Pathogenetische Bedeutung und klinische Assoziation und Relevanz des sonographischen Phänomens des „Periportalen Cuffing“ anhand einer retrospektiven Auswertung von 100 dokumentierten Fällen

The aim of my thesis was to determine the prevalence of echo-rich and echo-poor periportal cuffing and correlate ultrasonographic findings with clinical data. From April 2002 to April 2008 n = 100 patients with periportal cuffing of about 10500 abdominal examinations were detected in the interdisciplinary ultrasound unit of the University Hospital Marburg. I retrospectively analysed the clinical diagnoses and laboratory values of these 100 patients. Echomorphology of periportal cuffing was evaluated and clinical diagnoses of the underlying diseases were clustered in four main groups: Liver diseases, haematological diseases, bowel diseases and others. Furthermore, liver function tests and body mass index were determined. The patients (62 male/38 female) had a mean age of 57.06 years (SD ± 19.47). Mean body-mass-index was 24.87 kg/m² (SD ± 4.28). N = 91 patients (91 %) showed echorich periportal cuffing, while echo-poor periportal cuffing was detected in n = 9 patients (9 %). Echo-poor periportal cuffing was significantly more often associated with malignant diseases as echo-rich periportal cuffing (78 vs. 36 %) (p < 0.025). The liver diseases group was subdivided into malignant (n = 10), cholestatic (n = 8), infectious (n = 8), and autoimmune (n = 7). Bowel diseases (n = 34) were classified in the upper gastrointestinal tract n = 7 (7 %), lower gastrointestinal tract n = 21 (21 %) and the pancreas n = 6 (6 %). Haematological disorders (n = 15, 15 %) were grouped to chronic myeloproliferative n = 2 (2 %), lymphoma n = 8 (8 %), leukemia n = 4 (4 %) and miscellaneous n = 1 (1 %). Other diseases accounted for 18 (18%) of cases. The analysis of the laboratory values showed an elevation of the aspartat-aminotrasferase (AST) and alanin-aminotransferase (ALT) in 39 patients (40 %) and 34 patients (35 %), respectively. Total bilirubin was elevated in 35 patients (36 %). In contrast, raised levels of AP were found in 49 patients (50 %), while the γ-GT value was elevated in 58 patients (59 %). Periportal cuffing of the liver is an extremely rare ultrasonographic phenomenon with a prevalence of ~ 0.95 % in our unit. Echo-rich periportal cuffing occurs more frequently than echo-poor periportal cuffing. The majority of echo-poor periportal cuffing is associated with malignant disorders, in particular haematological diseases, whereas echo-rich periportal cuffing is most frequently seen in inflammatory bowel disease patients. To glean further insights into this intriguing imaging phenomenon, follow up examinations were conducted in a small subset of patients with inflammatory bowel disease (n = 4). In addition to B-mode ultrasonography, contrast-enhanced ultrasonography (CEUS) and a MRI/MRCP were performed. Furthermore p-ANCA, c-ANCA and the Crohn´s Disease Activity Index (CDAI) were determined. Interestingly, we identified an “on-and-off” phenomen of echo-rich periportal cuffing and a positiv p-ANCA-titer in all four patients. Owing to low and medium CDAI scores there was no evidence for an association of periportal cuffing and acute bowel inflammation. Furthermore periportal oedema and lymph fluid obstruction could be excluded by MRI/MRCP. Therefore, I conclude that echo-rich periportal cuffing may result from aberrant trafficking of mucosal lymphocytes to the portal tract of the liver via the enterohepatic circulation. However, histological and molecular evidence has not been provided so far and invasive sampling of tissue biopsies is hampered by the presence of main vessels in the vicinity of the bile ducts.