      Aspartate Transaminase
      ALT:
      Alanine Transaminase
      ALP:
      Alkaline Phosphatase

      Introduction

      Portal hypertension (PHTN), characterized by increase in the portal venous pressure, is an important consequence of cirrhosis of liver[
      • Cichoz-Lach H.
      • Celinski K.
      • Slomka M.
      • et al.
      Pathophysiology of portal hypertension.
      ]. It is a result of both increased intrahepatic vascular resistance and augmented blood flow through the portal venous system[
      • Cichoz-Lach H.
      • Celinski K.
      • Slomka M.
      • et al.
      Pathophysiology of portal hypertension.
      ]. The high pressure hepatopetal flow is directed through alternative pathways into the low-pressure systemic veins, leading to formation portosystemic collateral vessels (PSCV) [
      • Johns T.N.
      • Evans B.B.
      Collateral pathways in portal hypertension.
      ].

      Case Report

      In our index case, a 41-year-old male presented to us in our gastroenterology OPD with abdominal distension in the form of ascites since 2015.On evaluation he was diagnosed to be having cirrhosis (CHILD –B) CTP score 9/15 with a MELD score of 12.After thorough etiological evaluation and after ruling out all the possible etiologies of cirrhosis, patient was labelled to be having cryptogenic cirrhosis. The patient had no history of hematemesis, melena, hematochezia, postural symptoms, jaundice, altered sensorium, decreased urine output. The ascites component was managed medically well on low dose diuretics. Subsequently, patient had developed subcostal hernia, for which he underwent a mesh repair in 2015. The procedure was uneventful. Patient also developed umbilical hernia in 2016, for which again mesh repair surgery was done which was uneventful.
      From 2016 to 2021 patient was apparently asymptomatic when he started noticing spontaneously occurring right subcostal globular painless swelling in June 2021. The swelling was progressively increasing in size without any ulceration, or inflammatory changes over it (Figure 1). He never experienced complaints about other systems, and history review for other systems was non–contributory. He was otherwise a normotensive, vegetarian. non-diabetic, non-smoker and non-alcoholic.
      Figure 1
      Figure 1Clinical photograph from the right lateral aspect at presentation showing a prominent subcostal bulge (arrow) and a scar of previous mesh repair (dashed arrow).
      On examination, he was comfortable in a lying-down position. He was neither pale nor icteric. His vitals were stable, with a blood pressure of 122/74 mmHg and a pulse rate of 86 beats per minute. He had a respiratory rate of 16 per minute and a temperature of 98.4 °F.
      On abdominal examination, the swelling was globular, had regular surface and margin, soft in consistency, with no tenderness and was mobile in both the planes with no movement that occurred with respiration. On auscultation bruit was audible.
      On examination, patient had ascites (International Club of Ascites-grade II), and splenomegaly. On evaluation, patient had Hb-10.4g/dL, TLC-3930,platelet count-56000. Liver function test showed total bilirubin of 0.92 mg/dL with conjugated fraction being 0.41 mg/dL, elevated aspartate aminotransferase(AST)- 45 U/L, alanine aminotransferase(ALT)-54U/L, alkaline phosphatase(ALP)-145 U/L, with serum albumin of 4.1g/dL and globulin of 3.6 g/dL.Kidney function test showed urea of 17 mg/dL and creatinine of 0.8 dL. His coagulation profile showed PT-16.7 seconds and INR -1.2.His viral markers for HBsAg, HIV and Anti HCV Ab were negative. Upper gastrointestinal endoscopy done in October 2021 showed no varices and mild portal hypertensive gastropathy.
