Ciliated Hepatic Foregut Cysts: Not as Rare as Previously Believed.

Background. Ciliated hepatic foregut cysts (CHFCs) are uncommon cystic lesions within the liver. CHFCs can undergo a malignant transformation to form a primary squamous cell carcinoma of the liver. The true incidence and natural history of CHFCs is unknown and the risk of malignant transformation is unclear. We present a single centre's experience of CFHC management. Methods. A retrospective review of a departmental database identified all patients with CHFCs over a 4 year time period. Patients with CHFCs confirmed on histological assessment or suspected on radiological imaging were included in this study. Clinical information regarding patient demographics, symptomatic presentation, surgical management and histopathological features were noted. The radiological characteristics of CHFCs were recorded and the malignant transformation rate was calculated. Results. 15 patients with CHFC were identified (7 histologically confirmed and 8 radiologically suspected cases). All patients were asymptomatic and the CHFCs were incidental findings. No CHFC developed an interval change in cyst features or underwent a malignant transformation during follow up. MRI serves as the most sensitive modality to diagnose CHFC. Conclusions. CHFCs may be more prevalent than previously reported. Definitive management should encompass a patient centred discussion regarding the merits of long term follow up with serial imaging versus resection on an individual basis once CHFC is diagnosed.

Interobserver variation in the interpretation of magnetic resonance enterography in Crohn's disease.

OBJECTIVES: To evaluate interobserver variability for diagnosis of disease presence and extent of small bowel and colonic Crohn's disease using MR enterography (MRE). METHODS: Data from the first 73 consecutive patients (mean age 32, 33F, 28 new diagnosis, 45 suspected relapse) recruited to a multicentre, prospective diagnostic accuracy trial evaluating MRE for small bowel Crohn's disease were each read independently by three (from a pool of 20) radiologists. Radiologists documented presence and segmental location of small bowel Crohn's disease and recorded morphological mural/extramural parameters for involved segments. Per patient percentage agreement for disease presence and extent were calculated against an outcome-based construct reference standard (averaged between pairs of readers). Prevalence-adjusted bias-adjusted κ (PABAK) was calculated. RESULTS: Agreement for small bowel disease presence for new diagnosis/relapsed patients was 68%(κ = 0.36)/ 78% (κ = 0.56) and 43%(κ = 0.14)/ 53% for disease extent (κ = 0.07), respectively. For disease presence, all three radiologists agreed correctly with the reference standard in 41/59 (69%) of patients with small bowel involvement, and in 8/14 (57%) cases of without small bowel disease. Agreement was highest for multisegment disease, greater than 5 cm in length, with mural thickness>6 mm, and increased mural T2 signal. Agreement for colonic disease presence was 61% (κ = 0.21 fair agreement) for new diagnosis/ 60% (κ = 0.20, slight agreement) for relapsed patients. CONCLUSION: There is a reasonable agreement between radiologists for small bowel disease presence using MRE for newly diagnosed Crohn's disease, and patients with suspected relapse, respectively. Agreement is lower for disease extent. ADVANCES IN KNOWLEDGE: There is reasonable agreement between radiologists for small bowel disease presence using MRE for newly diagnosed (68%) Crohn's disease, and patients with suspected relapse (78%). Agreement is lower for disease extent (43% new diagnosis and 53% suspected relapse).

Reporting standards for primary sclerosing cholangitis using MRI and MR cholangiopancreatography: guidelines from MR Working Group of the International Primary Sclerosing Cholangitis Study Group.

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disorder affecting the bile ducts and is characterized by biliary strictures, progressive liver parenchymal fibrosis, and an increased risk of hepatobiliary malignancies primarily cholangiocarcinoma (CCA). PSC may lead to portal hypertension, liver decompensation, and liver failure with the need for liver transplantation. Magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP) are considered the imaging standard for diagnosis and follow-up in patients with PSC. Currently, there are no universally accepted reporting standards and definitions for MRI/MRCP features. Controversies exist about the definition of a high-grade stricture and there is no widely agreed approach to their management. The members of the MRI working group of the International Primary Sclerosing Cholangitis Study Group (IPSCSG) sought to define terminologies and reporting standards for describing MRI/MRCP features that would be applied to diagnosis and surveillance of disease progression, and potentially for evaluating treatment response in clinical trials. In this extensive review, the technique of MRI/MRCP and assessment of image quality for the evaluation of PSC is briefly described. The definitions and terminologies for severity and length of strictures, duct wall thickening and hyperenhancement, and liver parenchyma signal intensity changes are outlined. As CCA is an important complication of PSC, standardized reporting criteria for CCA developing in PSC are summarized. Finally, the guidelines for reporting important changes in follow-up MRI/MRCP studies are provided. KEY POINTS: • Primary sclerosing cholangitis is a chronic inflammatory disorder affecting the bile ducts, causing biliary strictures and liver fibrosis and an increased risk of cholangiocarcinoma. • This consensus document provides definitions and suggested reporting standards for MRI and MRCP features of primary sclerosing cholangitis, which will allow for a standardized approach to diagnosis, assessment of disease severity, follow-up, and detection of complications. • Standardized definitions and reporting of MRI/MRCP features of PSC will facilitate comparison between studies, promote longitudinal assessment during management, reduce inter-reader variability, and enhance the quality of care and communication between health care providers.

