Median arcuate ligament syndrome and arterial anastomotic bleeding inducing hepatic artery thrombosis after liver transplantation

Abstract

Rationale:Median arcuate ligament (MAL) may compress the coeliac trunk inducing median arcuate ligament syndrome (MALS). MALS is a risk factor for hepatic artery thrombosis (HAT) in liver transplant recipients.Patient concerns:A thin female complained about upper abdominal pain for two months.Diagnoses:The patient was diagnosed as primary biliary cirrhosis.Interventions:The patient received two liver transplantations. During the first liver transplantation, the hepatic artery (HA) pulsations were normal. Doppler B ultrasonography showed normal blood flow in the HA in the first week. A 4 cm hematoma was detected in the first porta hepatis. On the ninth day, the hematoma had increased to 9 cm along with development of HAT. Exploratory laparotomy was performed. Bleeding at the site of arterial anastomosis was considered to be the reason for the hematoma. Doppler imaging revealed no blood flow in the liver. Computed tomography angiography demonstrated MALS. Salvage liver transplantation combined with dissection of MAL was performed. The maximum velocity of HA increased to 87 cm/s.Outcomes:The patient was discharged from the hospital 17 days after the second transplantation. At discharge, the liver function was normal and Doppler showed good blood flow in the HA.Lessons:MALS can cause HAT after liver transplantation. Before the liver transplantation, we should use Doppler B ultrasonography and sagittal CT imaging to judge whether the patient is with MALS. Also, before arterial anastomosis in liver transplantation is conducted, we should observe the impacts on the HA caused by pre-blocking gastroduodenal artery, which determines if we are supposed to do MAL dissection or bridge HA with aorta.

Contemporary Management of Median Arcuate Ligament in Liver Transplantation

“…32,33 It was not until 1972 that Colapinto et al demonstrated the presence of the MAL using CT. 7 Imaging modalities including CT or MRI aid the diagnosis, especially a phase-contrast MRI. 34 Given the changes in flow with the respiratory cycle, Sun et al 1 have suggested an endinspiratory arterial phase and an end-expiratory portovenous phase with sagittal arterial reconstruction in cases where a CT angiogram is used for diagnosis.…”

“…3,4,9,12,13,15 There was a lack of uniformity in the severity classification of MAL across the manuscripts, 9,15 and the majority of the transplanted organs were from deceased donors. 1,[7][8][9]11,13,[21][22][23]…”

“…Computerized tomography (CT) [1][2][3]6,8,9,11,17,21,23,25,28,29 and duplex ultrasound 1,3,6,7,[11][12][13]17,22,23,25,26,28 were the radiological modalities most frequently used to diagnose and facilitate the management of MAL.…”

An Atypical Presentation of Median Arcuate Ligament Syndrome

“…In 10% to 24% of the population, an unusually low-lying MAL passes anterior to the celiac artery causing some degree of stenosis triggering abdominal symptoms [1,2,6]. Celiac trunk is most prone to constriction by MAL when the distance between the celiac trunk root and the diaphragmatic crura is short, i.e, an abnormally low-lying diaphragmatic MAL [7,8].…”

“…Patients are usually 30 to 50 years old, thin females, who have had several workups for diagnosing the source of abdominal pain [1,4]. Most patients have incidental findings diagnosed on computed tomography (CT) scan and require no treatment [7]. Kuruvilla et al mention the use of a mesenteric ultrasound during deep expiration as the modality makes use of the increased blood flow velocity developed in areas compressed due to celiac artery stenosis/constriction [6].…”