      Ultrasound with colour doppler (Figure 2) of the subcostal swelling showed a tangle of vessels in the subcutaneous plane just beneath the anterior abdominal wall in the subcostal location with prominent venous flow. The main portal was dilated and a prominent recanalized paraumbilical vein was also seen. For further evaluation of the same, a multiphase CT was performed, which showed evidence of cirrhosis with portal hypertension. It also confirmed the presence of aberrant PSCV with the formation of a tangle of collaterals beneath the right anterior abdominal wall in the subcostal location. The recanalized paraumbilical vein was the afferent supply to the aberrant PSCV and the internal mammary vein through the superior epigastric vein was the predominant efferent draining vein (Figure 3).
      Figure 2
      Figure 2Colour doppler ultrasound of the subcostal swelling showed a tangle of vessels beneath the abdominal wall (arrows) and recanalized paraumbilical vein (dashed arrows).
      Figure 3
      Figure 3Portal venous phase CT in axial (A), coronal (B) and sagittal (C) planes showing aberrant collaterals in the intramuscular/subcutaneous plane (arrows) in the subcostal location with recanalized paraumbilical vein (open arrows) as the afferent and the internal mammary vein (dashed arrows) through the superior epigastric vein as the predominant efferent/draining vein.
      As patient had risk of bleeding from aberrant collaterals at the site of globular swelling from trivial trauma,he underwent percutaneous embolisation as a treatment modality for the same. A combined retrograde-direct puncture approach was planned (Figure 4). Right common femoral vein access was established under ultrasound and fluoroscopic guidance. The right internal mammary vein was hooked and selective venogram showed opacification of the subcostal bunch of collaterals with prompt washout of contrast (secondary to flow from portal vein). A 16 x 12 mm vascular plug was deployed and 0.5 ml of 50 percent glue was injected just proximal to the plug. Subsequently, percutaneous puncture of the varices was performed under ultrasound guidance. Contrast study through the percutaneous puncture showed near complete stasis of contrast with minimal washout. This was followed by injection of a 50 percent mixture of glue(1 ml) and a mixture of 10 ml setrol, 5ml lipiodol and 15 ml air made as a foam. The percutaneous puncture tract was embolized with 50 percent of 1 ml glue (Figure 5).There were no periprocedural complications. Follow-up doppler on post-procedure day 1 showed complete thrombosis of the collaterals and was discharged. Clinical and radiological follow-up after two weeks and six weeks of the procedure, showed near-complete resolution of the subcostal globular swelling. Follow up endoscopy, showed low risk varices.There was no acute decompensation in the form of bleed, worsening ascites, encephalopathy, hepatorenal syndrome in follow up visits
      Figure 4
      Figure 4Percutaneous embolization of the aberrant collaterals. A combined retrograde-direct approach was planned. Lateral venogram (A) after selective cannulation shows opacification of the right internal mammary vein (arrow). Lateral fluoroscopic image (B) shows a vascular plug (dashed arrow) deployed in the right internal mammary vein from the femoral route. Direct puncture of the collateral was performed (open arrow in C) under ultrasound guidance and contrast study (C) shows near complete stasis. This was followed by injection of a mixture of glue and sclerosant (D) and embolization of the tract with glue (E) which is seen as a glue cast (asterisk).
      Figure 5
      Figure 5Post procedure non-contrast CT in axial (A), coronal (B) and sagittal (C) planes shows the complete deposition of glue and sclerosant cast in the subcostal collaterals (arrows) and the vascular plug (dashed arrows) with proximal glue cast (open arrows) in the right internal mammary vein.
      Figure 6
      Figure 6A pictorial representation of the aberrant collateral circulation along with the radiological management performed.