Prevalence and long-term outcome of sub-clinical primary sclerosing cholangitis in patients with ulcerative colitis.

BACKGROUND: Primary sclerosing cholangitis (PSC) is closely associated with inflammatory bowel disease, particularly ulcerative colitis (UC), with an increased risk of biliary and colorectal malignancy. We sought to clarify the prevalence, characteristics and long-term outcome of sub-clinical PSC diagnosed by magnetic resonance cholangiogram (MRC) in patients with UC and normal liver biochemistry, with or without colorectal dysplasia (CRD). METHODS: In this prospective case-control study, 70 patients with UC and normal liver function (51 extensive UC, 19 CRD), 28 healthy volunteers (negative controls) and 28 patients with PSC and cholestasis (positive controls) underwent MRC and blood evaluation. MRC scans were interpreted blindly by two radiologists who graded individually, the scans as definitive for PSC, possible for PSC or normal. Clinical outcome was assessed by blood monitoring, abdominal imaging and endoscopic surveillance. RESULTS: 7/51 (14%) with extensive UC and 4/19 (21%) with CRD had biliary abnormalities on MRC consistent with PSC. 7/11 (64%) with sub-clinical PSC had isolated intrahepatic duct involvement. Sub-clinical PSC was associated with advanced age (P = .04), non-smoking (P = .03), pANCA (P = .04), quiescent colitis (P = .02), absence of azathioprine (P = .04) and high-grade CRD (P = .03). Inter-observer (kappa = 0.88) and intra-observer (kappa = 0.96) agreement for MRC interpretation was high. No negative controls were assessed as definite PSC, 4/28 were considered on blinding as possible PSC. During follow-up of sub-clinical PSC (median 10.1(3.1-11.9) years), four patients developed abnormal liver biochemistry, two had radiological progression of PSC and seven developed malignancy, including two biliary and one colorectal carcinoma. CONCLUSIONS: Prevalence of sub-clinical PSC appears high in patients with extensive UC and normal liver biochemistry, with or without CRD. Disease progression and malignancy were identified on long-term follow-up. MRC should be considered for all patients with extensive UC or CRD to stratify surveillance.

Experience from the first UK inter-regional specialist multidisciplinary meeting in the diagnosis and management of IgG4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) is a complex multisystem fibro-inflammatory disorder, requiring diagnostic differentiation from malignancy and other immune-mediated conditions, and careful management to minimise glucocorticoid-induced toxicity and prevent progressive organ dysfunction. We describe the experience of the first inter-regional specialist IgG4-RD multidisciplinary team meeting (MDM) incorporating a broad range of generalists and specialists, held 6-weekly via web-link between Oxford University Hospitals NHS Foundation Trust and University College London Hospitals NHS Foundation Trust. Over 3 years, there were 206 discussions on 156 patients. Of these, 97 (62%) were considered to have definite or possible IgG4-RD; 67% had multi-organ involvement and 23% had a normal serum IgG4. The average number of specialist opinions sought prior to MDM was four per patient. Management was changed in the majority of patients (74%) with the treatment escalation recommended in 61 cases, including 19 for rituximab. Challenges arose from delays and misdiagnosis, cross-specialty presentation and the management of sub-clinical disease. Our cross-discipline IgG4-RD MDM enabled important diagnostic and management decisions in this complex multisystem disorder, and can be used as a model for other centres in the UK.

Evolving role of magnetic resonance techniques in primary sclerosing cholangitis.

Development of non-invasive methods to risk-stratify patients and predict clinical endpoints have been identified as one of the key research priorities in primary sclerosing cholangitis (PSC). In addition to serum and histological biomarkers, there has been much recent interest in developing imaging biomarkers that can predict disease course and clinical outcomes in PSC. Magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) continue to play a central role in the diagnosis and follow-up of PSC patients. Magnetic resonance (MR) techniques have undergone significant advancement over the last three decades both in MR data acquisition and interpretation. The progression from a qualitative to quantitative approach in MR acquisition techniques and data interpretation, offers the opportunity for the development of objective and reproducible imaging biomarkers that can potentially be incorporated as an additional endpoint in clinical trials. This review article will discuss how the role of MR techniques have evolved over the last three decades from emerging as an alternative diagnostic tool to endoscopic retrograde cholangiopancreatography, to being instrumental in the ongoing search for imaging biomarker of disease stage, progression and prognosis in PSC.

Exophytic cystic liver lesion: an unusual presentation of a hepatocellular carcinoma in a young female.

Hepatocellular carcinoma (HCC) is one of the most common malignant primary liver tumours. However, primary hepatic carcinomas are rare in young adults, accounting for approximately 1% of tumours in people below the age of 20. Hepatitis B virus (HBV) and hepatitis C virus (HCV) infection are the two most important aetiological agents of HCC. The average age at onset of HBV-related HCC (~50 years old) is 10 years younger than that of HCV-related HCC (61-64 years). Evidence for an association between the oral contraceptive pill (OCP) and development of HCC remains inconclusive. Here, we describe a case of a 28-year-old woman with normal background liver, who presented with a large palpable abdominal mass due to a bilobar, exophytic, cystic lesion arising from the right lobe of the liver, later diagnosed as HCC on histological analysis. We highlight the need for considering HCC even in the unusual setting of a cystic, exophytic lesion.

Possible congenital dilatation of the pancreatic duct.

The main pancreatic duct can become dilated in a number of conditions. We describe a patient with gross dilatation of the main pancreatic duct without evidence of causative underlying pathology suggesting congenital dilatation of the pancreatic duct. A 36-year-old man presented with signs of intestinal obstruction and a history of surgery for congenital pyloric stenosis. Incidental findings on CT showed a massively dilated main pancreatic duct. On MRI there was no duct irregularity or solid mural nodule, making a main duct intraductal papillary mucinous neoplasm unlikely. Endoscopic ultrasound findings were in keeping with those on MRI. Fine needle aspiration revealed a non-viscous fluid with a low carcinoembryonic antigen and high amylase concentration, consistent with normal pancreatic fluid levels rather than a mucinous collection. After 1 year, the cyst remains unchanged. This patient will be kept under surveillance with yearly MRI.

Transient small bowel intussusception in adults: an overlooked feature of coeliac disease.

Coeliac disease is the commonest immunological gastrointestinal disorder in the Western world. The symptoms of coeliac disease in adults are often non-specific, and a high index of suspicion may be required for timely diagnosis. We describe the case of a 46-year-old woman, with known dilated cardiomyopathy and pulmonary hypertension, who presented with non-specific abdominal symptoms, not initially attributed to gastrointestinal disease. Radiological investigations demonstrated transient small bowel intussusception without other abnormality, leading to the suggestion of coeliac disease as a cause, which was subsequently confirmed as the diagnosis.

Small bowel imaging in Crohn's disease.

Imaging of Crohn's disease of the small bowel is gradually moving away from barium fluoroscopy and towards cross-sectional modalities. This review explores the strengths and limitations of various techniques, and focuses on several current questions in small bowel imaging, such as the comparison between oral ingestion or nasojejunal intubation and enteroclysis for introduction of contrast, the use of computerised tomography (CT) versus magnetic resonance (MR) and the likely changes over the next decade.

Computed tomographic appearances of hepatic vascular lesions.

Improvements in Imaging technology have revolutionized liver imaging and allowed the recognition of abnormalities arising from various architectural components such as vessels and biliary ducts of the liver. A spectrum of various hepatic vascular disorders as demonstrated by multidetector CT (MDCT) is presented in this review article.

Minimal-preparation CT colon in detection of colonic cancer, the Oxford experience.

BACKGROUND: the main colonic imaging modalities, including barium enema, colonoscopy and computed tomography colonography, require bowel preparation. Performing these imaging procedures in the elderly can difficult due to immobility, incontinence and poor tolerance of bowel cleansing. Minimal preparation CT (MPCT) colon was introduced in the early 1990s in the UK. Much of the published literature on MPCT colon is limited by small patient numbers and short duration of follow-up. OBJECTIVE: the aim of this study is to review our experience with the MPCT technique involving a large consecutive cohort of patients with long follow-up. METHODS: all studies of MPCT performed in a 1-year period between July 2000 and July 2001 at our institution were reviewed retrospectively. MPCT reports were cross-referenced with the cancer registry to allow for an average period of 30 months follow-up. A definite diagnosis of cancer was only given following the appearance on the cancer registry. Those patients who had negative MPCT colon were assumed to be true negatives if no corresponding name was identified on the cancer registry. In the event of data mismatch, patient notes were reviewed to ascertain a diagnosis. RESULTS: 391 MPCT examinations were performed during the period of the study (209 males, median age 82: age range 56-91 years). Thirty-four patients who had MPCT colon during the study period appeared on the cancer registry. A further three patients with disseminated colorectal malignancy identified on MPCT colon died without histological confirmation (tumour prevalence = 9.5%). Thirty-two of the registry confirmed 34 cases were detected on MPCT colon, giving a sensitivity of 0.94 (95% confidence interval 0.86-1.00). Including the three cases without histological confirmation gives a slightly higher sensitivity of 0.95. There were seven patients with definitely abnormal MPCT colons, who did not appear on the registry, resulting in specificity for definite abnormality of 0.98 (confidence interval 0.97-1.0). However, three of these seven are those who died of disseminated colorectal malignancy as above, raising the specificity to 0.99. Fourteen cases (3.5%) of extra-colonic malignancies were observed in this study. CONCLUSION: even with the longer follow-up of this large cohort of patients the sensitivity and specificity in our study for the diagnosis of colorectal cancer with MPCT remains comparable with that of other studies and this technique competes well with other common colonic imaging modalities.