      Discussion

      Portosystemic pathways form usually secondary to angioarchitectural alterations in the liver in which the blood bypasses an occlusion, flowing from high pressure to low pressure areas of the vasculature.
      Detection of spontaneous PSCV forms an important tool in diagnosing portal hypertension and predicting prognosis[
      • Sharma M.
      • Rameshbabu C.S.
      Collateral pathways in portal hypertension.
      ]. The radiological appearances of the common PSCV, including gastro-oesophageal and paraoesophageal collaterals, gastrorenal or splenorenal shunts, and paraumbilical shunts have been studied in detail[
      • Cho K.C.
      • Patel Y.D.
      • Wachsberg R.H.
      • Seeff J.
      Varices in portal hypertension: evaluation with CT.
      ,
      • Moubarak E.
      • Bouvier A.
      • Boursier J.
      • et al.
      Portosystemic collateral vessels in liver cirrhosis: a three-dimensional MDCT pictorial review.
      ,
      • Kang H.K.
      • Jeong Y.Y.
      • Choi J.H.
      • et al.
      Three-dimensional multidetector row CT portal venography in the evaluation of portosystemic collateral vessels in liver cirrhosis.
      ,
      • Saad W.E.
      • Lippert A.
      • Saad N.E.
      • Caldwell S.
      Ectopic varices: anatomical classification, hemodynamic classification, and hemodynamic-based management.
      ,
      • Sabri S.S.
      • Saad W.E.
      Anatomy and classification of gastrorenal and gastrocaval shunts.
      ]. However, with the advent of multi-detector row computed tomography (CT), unusual pathways of portosystemic anastomoses are increasingly being recognized[
      • Itai Y.
      • Saida Y.
      • Irie T.
      • et al.
      Intrahepatic portosystemic venous shunts: spectrum of CT findings in external and internal subtypes.
      ,
      • Ibukuro K.
      • Tsukiyama T.
      • Mori K.
      • et al.
      Transhepatic portosystemic shunts: CT appearance and anatomic correlation.
      ,
      • Ito K.
      • Fujita T.
      • Shimizu A.
      • et al.
      Imaging findings of unusual intra and extrahepatic portosystemic collaterals.
      ,
      • Kadoya M.
      • Takashima T.
      • Matsui O.
      • Kitagawa K.
      • Chohtoh S.
      Unusual portosystemic collateral penetrating the liver parenchyma.
      ,
      • Gebrael J.
      • Yu H.
      • Hyslop W.B.
      Spontaneous portoazygos shunt in a patient with portal hypertension.
      ,
      • Philips C.A.
      • Anand L.
      • Kumar K.N.
      • Kasana V.
      • Arora A.
      Rare, spontaneous trans-splenic shunt and intra-splenic collaterals with attendant splenic artery aneurysms in an adult patient with compensated cirrhosis and portal hypertension.
      ]. Since these shunts could be an important cause of variceal bleeding and hepatic encephalopathy, their identification is imperative in therapeutic decision making. In addition, understanding their anatomy may help to avoid complications related to interventional radiological procedures and surgery.
      The emphasis of this case report is that in case of any swelling in a cirrhotic patient, possibility of aberrant portosystemic collaterals should be considered and it should not be sampled upfront.
      Our case report highlights on the development of portosystemic collaterals in a patient of cirrhosis in an aberrant site that is the site of prior surgical intervention, along with the fact that such collaterals can be managed without surgery by radiological intervention. Other interventional option pertaining to our case could have been Transjugular intrahepatic portosystemic shunt.
      As we are aware that ectopic varices are low flow collaterals, doing TIPS would not have decreased the aberrant collaterals. Instead local embolization was always a better therapy for addressing such ectopic varices like in cases of fundal varices. Moreover, our patient didn't have any indication for TIPS like recurrent bleed or refractory ascites (not controlled by drugs). Hence for our patient we didn’t go ahead with TIPS
      Caput medusa is a frequent incidental finding in patients with portal hypertension that usually represents paraumbilical vein portosystemic collateral vessels draining into body wall systemic veins.
      In a case report published in J Vasc Interv Radiol 2005, it was shown that a 53-year-old man with refractory major hemorrhage from the caput medusa (fed not by the left portal vein but by the left gastroepiploic vein),was successfully treated by transjugular intrahepatic portosystemic shunt creation and balloon-occluded variceal sclerosis. This report showed that TIPS can also be effective in treating scar-related cutaneous portosystemic collateral vessel hemorrhage
      We also emphasize on the fact that by proper understanding of the various patterns of portosystemic collateral channels and sensible management, prevention of accidental vascular injury during intervention can be achieved.

      Conclusion

      The case report depicts an unusual aberrant portosystemic collateral pathways. Since these could be an important cause of bleeding and hepatic encephalopathy, gastroenterologists and radiologists should be familiar with the imaging findings to effectively identify them and aid in therapeutic decision making. Also, pre-operative knowledge of the anatomy and course of these uncommon portosystemic collaterals is essential for interventional radiologists and gastroenterologists to avoid inadvertent vascular injury during the procedures.

      Authorship Contribution Statement

      Anany Gupta: Study concept, design and drafting of the case report
      Shivanand Gamangatti: Radiological intervention and provision of images
      Sanchit Sharma: Study Supervision
      Srikanth Gopi: Critical revision of the manuscript for important intellectual content; Study Supervision
      Naren Hemachandran: Radiological intervention and provision of images
      Anoop Saraya: Study concept and design; critical revision of the case report for important intellectual content; Study Supervision

      Declaration of Competing Interest

      The authors declare no conflicts of interest.

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